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Churg-Strauss syndrome (CSS) is a rare systemic necrotizing small-vessel vasculitis, with accompanying bronchial asthma, eosinophilia, and eosinophilic infiltration of various tissues. The purposes of our study were to characterize the clinical features of CSS and to identify factors associated with CSS prognosis in Koreans.
Medical records were re...
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Eosinophilic granulomatosis with polyangiitis, formerly Churg-Strauss Syndrome, is an uncommon disorder that carries a high mortality when coronary artery disease develops. Early recognition and treatment is crucial. We highlight an unusual presentation of acute coronary syndrome not associated with atherosclerotic coronary disease. (Level of Diffi...
Churg-Strauss syndrome (CSS) is a rare cause of vasculitic neuropathy. Although rare and potentially fatal, Churg-Strauss syndrome (CSS) is easily diagnosable and treatable. The presence of bronchial asthma with peripheral neuropathy in a patient alerts a physician to this diagnosis. This is vividly illustrated by the presented two cases who had ne...
Context: Churg-Strauss syndrome (CSS) is a rare antineutrophil
cytoplasmic antibody associated vasculitis characterized by
asthma, chronic rhinosinusitis, and persistent eosinophilia.
Although acute diarrhea is frequent clinical manifestations of
parasitic infection, it is not a main clinical presentation of CSS.
We report a 49-year-old man who pre...
A 58-year-old male presented with sensory motor polyneuropathy and rapidly progressive renal failure. Investigations revealed marked peripheral eosinophilia and elevated perinuclear antineutrophil cytoplasmic antibody titers. Renal biopsy showed pauci-immune cre-scentic glomerulonephritis with interstitial eosinophil infiltrates. He had no history...
Backgraund/Aim. Primary anti-neutrophil cytoplasmatic antibody (ANCA)-associated vasculitis are chronic multisystemic autoimmune diseases which include microscopic polyangitis (MPA), granulomatosis with polyangitis (WG), eosinophilic granulomatosis with polyangitis (EPGA; churg-strauss syndrome ? CSS), and also a localized forms of ill-ness. In our...
Citations
... С учётом лёгочных инфильтратов в анамнезе, преходящей эозинофилии, рецидивирующего синусита, аллергического ринита как аналога бронхиальной астмы на начальном этапе заболевания был выставлен диагноз «эгпа». в литературе имеются сведения о том, что бронхиальная астма не всегда возникает в дебюте заболевания [2]. но в течение года с момента манифестации заболевания не было зафиксировано никаких проявлений бронхообструктивного синдрома: приступы экспираторной одышки отсутствовали, при спирографии отмечались незначительное снижение офв1 и отрицательный результат бронходилатационного теста. ...
The article presents a prospective clinical observation with a fatal outcome in a patient with severe granulomatosis with polyangiitis (GPA) complicated by COVID 19 on the background of immunosuppression. At the onset of the disease, there were difficulties in making a diagnosis; differential diagnosis between eosinophilic granulomatosis with polyangiitis (EGPA) and GPA was carried out. Against the background of combined therapy — the use of high and ultra-high doses of glucocorticoids (GC) and cytostatic drugs, a short-term improvement was noted. However, within a year and a half of treatment, it was not possible to achieve remission of the disease. Taking into account the ineffectiveness of the therapy, by the decision of the medical commission, the patient was prescribed genetically engineered biological therapy rituximab (RTM). There was a decrease in the activity of the disease, the achievement of depletion of B-cells. During the pandemic period, despite observing the isolation regime, the patient fell ill with a coronavirus infection. Immunosuppression contributed to severe infection. After the infection was treated, the activity of vasculitis increased, which required the repeated administration of RTM for health reasons. During the therapy it was short-term stabilization of the condition. But after short period — fever, an increase in respiratory failure, the development of neutropenia. Taking into account the initial lesion of the lungs in the patient, differential diagnostics was carried out between the complications of coronavirus infection and the activity of the underlying disease. Despite anti-inflammatory, anticoagulant, antibacterial therapy, the patient's condition progressively worsened, respiratory failure increased, hemoptysis appeared. Spontaneous pneumothorax on the right was diagnosed, pneumomediastinum. After repeated negative PCR results, the causative agent of SARS COV-2 was again detected in smears. The immediate cause of death, according to clinical observation and autopsy, was severe respiratory failure, thrombosis in small vessels of the lungs in a patient with a new coronavirus infection against the background of immunosuppression for GPA.
