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Cryptogenic organizing pneumonia: Clinical and radiological features, treatment outcomes of 17 patients, and review of the literature

Authors:
  • University of Health Scienc Yedikule Hospital for Chest Disease and Thoracic Surgery

Abstract and Figures

Background/aim: We evaluated patients with cryptogenic organizing pneumonia (COP) who attended our clinic. Materials and methods: We retrospectively investigated the clinical and radiological findings, diagnostic methods, treatment, and follow-up outcomes of 17 patients who had been histopathologically diagnosed with COP. Results: The mean age of the patients was 49.8 ± 10.4 years. The most common symptom was cough (n = 15; 88.2%) and the most common radiological finding (n = 10) was consolidation in the inferior lobes on thoracic computed tomography. The diagnosis of COP was made by open lung biopsy in 11 (64.7%) patients, transbronchial biopsy in 5 (29.4%), and video-assisted thoracoscopic surgery biopsy in 1 (5.9%). The mean follow-up period was 28.7 ± 25.0 (range: 3-85) months. Twelve patients received oral corticosteroid therapy and seven of them improved without any fibrotic changes. One patient refused treatment; a chest radiography of that patient was found to be normal at the end of the 20-month follow-up period. Three patients received no other therapy, as the lesion had been completely excised. Conclusion: Common symptoms included cough and dyspnea, while the main radiological presentation of COP was consolidation. Corticosteroids are a good treatment option in general, but relapse may occur.
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http://journals.tubitak.gov.tr/medical/
Turkish Journal of Medical Sciences
Turk J Med Sci
(2016) 46: 1712-1718
© TÜBİTAK
doi:10.3906/sag-1508-114
Cryptogenic organizing pneumonia: clinical and radiological features,
treatment outcomes of 17 patients, and review of the literature
Elif Yelda NİKSARLIOĞLU*, Gülcihan Zehra ÖZKAN, Nur Dilek BAKAN, Sibel YURT, Lütfiye KILIÇ, Güngör ÇAMSARI
Department of Chest Diseases, Yedikule Chest Diseases and oracic Surgery Training and Research Hospital, İstanbul, Turkey
* Correspondence: eyelda2003@yahoo.com
1. Introduction
Cryptogenic organizing pneumonia (COP) is a disease
of unknown etiology, which is characterized by
granulation tissue obstructing the alveolar ducts and
chronic inammation occurring in contiguous alveoli
(1). Collagen vascular diseases, drugs, malignancies,
and aspiration can all lead to the clinical diagnosis of
secondary organizing pneumonia; the term COP, however,
is used for cases of unknown etiology. COP is included in
the class of idiopathic interstitial pneumonia in the joint
statement of the American oracic Society (ATS) and the
European Respiratory Society (ERS), which was revised in
2013 (2). Although there are various publications on the
clinical ndings, radiological ndings, and treatment of
this disease, most of the papers are case reports (3–5). In
the present study, we present the clinical and radiological
ndings, as well as the treatment and follow-up outcomes,
of 17 patients diagnosed with COP.
2. Materials and methods
A total of 17 patients who attended the Yedikule Training
and Research Hospital for Chest Diseases and oracic
Surgery and were subsequently diagnosed with COP were
retrospectively evaluated. Diagnosis of COP was made by
bronchoscopic transbronchial biopsy (TBB), video-assisted
thoracoscopy (VATS), and open lung biopsy (OLB). All
patients were followed between October 2006 and April
2014 at the hospital. ose with conrmed diseases that
might cause organizing pneumonia were excluded from
the study. e demographic data, symptoms, radiological
ndings, diagnostic methods, and treatment regimen for
each patient were collected from the patients’ hospital
records.
2.1. Statistical analysis
Descriptive statistics only are given. Statistical comparisons
were not performed because of the limited number of cases.
Continuous variables are presented as mean and standard
deviation, whereas categorical variables are presented as a
number and a percentage.
3. Results
Seventeen COP patients (49.8 ± 10.4 years; range: 29–
69) were retrospectively evaluated. Seven (41.2%) of the
patients were men. e demographic data are shown in
Background/aim: We evaluated patients with cryptogenic organizing pneumonia (COP) who attended our clinic.
Materials and methods: We retrospectively investigated the clinical and radiological ndings, diagnostic methods, treatment, and
follow-up outcomes of 17 patients who had been histopathologically diagnosed with COP.
Results: e mean age of the patients was 49.8 ± 10.4 years. e most common symptom was cough (n = 15; 88.2%) and the most
common radiological nding (n = 10) was consolidation in the inferior lobes on thoracic computed tomography. e diagnosis of COP
was made by open lung biopsy in 11 (64.7%) patients, transbronchial biopsy in 5 (29.4%), and video-assisted thoracoscopic surgery
biopsy in 1 (5.9%). e mean follow-up period was 28.7 ± 25.0 (range: 3–85) months. Twelve patients received oral corticosteroid
therapy and seven of them improved without any brotic changes. One patient refused treatment; a chest radiography of that patient
was found to be normal at the end of the 20-month follow-up period. ree patients received no other therapy, as the lesion had been
completely excised.
Conclusion: Common symptoms included cough and dyspnea, while the main radiological presentation of COP was consolidation.
Corticosteroids are a good treatment option in general, but relapse may occur.
Key words: Cryptogenic organizing pneumonia, follow-up, radiology, symptom, treatment
Received: 25.08.2015 Accepted/Published Online: 04.03.2016 Final Version: 20.12.2016
Research Article
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NİKSARLIOĞLU et al. / Turk J Med Sci
Table 1. e mean duration of cough, which was the most
common symptom, was 54 ± 30 days. Of those patients
with pleuritic chest pain, one had a pleural eusion and
four had peripheral lesions. Pulmonary function tests
(PFTs) were performed in 14 patients: one patient had
an obstructive pattern and ve patients had restrictive
patterns, whereas PFTs were considered to be normal in
the others (Table 2).
e most common radiological nding was
consolidation in the le (n = 10) and right (n = 6) lower
lobes (Figure; Table 3). While lesions were present in
more than one segment on computed tomography/high-
resolution computed tomography (CT/HRCT) in nine
(53%) patients, only one segment was involved in the
remaining eight patients. One of the patients had a mass
lesion on thorax CT with minimal pleural eusion on the
Table 1. General characteristics and symptoms of the patients.
