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![Lymphocytic interstitial pneumonia pattern in a patient with Sjögren syndrome. (A, B) Various degree of the interstitial thickening with abundant lymphoplasmacytic cells aggregates (arrows) is present (hematoxylin and eosin stain, ×40 [A] and ×100 [B]). Courtesy of Professor Jin-Haeng Chung, M.D., Seoul National University Bundang Hospital.](publication/330399725/figure/fig4/AS:715403772850177@1547576959643/Lymphocytic-interstitial-pneumonia-pattern-in-a-patient-with-Sjoegren-syndrome-A-B.png)
Lymphocytic interstitial pneumonia pattern in a patient with Sjögren syndrome. (A, B) Various degree of the interstitial thickening with abundant lymphoplasmacytic cells aggregates (arrows) is present (hematoxylin and eosin stain, ×40 [A] and ×100 [B]). Courtesy of Professor Jin-Haeng Chung, M.D., Seoul National University Bundang Hospital.
Source publication
Connective tissue diseases (CTDs) can affect all compartments of the lungs, including airways, alveoli, interstitium, vessels, and pleura. CTD-associated lung diseases (CTD-LDs) may present as diffuse lung disease or as focal lesions, and there is significant heterogeneity between the individual CTDs in their clinical and pathological manifestation...
Context in source publication
Context 1
... reveals various features of cellular NSIP pattern, follicular bronchiolitis, lymphoid interstitial hyperplasia, and LIP [1,31]. Common histological patterns of SjS-ILD are NSIP with cellular, fibrotic, or mixed patterns [32]. SjS-LIP shows florid peribronchiolar and interstitial lymphoplasmacytic infiltrates with expansion to the alveolar septa (Fig. 7). The LIP lesion also reveals germinal centers, and occasionally non-necrotizing granulomas [1,33]. LIP is most frequently associated with SjS among CTD-LDs, and can be a precursor lesion of malignant lymphoma. About 25% of LIP patients have SjS [1,31,33]. SjS frequently shows involvement of the large and small airways, and the small ...
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Introduction:
Statistical data on the structure of acute respiratory diseases incidence in the paediatric population are still scarce. The demand for such data results mainly from the need to constantly implement new systemic and economic solutions. The aim of the study was to attempt to use reported data for an assessment of the incidence of acut...
Citations
... This discrete IIP is morphologically defined by the histopathological pattern of Nonspecific Interstitial Pneumonia (NSIP) [59]. Interestingly, the NSIP pattern could be found in a broad range of clinical settings, such as pulmonary diseases of known cause, like Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD) or Hypersensitivity Pneumonitis (HP), as well as in the setting of an IIPs [60][61][62][63][64][65][66][67][68][69]. The NSIP pattern is defined by an interstitial inflammatory and/or fibrosing process with diffuse and uniform distribution throughout the bioptic sample [59,70]. ...
Idiopathic Interstitial Pneumonias (IIPs) are a heterogeneous group of the broader category of Interstitial Lung Diseases (ILDs), pathologically characterized by the distortion of lung parenchyma by interstitial inflammation and/or fibrosis. The American Thoracic Society (ATS)/European Respiratory Society (ERS) international multidisciplinary consensus classification of the IIPs was published in 2002 and then updated in 2013, with the authors emphasizing the need for a multidisciplinary approach to the diagnosis of IIPs. The histological evaluation of IIPs is challenging, and different types of IIPs are classically associated with specific histopathological patterns. However, morphological overlaps can be observed, and the same histopathological features can be seen in totally different clinical settings. Therefore, the pathologist’s aim is to recognize the pathologic–morphologic pattern of disease in this clinical setting, and only after multi-disciplinary evaluation, if there is concordance between clinical and radiological findings, a definitive diagnosis of specific IIP can be established, allowing the optimal clinical–therapeutic management of the patient.
Background
Acute fibrinous and organizing pneumonia (AFOP) is a rare histologic interstitial pneumonia pattern characterized by the intra-alveolar fibrin deposition and organizing pneumonia. Its clinical characteristics are still not well known and there is no consensus on treatment yet.
Case presentation
We report two female cases in their fifties diagnosed with AFOP confirmed by a second lung biopsy. Case 1 was idiopathic AFOP with manifestation of 6-week fever, dyspnea, and cough, while case 2 was secondary to systemic lupus erythematosus and fever was the major symptom. Their chest CT scans revealed bilateral multiple consolidations, predominantly in the lower lobes. Both cases were initially diagnosed with pneumonia, but did not improve after treatment with broad-spectrum antibiotics. In both cases, transbronchial biopsy and bronchoalveolar lavage fluid examination were inconclusive and the pathological diagnosis was confirmed by percutaneous lung biopsy. Both patients had a good clinical response to prednisone.
Conclusions
We report two rare AFOP cases to highlight the importance of awareness of this disease. We further perform the most comprehensive review to date in AFOP, including 150 patients since 2002. Consolidation was the most common imaging pattern, followed by ground-glass opacity and nodules. A lung biopsy is required for a definitive diagnosis. Corticosteroids is recommended as the most effective therapy, but treatment options should depend on the etiology and disease severity.
Background
Acute fibrinous and organizing pneumonia (AFOP) is a rare histologic interstitial pneumonia pattern characterized by the intra-alveolar fibrin deposition and organizing pneumonia. Though a small amount of cases has been reported, its causes and clinical characteristics are still not well known and there is no standard treatment yet.
Case presentation
We report two cases with a histological diagnosis of AFOP. Case 1 was idiopathic AFOP and presented with fever, dyspnea, cough, while the case 2 was associated with SLE and fever was her most common symptom. Their chest CT scan revealed bilateral multiple consolidations, predominantly in the lower lobes. Both cases were diagnosed after a second lung biopsy and treated with corticosteroids.
Conclusions
We not only describe two rare AFOP cases but also demonstrate the most comprehensive review to date in AFOP, including 150 patients since 2002. Most common symptoms include dyspnea, cough, and fever. Consolidation was the most common imaging pattern, followed by GGO and nodules. Given the various etiology and nonspecific clinical presentation, AFOP probably had been under diagnosed. Early identification is very important for AFOP management and a lung biopsy is required for a definitive diagnosis. Corticosteroids was recommended to be the most useful therapy, and treatment options should depend on the etiology and disease severity.