... Rhinitis has been reported in 15-75% of total cases, occurring before, or at the onset of asthma. Chronic sinusitis has been reported in 60-80% of cases, and PNS abnormality is accompanied in 75% [1,[3][4][5][6][7][8][9][10]. As for otologic manifestations, hearing loss and middle ear effusion (MEE) are the most common presentation [11]. ...
Background
Ear, nose, and throat involvement are common in eosinophilic granulomatosis with polyangiitis (EGPA). Among otologic manifestation, middle ear effusion (MEE) is less recognized but a problematic condition as it may progress to hearing impairment when left untreated. This study aimed to evaluate the characteristics, risk factors and clinical outcomes of MEE in EGPA patients.
Methods
This is a case–control study of patients who were diagnosed and treated for EGPA from January 1995 to November 2018. Patients with ear symptoms (ear fullness, ear discharge, tinnitus or hearing loss) were assessed by otologists and were included in the case group (n = 23) if clinically relevant. The other patients without MEE were included in the control group (n = 52). Risk of MEE was calculated using the Cox proportional-hazard model.
Results
During median follow-up of 9.9 years, 23 (30.7%) out of 75 patients had MEE. In MEE group, 12 (52.2%) patients had hearing loss; conductive type in 10 (10/12, 83.3%) and mixed type in two (2/12, 16.7%). In multivariable regression analysis, major organ involvement at diagnosis (adjusted hazard ratio [aHR] 65.4; 95% confidence interval [CI], 1.50—2838.39; P = 0.030] , early onset of ear symptom after systemic therapy (< 6 months) (aHR 40.0; 95% CI, 1.35—1183.43; P = 0.033) and continuing the maintenance steroid without cessation (aHR 8.59; 95% CI, 1.13—65.42; P = 0.038) were independently associated with a risk of MEE. To control MEE, 16 (69.6%) patients had to increase maintenance steroid dose and 9 (39.1%) patients experienced recurrent MEE whenever maintenance dose was tapered.
Conclusions
MEE is a common but frequently neglected condition in EGPA which is often intractable. The maintenance steroid dose should be adequately adjusted to control MEE and to prevent from progressive hearing loss. Novel biologic agents possibly have a role in controlling MEE in EGPA.
... However, in EGPA, up to 80% of the patients have persistent and/or "relapsing" asthma or sinus problems, thus remain steroiddependent. 32,114,115 Diagnosing and treating promptly relapses can be challenging, as there are no good predictive or diagnostic markers. Serial measurement of ANCA is by default still the "best" available predictive option but lacks both specificity and sensitivity. ...
Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic poly-angiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Renal-limited AAV can be considered a fourth entity. Despite their rarity and still unknown cause(s), research into AAV has been very active over the past decades and has allowed for the development of new therapeutic regimens. The pathogenesis is a complex process of immune dysregulations with genetic and environmental influences. Recent genome-wide association studies have identified multiple genetic predisposing variants, especially at the major histocompatibility complex region. The pathogenic role of antimyeloperoxidase ANCA (MPO-ANCA) is well supported by several animal models, but that of antiproteinase 3 ANCA (PR3-ANCA) is not as strongly demonstrated. B cells likely play a major role in the pathogenesis because they produce ANCAs, as do neutrophil abnormalities, imbalances in T-cell subtypes, and/or cytokine-chemokine networks. The role of the alternative complement pathway was established more recently, and studies of the antagonist of human C5a receptor (avacopan) in AAV have just been completed, with promising results. The current standard management of severe AAV still consists of remission induction therapy with glucocorticoids combined with rituximab or, less often now, cyclophosphamide. Several studies showed that reduced-dose regimens of glucocorticoids are noninferior to the previously used heavier regimens, for therefore less cumulative exposure to glu-cocorticoids. Avacopan use may even lead to new steroid-free therapeutic approaches, at least for some selected patients. Several trials and studies have now shown the superiority of rituximab over azathioprine or methotrexate as maintenance therapy. However, the optimal dosing regimen and duration for maintenance remain to be better defined, at the individual patient level. Many changes have occurred in the standard of care for AAV over the past decades, and more are expected soon, including with use of avacopan, but also, likely, a few other agents under investigation or development.