Variables Results
Number of patients 17
Age, years 49.8 ± 10.4 (range: 29–69)
Sex (female) 10 (58.8)
Smoking 6 (35.3)
Current smoker 2 (12.5)
Ex-smoker 4 (25)
Cough 15 (88.2)
Dyspnea on exertion 8 (47.1)
Weakness 6 (35.3)
Pleuritic chest pain 6 (35.3)
Fever 4 (23.5)
Hemoptysis, 2 (12.5)
Comorbidity* 3 (18.5)
Data are presented as n (%), *: Vertigo, asthma, obstructive sleep apnea syndrome.
Table 2. Laboratory ndings and pulmonary function tests of patients.
Findings Valu e
Hemoglobin, g/dL 12.8 ± 2.3
Leukocyte count, /mm38488 ± 2077
Platelet count, /mm3313,700 ± 9150
Eosinophil, % 3.3 ± 1.8
Sedimentation, mm/h 45.8 ± 40.0
CRP, mg/L 5.9 ± 5.3
FEV1 (L)* 2.7 ± 0.8
FEV1 (%, predicted)* 88.1 ± 16.3
FVC (L)* 3.0 ± 0.9
FVC (% predicted)* 86.4 ± 18.6
FEV1/FVC* 84.2 ± 10.1
DLCO (mL/min/mmHg)** 36 ± 35
DLCO (% predicted)** 69.6 ± 5.5
Data are presented as mean ± standard deviation, * n = 13 cases; ** n = 3 cases, FEV1:
forced expiratory volume in 1 s, FVC: forced vital capacity, DLCO: diusion capacity of
carbon monoxide.
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NİKSARLIOĞLU et al. / Turk J Med Sci
same side. is patient had undergone positron emission
tomography (PET-CT) scanning at another center due to a
suspicion of malignancy before admission to our hospital,
and the maximum standardized uptake value (SUVmax)
was reported to be 10. In a second patient with a mass
lesion, PET-CT scans revealed no FDG uptake. e third
patient with a mass on thoracic CT was not evaluated with
PET-CT scan.
Fieen patients had undergone beroptic
bronchoscopy. Bronchoalveolar lavage (BAL) was
performed in ve patients. ree of the patients had a
normal cellular pattern on BAL, whereas two patients
had an elevated lymphocyte count (30% and 25%). No
growth was observed in bronchoalveolar lavage cultures.
e diagnosis of COP was made by OLB in 11 (64.7%)
patients, by TBB in 5 (29.4%), and by VATS biopsy in
1 (5.9%) Hepatitis markers were found to be negative.
Collagen markers were also negative in 15 patients.
Among the collagen vascular markers, antinuclear
antibody (ANA) positivity was determined in one patient,
and anticardiolipin antibody positivity was determined
in another. However, no pathology was detected upon
rheumatologic evaluation. None of the patients were
further diagnosed with collagen vascular disease over the
course of the follow-up period.
e mean follow-up period of the patients was 28.7 ±
25.0 months (median: 23 months; range: 3–85 months).
Eleven patients (64.7%) received oral corticosteroid (OCS)
therapy; the mean starting dose of methylprednisolone
was 23.2 ± 3 mg (median: 20 mg, range: 16–32 mg)
and the mean treatment period was 8.5 ± 4.7 months
(median: 8 months, range: 2–18 months). ere was a
complete improvement without any brotic changes in
eight patients who received OCS. Five patients did not
receive treatment; one of them, having refused treatment,
subsequently showed a spontaneous improvement in
symptoms and chest radiography was also normal at the
end of the 21st month. Four patients who did not receive
treatment had mass lesions at the start of treatment and a
total excision of the lesion was performed in all. Fibrotic
Figure. Chest radiography of a patient with COP. Bilateral consolidation and ground glass opacities are seen on thoracic computed
tomography.
Table 3. oracic computed tomography ndings of the cases.
Findings N (%)
Consolidation 8 (47.1)
Consolidation and ground glass opacities 3 (17.7)
Consolidation and nodules 2 (11.8)
Mass 3 (17.6)
Pleural eusion and nodule 1 (5.9)
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NİKSARLIOĞLU et al. / Turk J Med Sci
changes secondary to the procedure were observed in the
four patients who had undergone excision. No recurrence
was observed in these patients over the course of the
approximately 20-month follow-up period. One patient
couldn’t use OCS because her serum glucose level was
at the upper limit of normal values, so clarithromycin
and an inhaled corticosteroid/long-acting beta-mimetic
combination therapy were started. Partial regression was
seen on chest X-rays within 2 months but pulmonary
symptoms like cough and dyspnea persisted and a new
inltration was observed in a dierent location in the 3rd
month of follow-up. Aer excluding infectious etiologies,
32 mg of methylprednisolone was added to the existing
clarithromycin therapy. e patient is currently still being
followed. None of the patients in this study died over the
course of the follow-up period.
One patient was excluded for recurrence analyses
because the follow-up period was shorter than 5 months
(6). ree patients (18.7%) who received OCS developed a
recurrence of the disease during the course of the follow-
up period. Radiologic and clinic recurrence was detected
in two cases aer the steroid dose had been decreased;
therefore, the methylprednisolone dose was increased to 8
mg (stable dose for symptoms and radiological regression)
and regression was determined in the symptoms and
radiological ndings during follow-up. e patients were
in the 9th and 5th months of treatment when this article
was written and are still being followed. Partial regression
was observed in one patient receiving OCS.
4. Discussion
COP is a disease of unknown etiology and is diagnosed
histopathologically. (7). It was observed predominantly
in women and patients were aged between 29 and 69
years in our study. Common symptoms included cough
and dyspnea on exertion. Pleuritic chest pain and
weakness were seen in one-third of the patients. e main
radiological presentation of COP was consolidation in the
lower lobe. Corticosteroids are a good treatment option
in general, but relapse may occur. Clarithromycin therapy
did not prevent relapse in our patient.
COP is a subacute disease seen approximately between
the ages of 50 to 60 years, with an equal prevalence in
males and females (7–9). In the present study, the mean
age of our patients was 49.8 years and 10 of them were
female. e number of female patients was slightly higher
in the present study, and this was similar to the study of
Lazor et al. (10). COP is, in particular, more prevalent in
nonsmoking females and/or females who have stopped
smoking (10). In our patient group, 11 of the 17 patients
were nonsmokers, and two of the three female smokers had
stopped smoking. Despite the limited number of patients
in this study it can be seen that COP was more prevalent in
nonsmokers or in those who had stopped smoking.
e duration of symptoms varied between 1 and 3
months, with a mean duration of 1 month. Some studies
have reported a prolonged duration of symptoms,
up to 2–4 months (10–12). COP cases are frequently
diagnosed as pneumonia based on clinical, laboratory,
and radiological ndings. However, a denitive diagnosis
is made from further analyses performed in patients who
are unresponsive to treatment. e duration from onset
of symptoms to diagnosis varies between studies, and this
may lead to 6 to 10 weeks of delay in reaching a diagnosis
(8).
e most common symptoms encountered in our
patients were cough, dyspnea, pleuritic chest pain, and
weakness. Oymak et al. conducted a study of 26 cases
with bronchiolitis obliterans organizing pneumonia
(BOOP) and reported cough, dyspnea, pleuritic chest
pain, hemoptysis, and fever as being the most common
symptoms (3). Two patients were determined to have mild
hemoptysis in the present patient series. Hemoptysis has
been rarely determined in many previous studies, with
no information on the prevalence. A paper published in
2011 reported cough, fever, weakness, and dyspnea as the
most common symptoms in a group of 40 cases (12). In
that study, fever frequency was higher than in our study
(25% vs. 71.8%). Pleuritic chest pain is present particularly
in cases of peripheral lesions and pleural eusion. In the
present group, pleuritic chest pain was determined in six
cases. e prevalence of this symptom was close to the
rates found in some previous studies (3,13).
Considering the results of laboratory tests, previous
studies have reported slightly elevated ESR and serum
CRP levels and nonspecic increases in the peripheral
neutrophil count (5,14). In the present study, ESR was
found to be elevated in ve cases, CRP in four cases, and
peripheral eosinophil count in two cases in comparison
to the reference values. In another study, ANA positivity
was detected in 18.2% of patients and rheumatoid factor
positivity was detected in 10.3% (12). Yoo et al. compared
cases with COP with cases with connective tissue disease-
related organizing pneumonia and reported ANA positivity
in 31.9% of COP patients (15). In our patient group there
was isolated ANA positivity in one case and anticardiolipin
antibody positivity in another. No rheumatologic disease
was determined during follow-up in any of the cases.
ere are three main imaging patterns of COP: the
most prevalent is the alveolar opacities, followed by
solitary opacities and inltrative opacities. Migratory
bilateral patchy alveolar inltrates are the most typical
imaging patterns of COP (2,8,13). Alveolar opacities were
reported with a prevalence of 69% to 82% (10,12,16).
Drakopanagiotakis et al. determined consolidation in 82%
of their cases and observed migrating inltrates in 11.5%
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NİKSARLIOĞLU et al. / Turk J Med Sci
of them (12). ere are also publications that suggest that
a reversed halo sign is especially typical of COP (18,19).
Similar to the previous studies, consolidation was observed
in 76.6% (n = 13) of our cases. In addition, consolidations
with millimetric nodules (11.8%) or ground glass opacities
(17.7%) were observed in some cases. However, migrating
inltrates were observed in only four patients. e
reversed halo sign was not observed in any patient. Lee
et al. evaluated radiological ndings in COP patients and
found that consolidation was present in 17 (77%) out of 22
cases, in agreement with the results of our patient group.
Moreover, 86% of the patients had ground glass opacities
and 32% had nodules (19). e second most prevalent
radiological pattern in COP is solitary opacities or mass
lesions. e diagnosis of such cases is made by OLB since
these lesions may be radiologically confused with alveolar
adenocarcinoma (7). Drakopanagiotakis et al. observed
mass lesions in 8.2% of COP cases (12). Among our cases, a
diagnosis of COP was made by OLB in four (23.5%) patients
who had a mass image on thoracic CT. ree patients with
mass lesions were evaluated by PET-CT; the FDG uptake
(SUVmax) was 7.5 and 10, respectively, in two cases. FDG
uptake was not determined in one of these cases. In the
literature, a study of COP patients with PET-CT reported
that the mean SUVmax value was 2.47 (20). Tateishi et al.
reported that SUVmax is higher in consolidations with an
air bronchogram as compared to those without, and that it
is correlated with disease activity (21). Two patients with
FDG uptake on PET-CT showed a complete radiological
regression with no sign of malignancy over the course of
a 23-month and 22-month follow-up period, respectively.
In some publications, it has been stated that 10%–
20% of patients might have unilateral or bilateral pleural
eusion (22–24). In our group, minimal pleural eusion
was detected in only one patient with a mass lesion. In
all probability, there will be radiological diversity as the
number of patients increases.
e most common PFT nding in COP patients was
a mild to moderate restrictive ventilatory pattern and
decreased diusion capacity (7,8). An obstructive pattern
may be observed in smokers. In our study, a restrictive
pattern was determined in ve patients, and an obstructive
pattern was determined in one patient who was a smoker.
Normal PFT was determined in the remaining patients.
Only three patients had DLCO test ndings with decreased
diusion capacity, which was consistent with the literature.
In our patient group, 15 patients had undergone
bronchoscopy, and infectious etiologies were excluded
based on the examination of the lavage uid. In our group,
diagnosis was made by OLB in 11 (64.7%) cases, by TBB
in 5 (29.4%), and by VATS in only one case. Contrary
to the present study, Oymak et al. diagnosed by TBB in
46% of their cases (3). Lazor et al. diagnosed COP by
OLB/VATS in 69% of 48 cases and by TBB in 31% (10).
Although the number of patients was dierent than ours,
the rate of diagnostic methods used was similar to that
used in our study. Patients with mass lesions had been
diagnosed by OLB since the provisional diagnosis also
included malignancy. Moreover, patients that could not
been diagnosed by TBB also underwent OLB.