... The overall mortality rate was 4%. In previous European and Asian cohorts from 1957 to 2014 with a similar mean duration of follow-up, mortality rates were 7% to 14%, although this improved in most recent reports (10,12,(22)(23)(24)(25). Similar to the current study, most other studies also reported no difference in death rates based on ANCA status (8,9,19,20,23), although one study reported more deaths in ANCA-negative cohorts, presumably because of more frequent cardiac manifestations (10). ...
Objective
To describe clinical manifestations and outcomes in patients with eosinophilic granulomatosis with polyangiitis (EGPA) in North America.
Methods
Analysis of patients aged 18 years or older who fulfilled the 1990 American College of Rheumatology Classification Criteria for EGPA enrolled in the Vasculitis Clinical Research Consortium from 2003 to 2019. Main clinical characteristics, treatments, outcomes, and accumulated damage were studied.
Results
The cohort included 354 patients; 59% female; age at diagnosis of 50.0 (±14) years; 39% were antineutrophil cytoplasm antibody (ANCA) positive. Time from diagnosis to last follow-up was 7.0 (±6.2) years; 49.4% had one or more relapse. Patients positive for ANCA more commonly had neurological and kidney involvement when compared with patients negative for ANCA, who had more cardiac and lung manifestations. At last study visit, only 35 (12.6%) patients had been off all therapy for more than 2 years during their follow-up. The overall mortality rate was 4.0% and did not differ by ANCA status or cyclophosphamide use. Scores on the Vasculitis Damage Index (VDI) for 134 patients with two or more visits and more than 1 year of follow-up increased from 1.7 (±1.8) at enrollment (3.7 [±5.1] years after diagnosis) to 3.35 (±2.1) at last follow-up (7.5 [±5.8] years after diagnosis), mainly represented by chronic asthma (67.5%), peripheral neuropathy (49.6%), and chronic sinusitis (31.3%). Longer duration of glucocorticoid use and relapse were associated with higher VDI scores.
Conclusion
This analysis describes the many clinical manifestations and varied outcomes of EGPA and highlights the ongoing need to attain more sustained, long-term remission to limit the accrual of disease-related damage.
... However, a retrospective analysis from a single center in Korea showed that the respiratory tract (bronchial asthma) was the most commonly involved organ, and its involvement was associated with a more favorable outcome. 74 EGPA occurs in a minority of SA patients. However, EGPA should be considered when dealing with severe uncontrolled asthma as these patients require additional treatment, such as immunosuppressants and OCS. ...
Severe asthma (SA) presents in about 3%-5% of adult asthmatics and is responsible for over 60% of asthma-related medical expenses, posing a heavy socioeconomic burden. However, to date, a precise definition of or clear diagnostic criteria for SA have not been established, and therefore, it has been challenging for clinicians to diagnose and treat this disease. Currently, novel biologics targeting several molecules, such as immunoglobulin E, interleukin (IL)5, and IL4/IL13, have emerged, and many new drugs are under development. These have brought a paradigm shift in understanding the mechanism of SA and have also provided new treatment options. However, we need to agree on a precise definition of and its diagnostic criteria for SA. Additionally, it is necessary to explain the diagnostic criteria and to summarize current standard and additional treatment options. This review is an experts' opinion on SA from the Korean Academy of Asthma, Allergy, and Clinical Immunology, the Working Group on Severe Asthma, and aims to provide a definition of and diagnostic criteria for SA, and propose future direction for SA diagnosis and management in Korea.
... Remission for more than 6 months was more often observed in patients with older age, diagnosis in an earlier stage, pulmonary manifestations, generalized symptoms, and high C-reactive protein than those without. 33 We included 30 Korean patients with EGPA and investigated the initial predictors at diagnosis of relapse during follow-up. Respiratory symptoms were common clinical manifestations, such as asthma (86.7%) and lung parenchymal involvement (76.7%). ...