Standard treatment of COP includes corticosteroids.
Regression in symptoms and radiological improvement
are usually observed over the course of days (7). Lee et al.
stated that response to corticosteroids is better in lesions
with an air bronchograms (25). In previous studies, the
starting daily dose of corticosteroid therapy was 0.75–1.5
mg/kg. Treatment was continued at those doses for 2–4
weeks and then discontinued at 6–12 months by gradually
decreasing the dose (7,8,10). In our study, 12 patients
(70.6%) received OCS: eight patients showed a complete
resolution, but three (18.7%) developed recurrence as
the dose of corticosteroid was decreased. Radiological
regression in patients that received OCS is consistent with
the literature (7,10). In this study, one patient showed
regression while undergoing clarithromycin therapy. In
that case, OCS was added to the treatment regimen as the
patient showed progression during follow-up. Treatment
with dierent agents such as macrolides has become a
current issue in the presence of steroid-related adverse
eects or where steroids are contraindicated due to various
reasons. e antiinammatory activity of macrolides has
been known for a long time. eir usage in organizing
pneumonia is based on this activity (26–28). However,
publications on this topic have usually been in the form
of case reports.
Although COP is generally a disease with good
prognosis, recurrence might develop while decreasing
the dose or stopping treatment in patients receiving
corticosteroids. In the literature, recurrence rates vary
between 9% and 58% (10,29). In our study, the recurrence
rate was 18.7%. e dierence between the recurrence
rates found in studies might have resulted from the
dierence in the frequency and the duration of follow-
up. In addition, the recurrence rates of both COP and
secondary organizing pneumonia have been reported
together in some publications. Nevertheless, a delay in
diagnosis and treatment increases the rate of recurrence,
as was reported by Lazor et al. (10). Moreover, elevated
gamma-glutamyl transferase, alanine aminotransferase,
and alkaline phosphatase enzyme concentrations were
detected in cases with multiple recurrences (10). In the
present study, the concentrations of these enzymes were
normal in the three patients with recurrence. In a recent
study, recurrence rates were found to have increased in
the three event zones (upper, intermediate, lower) of the
lungs involved (30). In our study, such an involvement
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NİKSARLIOĞLU et al. / Turk J Med Sci
was not observed in the patients that showed recurrence.
In Nishino et al.s study, patients with high brin in
pathologic specimens were associated with COP relapse
(6). We retrospectively evaluated pathologic specimens
and only one patient had brin in biopsy. No relapse was
determined in this patient.
e most important limitations of the present study are
its retrospective nature and the limited number of cases.
In conclusion, COP was more prevalent in middle-
aged female patients in our study group, while cough and
dyspnea on exertion were the most common symptoms
in these cases. Analogous with the previous studies,
consolidation is the predominating radiological pattern.
Diagnosis can be reached by performing OLB or TBB. e
recurrence rate of COP is relatively low.
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30. Izumi T, Kitaichi M, Nishimura K, Nagai S. Bronchiolitis
obliterans organizing pneumonia: clinical features and
dierential diagnosis. Chest 1992; 102: 715-719.
... In our study, the mean age of onset was 58 years, and patients were aged between 25 and 84 years; a predominance in women (68%) was observed. These data are in line with the study of Niksarlioglu et al. [38] and the study of Lee et al. [39]. COP is usually a no smoke-related disease; our results confirm this data, as seen also in Niksarlioglu's and Lee's studies, with only five patients currently or ex-smokers (80.8% non-smokers). ...
... In comparison to Sveinsson's study of 2007 [40], where fever was present in 50% of patients, in our study, only 13.6% of patients complained this symptom. In Niksarlioglu et al.'s study, cough was the most common symptom (88.2% of patients), while 54.5% of our patients experienced cough (in the majority of case it was dry cough) [38]. ...
... The most common abnormalities consisted of restrictive ventilator defect at spirometry and reduced DLCO [13]; these reports agree with our data. Niksarlioglu et al. have described that "an obstructive pattern may be observed in smokers ( . . . ) and an obstructive pattern was determined in one patient who was a smoker" [38]; however, in our study, we did not observe any obstructive pattern. ...
Article
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To evaluate the radiological findings in patients with cryptogenic organizing pneumonia (COP) before steroid treatment and their behavior after therapy, we retrospectively evaluated a total of 22 patients with a diagnosis of COP made by bronchoalveolar lavage (BAL), biopsy or clinical/radiological features, and the patients were followed between 2014 and 2018 at the hospital; the demographic data, symptoms, radiologic findings, diagnostic methods and treatment plans of patients were collected from patients’ hospital records. At least two CT scans of 22 patients (16 female and six men) were evaluated, the first one before starting steroid therapy and the others after therapy. At baseline CT scans, the most common radiological finding was the presence of consolidations (18/22 patients, 81.8%); ground-glass opacities were also very common (15/25, 68.1%). The other findings were as follows: nodules and masses (5/22, 22.7%), atoll sign (4/22, 18.1%), perilobular pattern (3/22, 13.6%) and parenchymal bands (3/22, 13.6%). Two patients had a significant relapse after reducing/interrupting therapy, while three had a complete resolution and are not currently under therapy (maintenance of clinical remission with no oral corticosteroid (OCS)). In High-resolution computed tomography (HRCT) scans after therapy, consolidations were still observable in seven patients (five in new areas of the lung-migratory infiltrates), while most of them disappeared, leaving a residual area of ground glass opacity in two patients. One patient had a residual of the perilobular pattern, with the disappearing of the other findings (consolidations and ground-glass opacities). Two patients developed a fibrosing pattern despite the therapy (9.5%). Cryptogenic organizing pneumonia tends to respond to oral corticosteroid treatment, but some patients may have a null or partial response. We highlight the behavior of this disease after proper therapy.
... The exact aetiology of this inflammatory reaction remains unclear [11]. OP does not exhibit a sex predilection and is more common in older people, with a mean age of onset of 50-60 years [12]. Rare cases have been reported in children. ...
... The most typical imaging pattern of OP is a migratory bilateral patchy alveolar infiltrate [14,18,19]. Niksarlıoğlu et al. reported that consolidation was observed in 76.6% of their cases [12]. Maimon et al. found that consolidation was present in 77% of their cases, 86% had ground glass opacities (GGO), and 32% had nodules [20]. ...
... Relapses are common upon corticosteroid dose reduction or treatment suspension, thus often leading to prolonged treatment [26][27][28]. Recurrence rates vary between 9 and 58% [10,12,29]. However, long-term corticosteroid treatment often results in significant side-effects, such as gastrointestinal bleeding, bone fracture, diabetes mellitus, arterial hypertension, upper respiratory tract infections, urinary tract infections and body weight increase [30,31]. ...
Article
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Background: Organizing pneumonia (OP) is a rare disease that is often easily misdiagnosed as a malignancy. The diagnosis of OP can prove quite challenging. Patients typically receive treatment with high-dose corticosteroids. Relapse is common if corticosteroid treatment is reduced or stopped. However, given that long-term corticosteroid treatment often results in significant side-effects, the aim of this study was to discuss the diagnosis and surgical treatment of OP. Material and methods: The medical records of 24 patients with pathologically diagnosed OP between October 2007 and January 2019 were retrospectively reviewed. All patients underwent thoracic computed tomography (CT) and transbronchial biopsy or CT-guided percutaneous needle aspiration. We analysed the clinical manifestations, radiological findings, diagnostic methods, treatment, and follow-up outcomes of all patients. Results: In total, 24 patients with OP were identified. The study included 17 (70.8%) men and 7 (29.2%) women, and the mean age was 61.25 ± 11.33 years (range: 31-82). The most common symptom was cough (n = 16; 66.6%), and the most common radiological finding was consolidation (n = 13; 54.2%) on thoracic CT. The diagnosis of OP was made by transbronchial biopsy in 11 patients (45.8%), and percutaneous needle aspiration biopsy in 13 (54.2%). We performed 11 wedge resections, 9 segmentectomy, and 4 lobectomies. Twenty patients underwent video-assisted thoracoscopic surgery (VATS), and 4 underwent thoracotomy. Complete lesion resection was obtained in all patients, and all patients were discharged from the hospital between 5 and 11 days after surgery. The mean follow-up period was 59.1 ± 34.5 (range: 2-134) months. Residual lesions or local or distant recurrence were not observed. Conclusions: OP is a rare disease, and the exact aetiology remains unclear. Preoperative diagnosis is difficult to achieve despite the use of transbronchial biopsy or CT-guided percutaneous needle aspiration. Complete surgical resection represents an effective method for the treatment of OP.
... 3 Niksarlioglu et al. found 76.6% of organizing pneumonia as consolidation. 5 Maimon et al. noted consolidation in 77%, ground-glass opacities in 86%, and nodules in 32% of organizing pneumonia. 6 In this report, Case one presented with multiple bilateral nodules in which few were cavitating whereas the second case presented as multifocal patchy air space opacities in the form of conglomerate nodules in which few nodules were cavitating throughout bilateral lung fields which is not specific to organizing pneumonia. ...
... In the case of rapidly progressing disease high dose glucocorticoid therapy is recommended (I.V methylprednisolone 500-1000mg for 3-5 days). 5 There was no consensus regarding the duration of treatment and it is based on the clinical and radiological assessment, however, there were reports which showed relapse during tapering or after stopping the steroids. 8 Typical COP has an excellent prognosis following steroid treatment but determining the prognosis of organizing pneumonia due to secondary cause is more difficult because of the diversity of reported cases. ...
Article
Full-text available
Organizing pneumonia is an interstitial lung disease that affects the distal bronchiole, respiratory bronchiole, alveolar ducts, and walls. To diagnose cryptogenic organising pneumonia, other aetiologies, such as inflammatory infections, connective tissue disease, drug responses, pulmonary infarction, and organ transplantation need to be ruled out. Radiological and histological progress in this disease will help to understand the disease in a better way. Early diagnosis of organizing pneumonia is important because of a good prognosis if it is treated earlier. But atypical clinical and radiological presentation will lead to difficulty in diagnosis and delay in treatment. Here we report two atypical presentations of organizing pneumonia cases to highlight the importance of upfront aggressive multimodality diagnostic approaches to rule out rare causes of cavitating lesions.
... 236 While no RCTs have evaluated the utility of CSs in the treatment of COP, several case series have supported the use of CSs as effective treatment in controlling disease activity. [237][238][239][240][241][242][243] From pooled data comprising of 12 case series and approximately 160 patients with histologically confirmed COP, treatment with CSs was associated with a complete response (generally with resolution of presenting symptoms and pulmonary opacities without leaving significant physiologic or imaging sequalae) in 59.4% of patients while a partial response was noted in 26.9%. Of the remaining 20%, only 6% had a fatal outcome. ...
Article
Full-text available
Each year, hundreds of millions of individuals are affected by respiratory disease leading to approximately 4 million deaths. Most respiratory pathologies involve substantially dysregulated immune processes that either fail to resolve the underlying process or actively exacerbate the disease. Therefore, clinicians have long considered immune-modulating corticosteroids (CSs), particularly glucocorticoids (GCs), as a critical tool for management of a wide spectrum of respiratory conditions. However, the complex interplay between effectiveness, risks and side effects can lead to different results, depending on the disease in consideration. In this comprehensive review, we present a summary of the bench and the bedside evidence regarding GC treatment in a spectrum of respiratory illnesses. We first describe here the experimental evidence of GC effects in the distal airways and/or parenchyma, both in vitro and in disease-specific animal studies, then we evaluate the recent clinical evidence regarding GC treatment in over 20 respiratory pathologies. Overall, CS remain a critical tool in the management of respiratory illness, but their benefits are dependent on the underlying pathology and should be weighed against patient-specific risks.
... This pattern has infiltration of the distal bronchioles, respiratory bronchioles, and alveoli by polypoid granulation inflammatory tissue [25] and is characterized by areas of consolidation predominantly in the peripheral, subpleural and/or peri-bronchial distribution on imaging. Nodules may also be noted in a similar distribution. ...