... In terms of Korean patients with EGPA, Kim, et al. 33 compared clinical features between ANCA-positive and negative patients. ANCA-positive EGPA patients showed a higher frequency of renal involvement than ANCA-negative EGPA patients. ...
Eosinophilic granulomatosis with polyangiitis (EGPA) is one form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Identical to what has been called Churg-Strauss syndrome, EGPA exhibits both allergic and vasculitis features. EGPA was first described as a syndrome consisting of asthma, fever, eosinophilia, and organ involvement including heart failure, neuropathy, and kidney damage, by Churg and Strauss in 1951. On the basis of the 2012 Chapel Hill Consensus Conferences Nomenclature of Vasculitis, EGPA comprises three typical allergic components, including asthma, peripheral eosinophilia, and eosinophil-rich granuloma of the respiratory tracts. EGPA has three clinical and histological stages. The first is an allergic stage composed of asthma and sinusitis, and the second is an eosinophilic stage characterised by peripheral hypereosinophilia and intra-organ infiltration of eosinophils. The last is a vasculitic stage, including necrotising inflammation of small vessels and end-organ damage. In this review, we describe the classification criteria for EGPA and recommendations for the evaluation and management of EGPA with conventional and newly suggested drugs for EGPA. Also, we discuss a variety of clinical aspects such as predictive values for prognosis and associations with other Th2-mediated diseases and hepatitis B virus.
... In terms of EGPA, Kim, et al. 41 analysed the clinical features and prognosis in Korean patients with EGPA. They divided EGPA patients according to either ANCA positivity or responses to treatment and compared organ involvements between the two groups. ...
Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a group of systemic necrotising vasculitides, which often involve small vessels, and which lead to few or no immune deposits in affected organs. According to clinical manifestations and pathological features, AAV is classified into three variants: microscopic polyangiitis, granulomatosis with polyangiitis (GPA), and eosinophilic GPA. The American College of Rheumatology 1990 criteria contributed to the classification of AAV, although currently the algorithm suggested by the European Medicines Agency in 2007 and the Chapel Hill Consensus Conference Nomenclature of Vasculitides proposed in 2012 have encouraged physicians to classify AAV patients properly. So far, there have been noticeable advancements in studies on the pathophysiology of AAV and the classification criteria for AAV in Western countries. However, studies analysing clinical features of Korean patients with AAV have only been conducted and reported since 2000. One year-, 5 year-, and 10 year-cumulative patient survival rates are reported as 96.1, 94.8, and 92.8%. Furthermore, initial vasculitis activity, prognostic factor score, age and specific organ-involvement have been found to be associated with either all-cause mortality or poor disease course. The rate of serious infection is 28.6%, and 1 year-, 5 year- and 10 year-cumulative hospitalised infection free survival rates range from 85.1% to 72.7%. The overall standardised incidence ratio of cancer in AAV patients was deemed 1.43 compared to the general Korean population.
... cases per million persons. 2 This syndrome is diagnosed by the presence of any four or more of the six criteria according to American college of Rheumatology including; bronchial asthma, peripheral blood eosinophilia greater than 10%, paranasal sinusitis, pulmonary infiltration, histologically confirmed vasculitis and neuropathy. 3 This case is presented because of the rarity and the need for its consideration in patients with difficult-to-treat bronchial asthma and multiple organ involvement. ...
... 8 She had florid respiratory symptoms and her c-ANCA and p-ANCA results were negative, similar to the findings of a Korean study with prominence of respiratory symptoms. 2 The commonest extra-pulmonary manifestations of this syndrome are neuropathy and vasculitis, which the index patient had. 9 Following diagnosis and initiation of therapy, she showed considerable clinical improvement following the commencement of oral steroids. This has been the mainstay of treatment and has seen transformation of the prognosis of the disease from near certain mortality to one which is now considered relatively favourable. ...
... Encouraging case finding and clinical diagnosis of the EGPA among treatment-resistant asthma patients may be the way to go for resource poor settings, as the incidence is relatively higher among asthmatics. 2 This distinct clinical presentation should be kept in mind, and the emphasis on pathologic evidence, which is not pathognomonic for diagnosis, de-emphasised. 7 ...