Article
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Chemotherapy while revolutionizing cancer management by improving survival and quality of life; is also associated with several adverse effects. Lung is the most common organ affected in chemotherapy-related complications, due to either drug toxicity or more commonly due to infections caused by immunosuppression and less commonly due to immune-mediated injury. Radiology, when used in combination with clinical and lab data, can help reach the specific diagnosis or narrow down the differentials. The common radiological patterns of drug toxicity include pulmonary interstitial and airway infiltrates, diffuse alveolar damage, nonspecific interstitial pneumonia, eosinophilic pneumonia, cryptogenic organizing pneumonia, pulmonary hemorrhage, edema and hypertension. Cancer patients are immunosuppressed due to the underlying malignancy itself or due to therapy and are prone to a gamut of opportunistic infections including viral, bacterial, fungal and mycobacterial pathogens. Immune reconstitution inflammatory syndrome (IRIS), a well-known complication in HIV, is now being increasingly recognized in non-HIV patients with immunosuppression. Engraftment syndrome is specifically seen following hematopoietic stem cell transplant during neutrophil recovery phase. Pulmonary involvement is frequent, causing a radiological picture of noncardiogenic pulmonary edema. Thus, radiology in combination with clinical background and lab parameters helps in detecting and differentiating various causes of pulmonary complications. This approach can help alter potentially toxic treatment and initiate early treatment depending on the diagnosis.
... Its onset is usually subacute or acute. Symptoms typically comprise respiratory complaints, including cough and dyspnoea (particularly with exertion), and are frequently accompanied by fever, fatigue, weight loss and other systemic symptoms (16). Physical examination may at times reveal wet rales on auscultation of the lungs. ...
Article
Full-text available
A 49-year-old female patient developed chest tightness and shortness of breath without apparent cause and presented to a local hospital. Chest radiography indicated increased thickening of the lung texture, increased multiple patchy densities in the lower lobes of the bilateral lungs and a slightly enlarged thyroid. The patient was treated for pulmonary infection with antibiotics but the symptoms persisted. A repeated CT scan revealed ground-glass attenuation of the bilateral lungs with multiple flaky exudates and visible bronchogenic signs. The symptoms did not improve after broadening anti-microbial coverage. Bronchoscopy and biopsy confirmed cryptogenic organizing pneumonia (COP). Thyroid ultrasound and thyroid function tests jointly confirmed the diagnosis of Hashimoto's thyroiditis (HT). After receiving corticosteroid treatment, the patient's condition improved and she was discharged. This case demonstrates that COP may present in combination with Hashimoto's thyroiditis (HT) and may possibly even be caused by HT. Early confirmation of diagnosis and treatment will help to improve the prognosis of such patients.
Article
Objective The organizing pneumonia (OP) pattern is the second most common finding in anti-synthase antibody syndrome (ASS), and nonspecific interstitial pneumonia (NSIP) is the most common finding. This study analysed the OP score changes by semiquantitative and quantitative analysis methods and the correlation between the high-resolution computed tomography (HRCT) indexes and the pulmonary function test parameters (PFTs) in ASS patients. Methods Data from ASS-OP patients admitted to the respiratory department of Ping Jin Hospital from October 2014 to June 2020 were retrospectively reviewed and analysed. Results Fourteen ASS-OP patients were recruited for this study. (1) In method-1, the consolidation (CO) score and the mean lung attenuation (MA) of poorly aerated and fibrosis lung fields (MAfibrosis) (r = 0.56, P = 0.04), the ground-glass opacity (GGO) score and the MA of non-aerated lung fields (MAnonaerated) (r = −0.64, P = 0.01), and the CO plus the GGO (CG) score and the MAnonaerated (r = −0.59, P = 0.03) of the lung fields had liner correlations. In method-2, the GGO score to the MAnonaerated (r = −0.58, P = 0.03), and the CG (r = −0.68, P = 0.01) score to the MAnonaerated had liner correlations. The FVC% (r = 0.68, P = 0.01) and FEV1% (r = 0.64, P = 0.01) to the MAfibrosis had good linear correlations. (2) Compared to the values before treatment, the CO pattern score, volume and weight percentages of the extracted whole lung volume with attenuation values of the nonaerated area (Vnonated%, Wnonaerated%), the volume of poorly aerated and fibrosis lung tissue (Vfibrosis%, Wfibrosis%), the weight percentages of normal aerated lung (Wnormal%), and the MAfibrosis exhibited significant differences during the 3–6 month follow-up period. Conclusion The GGO and CO scored by the semiquantitative or quantitative analysis methods was similar. The HRCT quantitative analysis parameters showed a good correlation with the PFTs in ASS-OP patients, can provide an accurate OP pattern interpretation, and may be used as a monitoring and therapeutic indicator for ASS-OP patients.
Article
Objective The organizing pneumonia (OP) pattern is the second most common finding in anti-synthase antibody syndrome (ASS), and nonspecific interstitial pneumonia (NSIP) is the most common finding. This study analysed the OP score changes by semiquantitative and quantitative analysis methods and the correlation between the high-resolution computed tomography (HRCT) indexes and the pulmonary function test parameters (PFTs) in ASS patients. Methods Data from ASS-OP patients admitted to the respiratory department of Ping Jin Hospital from October 2014 to June 2020 were retrospectively reviewed and analysed. Results Fourteen ASS-OP patients were recruited for this study. (1) In method-1, the consolidation (CO) score and the mean lung attenuation (MA) of poorly aerated and fibrosis lung fields (MAfibrosis) (r = 0.56, P = 0.04), the ground-glass opacity (GGO) score and the MA of non-aerated lung fields (MAnonaerated) (r = −0.64, P = 0.01), and the CO plus the GGO (CG) score and the MAnonaerated (r = −0.59, P = 0.03) of the lung fields had liner correlations. In method-2, the GGO score to the MAnonaerated (r = −0.58, P = 0.03), and the CG (r = −0.68, P = 0.01) score to the MAnonaerated had liner correlations. The FVC% (r = 0.68, P = 0.01) and FEV1% (r = 0.64, P = 0.01) to the MAfibrosis had good linear correlations. (2) Compared to the values before treatment, the CO pattern score, volume and weight percentages of the extracted whole lung volume with attenuation values of the nonaerated area (Vnonated%, Wnonaerated%), the volume of poorly aerated and fibrosis lung tissue (Vfibrosis%, Wfibrosis%), the weight percentages of normal aerated lung (Wnormal%), and the MAfibrosis exhibited significant differences during the 3–6 month follow-up period. Conclusion The GGO and CO scored by the semiquantitative or quantitative analysis methods was similar. The HRCT quantitative analysis parameters showed a good correlation with the PFTs in ASS-OP patients, can provide an accurate OP pattern interpretation, and may be used as a monitoring and therapeutic indicator for ASS-OP patients.
Article
Full-text available
Idiopathic FOP is a rare type of COP. What we know on this subject is made up of a few clinical cases published in recent years. Our patient was admitted to the hospital with an intermittent coughing complaint that worsens over time. Due to a suspicion of malignancy, a radiological evaluation was requested including a PET-CT and a transbronchial biopsy was performed. Until the last part of our algorithm, the patient profile was clinically and radiologically in favor of the diagnosis of malignancy but, in the end, the diagnosis of FOP was fixed with a follow-up decision. In conclusion, FOP is a relatively new entity that should be kept in mind in the differential diagnosis of malignancy.
Article
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Cryptogenic organizing pneumonia is an idiopathic form of organizing pneumonia (also known as bronchiolitis obliterans organizing pneumonia). Because cryptogenic organizing pneumonia is considered an inflammatory disease, it characteristically responds to the broad-spectrum antiinflammatory corticosteroids, although relapse is common on discontinued use. Additionally, long-term use of corticosteroids has many side effects. In severe cases in which corticosteroids have failed, either cytotoxic therapy or macrolide therapy is used. Because of the toxicity and adverse effects of cytotoxic therapy (e.g., cyclophosphamide), this therapy option cannot be used long term in refractory cases. Macrolide therapy has been shown to be an effective antiinflammatory agent that is relatively safe when used on a long-term basis in patients with cryptogenic organizing pneumonia.
Article
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Although the overall prognosis of CTD-related interstitial pneumonia is better than that of idiopathic interstitial pneumonia, the prognosis of CTD-related organizing pneumonia (CTD-OP) was suggested to be worse than that of cryptogenic organizing pneumonia (COP). The aim of this study was to compare the clinical features and outcome of the two conditions. A retrospective review of 100 patients diagnosed by lung biopsy as having organizing pneumonia patterns (CTD, 24; COP, 76) at three tertiary referral centres. Underlying CTDs were mostly RA, SS and PM/DM. The median follow-up period was 43.6 months. There were no differences in initial symptoms, lung function or bronchoalveolar lavage fluid findings except significantly more females (83.3 vs. 59.2%, P = 0.048) in the CTD-OP than in the COP group. Over 80% of the patients in both the groups improved. However, complete recovery rate was lower in CTD-OP (20.8%) than in COP (46.1%; P = 0.028) with a tendency towards higher recurrence rate in CTD-OP (40.0 vs 20.3%; P = 0.072). There was no significant difference in the frequency of rapid progression or overall survival between the two groups. The clinical features and prognosis of CTD-OP are similar to COP. However, lower complete recovery rate with a tendency towards higher recurrence rate in CTD-OP compared with COP suggest the need for closer follow-up in patients with CTD-OP.
Article
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A 47-yr-old female was referred to a tertiary centre for further evaluation of chronic cough, fever, progressive exertional dyspnoea and fatigue. From a respiratory point of view she had been well until 7 months previously when she had started to experience systemic fatigue, low-grade fever and chronic dry cough. A computed tomography scan of the chest demonstrated diffuse bilateral ground-glass, ill-defined pulmonary opacities affecting all lobes. Some had central ground-glass hazy density with peripheral areas of increased opacity, which is consistent with "reversed halo sign". Cryptogenic organising pneumonia (COP) is a clinical, radiological and pathological diagnosis which is made when no definite cause, such as infection or connective tissue disease, is found. It is characterised histopathologically by the presence of patchy distribution of granulation tissue, which consists of fibroblasts and myofibroblasts embedded in a loose connective matrix, present in the lumen of the distal airspaces including alveoli, alveolar ducts and bronchioles. This case report illustrates the association of the reversed halo sign with COP. Although only seen in one fifth of patients with the disease, it appears to suggest the diagnosis of COP and, with proper clinical correlation, it may be another diagnostic adjunct.
Article
Full-text available
Bronchiolitis obliterans organizing pneumonia (BOOP) was first described in the early 1980s as a clinicopathologic syndrome characterized symptomatically by subacute or chronic respiratory illness and histopathologically by the presence of granulation tissue in the bronchiolar lumen, alveolar ducts and some alveoli, associated<sup> </sup> with a variable degree of interstitial and airspace infiltration<sup> </sup> by mononuclear cells and foamy macrophages. Persons of all ages can be affected. Dry cough and shortness of breath of 2 weeks to 2 months in duration usually characterizes BOOP. Symptoms persist despite antibiotic therapy. On imaging, air space consolidation can be indistinguishable from chronic eosinophilic pneumonia (CEP), interstitial pneumonitis (acute, nonspecific and usual interstitial pneumonitis, neoplasm, inflammation and infection). The definitive diagnosis is achieved by tissue biopsy. Patients with BOOP respond favorably to treatment with steroids.
Article
Organizing pneumonia (formerly called bronchiolitis obliterans with organizing pneumonia) is an uncommon type of inflammatory and fibroproliferative lung disorder. Its idiopathic and most frequent form is termed cryptogenic organizing pneumonia. Organizing pneumonia can also be secondary to a variety of causes and particular clinical contexts, which must be carefully ruled out before accepting the diagnosis of cryptogenic organizing pneumonia. Clinically, the disease has usually a subacute onset with cough, dyspnea, fever, fatigue, weight loss, crackles at chest auscultation, and elevated inflammatory biologic markers. Bronchoalveolar lavage typically reveals a mixed pattern alveolitis with increased lymphocytes, neutrophils, and eosinophils. With chest imaging, the most typical features consist of multiple patchy, possibly migratory, alveolar opacities predominating in the subpleural areas. Lung biopsy discloses buds of granulation tissue filling distal airspaces. Response to corticosteroid treatment is usually excellent within days to weeks. Relapses are frequent but can be controlled under moderate doses of prednisone and do not alter the final prognosis.