Eosinophilic granulomatosis with polyangitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a rare systemic vasculitis of unknown aetiology characterised by necrotising small-vessel vasculitis and eosinophil-rich granulomatous inflammation of tissues and vessels, associated with asthma and peripheral blood eosinophilia.1 We report the rare case of a 36-year- old lady with a one-year history of difficult-totreat
bronchial asthma with rhino-sinusitis, vasculitic skin lesions, symptoms of peripheral neuropathy, peripheral blood eosinophilia and chest x-ray showing hyperinflation with
pulmonary infiltrates. A diagnosis of EGPA was made and patient made significant improvement on therapy.
... cases per million persons. 2 This syndrome is diagnosed by the presence of any four or more of the six criteria according to American college of Rheumatology including; bronchial asthma, peripheral blood eosinophilia greater than 10%, paranasal sinusitis, pulmonary infiltration, histologically confirmed vasculitis and neuropathy. 3 This case is presented because of the rarity and the need for its consideration in patients with difficult-to-treat bronchial asthma and multiple organ involvement. ...
... 8 She had florid respiratory symptoms and her c-ANCA and p-ANCA results were negative, similar to the findings of a Korean study with prominence of respiratory symptoms. 2 The commonest extra-pulmonary manifestations of this syndrome are neuropathy and vasculitis, which the index patient had. 9 Following diagnosis and initiation of therapy, she showed considerable clinical improvement following the commencement of oral steroids. This has been the mainstay of treatment and has seen transformation of the prognosis of the disease from near certain mortality to one which is now considered relatively favourable. ...
... Encouraging case finding and clinical diagnosis of the EGPA among treatment-resistant asthma patients may be the way to go for resource poor settings, as the incidence is relatively higher among asthmatics. 2 This distinct clinical presentation should be kept in mind, and the emphasis on pathologic evidence, which is not pathognomonic for diagnosis, de-emphasised. 7 ...
Eosinophilic granulomatosis with polyangitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a rare systemic vasculitis of unknown aetiology characterised by necrotising small-vessel vasculitis and eosinophil-rich granulomatous inflammation of tissues and vessels, associated with asthma and peripheral blood eosinophilia.1 We report the rare case of a 36-year- old lady with a one-year history of difficult-totreat bronchial asthma with rhino-sinusitis, vasculitic skin lesions, symptoms of peripheral neuropathy, peripheral blood eosinophilia and chest x-ray showing hyperinflation with pulmonary infiltrates. A diagnosis of EGPA was made and patient made significant improvement on therapy.
... Moreover, the low rate of ANCA-positive patients with EGPA in another study conducted in Korea indicates that racial differences might exist. 17 A previous study observed an average interval of 10 years between the onset of asthma and the systemic symptoms of EGPA. 10 We saw a wide interval range of 0 to 53 years, with an average interval of 3.25 years (Fig. 1). The interval between the onset of asthma and EGPA was not associated with the severity of PN or the treatment response. ...
Background and Purpose
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small-vessel vasculitis accompanied by asthma, eosinophilia, and eosinophilic inflammation of various tissues including the peripheral nerves. This study investigated the clinical course and long-term outcomes of peripheral neuropathy in patients with EGPA.
Methods
Seventy-one patients with physician-diagnosed EGPA were identified at Samsung Medical Center between January 1995 and April 2014. Sixty-one of these patients were followed-up for more than 1 year and received corticosteroid therapy with or without intravenous cyclophosphamide pulse therapy for 6 to 18 months. Medical records of the 61 patients including demographic data, clinical features, laboratory and pathological findings, treatments, and outcomes were reviewed.
Results
Peripheral neuropathy as a manifestation of EGPA was present in 46 (75%) of the 61 patients. The mean follow-up duration of the patients with neuropathy was 6.4 years (range 1.2–18.8 years). The scores on the neurological functional disability scale before and after the combination treatment with corticosteroid and cyclophosphamide were 2.43±0.86 and 0.54±0.95 (mean±SD; p<0.001), respectively. The peripheral neuropathy relapsed in one patient.
Conclusions
The long-term clinical outcome of peripheral neuropathy in patients with EGPA receiving initial corticosteroid and cyclophosphamide combination therapy was favorable with a very low relapse rate.