Article
In 2002 the American Thoracic Society/European Respiratory Society (ATS/ERS) classification of idiopathic interstitial pneumonias (IIPs) defined seven specific entities, and provided standardized terminology and diagnostic criteria. In addition, the historical "gold standard" of histologic diagnosis was replaced by a multidisciplinary approach. Since 2002 many publications have provided new information about IIPs. PURPOSE: The objective of this statement is to update the 2002 ATS/ERS classification of IIPs. METHODS: An international multidisciplinary panel was formed and developed key questions that were addressed through a review of the literature published between 2000 and 2011. RESULTS: Substantial progress has been made in IIPs since the previous classification. Nonspecific interstitial pneumonia is now better defined. Respiratory bronchiolitis-interstitial lung disease is now commonly diagnosed without surgical biopsy. The clinical course of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia is recognized to be heterogeneous. Acute exacerbation of IIPs is now well defined. A substantial percentage of patients with IIP are difficult to classify, often due to mixed patterns of lung injury. A classification based on observed disease behavior is proposed for patients who are difficult to classify or for entities with heterogeneity in clinical course. A group of rare entities, including pleuroparenchymal fibroelastosis and rare histologic patterns, is introduced. The rapidly evolving field of molecular markers is reviewed with the intent of promoting additional investigations that may help in determining diagnosis, and potentially prognosis and treatment. CONCLUSIONS: This update is a supplement to the previous 2002 IIP classification document. It outlines advances in the past decade and potential areas for future investigation.
Article
Organizing pneumonia (OP) is a histopathologic pattern of response to lung injury. Fibrin is a marker of acute microvascular injury, and variable amounts of intraalveolar fibrin are seen in OP; however, its relevance to clinical outcomes is unclear. We examined lung wedge biopsies of 26 patients with cryptogenic organizing pneumonia (COP), assessed the amount of fibrin associated with airspace organization, and correlated fibrin levels with other histologic, clinical, and radiographic findings. Seven patients with COP had disease relapse. Patients with multifocal fibrin deposits or acute fibrinous and organizing pneumonia (collectively, "high fibrin") showed a higher rate of OP relapse compared to those with no or focal fibrin (60% versus 6%, P < .05). Patients with radiographic evidence of disease involving all three lung zones (upper, middle, and lower) also showed higher rates of relapse compared to those in whom disease was limited to one or two zones (41% versus 0%, P = .055). In patients with both pathologic evidence of high fibrin and radiographic evidence of three-zone disease, OP relapse could be predicted with a sensitivity of 86% and specificity of 84% (positive predictive value of 67% and negative predictive value of 94%). The presence of high levels of intraalveolar fibrin in lung biopsies and radiographic evidence of disease involving all three lung zones is associated with increased risk of relapse in patients with COP, and these features may help identify patients who may benefit from more intensive steroid therapy.
Article
We conducted a review of serial high-resolution CT (HRCT) findings of cryptogenic organizing pneumonia (COP). Over the course of 14 years, we saw 32 patients with biopsy-confirmed COP. Serial HRCT scans were available for only 22 patients (seven men and 15 women; mean age, 52 years; median follow-up period, 8 months; range, 5-135 months). Serial CT scans were evaluated by two chest radiologists who reached a conclusion by consensus. Overall changes in disease extent were classified as cured, improved (i.e., ≥ 10% decrease in extent), not changed, or progressed (i.e., ≥ 10% increase in extent). When there were remaining abnormalities, the final follow-up CT images were analyzed to express observers' ideas regarding what type of interstitial lung disease the images most likely suggested. The two most common patterns of lung abnormality on initial scans were ground-glass opacification (86% of patients [19/22]) and consolidation (77% of patients [17/22]), distributed along the bronchovascular bundles or subpleural lungs in 13 patients (59%). In six patients (27%), the disease disappeared completely; in 15 patients (68%), the disease was decreased in extent; and in one patient (5%), no change in extent was detected on follow-up CT. When lesions remained, the final follow-up CT findings were reminiscent of fibrotic nonspecific interstitial pneumonia in 10 of 16 patients (63%). Although COP is a disease with a generally good prognosis, most patients (73%) with COP have some remaining disease seen on follow-up CT scans, and, in such cases, the lesions generally resemble a fibrotic nonspecific interstitial pneumonia pattern.
Article
Organizing pneumonia (OP) is a distinct clinical and pathologic entity. This condition can be cryptogenic (COP) or secondary to other known causes (secondary OP). In the present study, we reviewed the features associated with COP and secondary OP in patients from two teaching hospitals. The medical records of 61 patients with biopsy-proven OP were retrospectively reviewed. Forty patients were diagnosed with COP and 21 patients with secondary OP. The clinical presentation, radiographic studies, pulmonary function tests (PFTs), laboratory data, BAL findings, treatment, and outcome were analyzed. The mean age at presentation was 60.46 ± 13.57 years. Malaise, cough, fever, dyspnea, bilateral alveolar infiltrates, and a restrictive pattern were the most common symptoms and findings. BAL lymphocytosis was observed in 43.8% of patients with OP. The relapse rate and mortality rate after 1 year of follow-up were 37.8% and 9.4%, respectively. The in-hospital mortality was 5.7%. The clinical presentation and radiographic findings did not differ significantly between patients with COP and secondary OP. A mixed PFT pattern (obstructive and restrictive physiology) and lower blood levels of serum sodium, serum potassium, platelets, albumin, protein, and pH were observed among patients with secondary OP. Higher blood levels of creatinine, bilirubin, Paco₂, and BAL lymphocytes were also more common among patients with secondary OP. There were no differences in the relapse rate or mortality between patients with COP and secondary OP. The 1-year mortality correlated with an elevated erythrocyte sedimentation rate, low albumin, and low hemoglobin levels. The clinical and radiographic findings in patients with COP and secondary OP are similar and nonspecific. Although certain laboratory abnormalities are more common in secondary OP and can be associated with worse prognosis, they are likely due to the underlying disease. COP and secondary OP have similar treatment response, relapse rates, and mortality.