2017 ◽  
Author(s):  
Lawrence A Ho ◽  
Bridget F Collins ◽  
Ganesh Raghu

Idiopathic lung diseases are diffuse parenchymal lung diseases that are grouped under the term interstitial lung disease (ILD). The term interstitial (or interstitium) is, however, misleading as the term interstitium refers to the microscopic anatomic space between the basement membranes of the endothelial and epithelial cells. The pathologic processes involved in these diseases, however, are not limited to the interstitium and can affect other elements of the gas exchange units as well as bronchiolar lumen, terminal bronchioles, pulmonary parenchyma, and pleural and vascular spaces. Since there are potentially hundreds of agents and clinical situations that are associated with ILD, a simplified grouping scheme includes seven main entities: ILD associated with (1) occupational and environmental factors (inhalation cause), (2) collagen vascular diseases, (3) granulomatous lung disease of known and unknown causes (eg, hypersensitivity pneumonitis [HP], sarcoidosis), (4) inherited diseases, (5) iatrogenic/drug induced, (6) certain specific entities (eg, pulmonary Langerhans cell histiocytosis [PLCH], lymphangioleimyomatosis, and (7) idiopathic interstitial pneumonia (IIP). As idiopathic pulmonary fibrosis (IPF) is a subgroup of IIP, this review focuses on the clinical features and management of the major IIPs, and IPF is discussed more in the review, “Idiopathic Pulmonary Fibrosis,” found elsewhere in this publication. This review also focuses on HP as it is a key disease in the differential diagnosis of the IIPs. Figures depict chest radiographs, high resolution computed topography (HRCT) scans, and histopathologic features of various ILDs. Tables list the American Thoracic Society (ATS) and the European Respiratory Society (ERS) classification of IIPs, and common antigens associated with HP and their potential sources. This review contains 22 highly rendered figures, 2 tables, and 156 references.


PLoS ONE ◽  
2021 ◽  
Vol 16 (4) ◽  
pp. e0249300
Author(s):  
Yasutaka Onishi ◽  
Tetsuji Kawamura ◽  
Takanori Higashino ◽  
Rokuro Mimura ◽  
Hiroaki Tsukamoto ◽  
...  

Background Acute fibrinous and organizing pneumonia (AFOP) is a rare histologic pattern of acute lung involvement with intra-alveolar fibrin deposition. However, the clinical significance of the pathological findings of AFOP remains unclear. This study aimed to explore the clinical significance of AFOP through a comprehensive clinical examination. Methods The medical records of patients with lung diseases accompanied by the pathological finding of intra-alveolar organization between January 2010 and December 2019 were retrospectively reviewed. The clinical and radiological findings were compared between the groups with and without the histologic pattern of AFOP. Results We identified 34 patients with AFOP (AFOP group) and 143 without AFOP (non-AFOP group). The underlying diseases of the AFOP group were as follows: 19 patients had cryptogenic organizing pneumonia (OP), 5 had connective tissue diseases, 3 had radiation pneumonitis, 3 had chronic eosinophilic pneumonia, 2 had myelodysplastic syndromes, and 2 had drug-induced pneumonia. Fever was more common, the time from symptom onset to biopsy was shorter, and the serum C-reactive protein level was higher in the AFOP group than in the non-AFOP group. On high-resolution computed tomography, 85% of patients had OP pattern, and halo sign was more common in the AFOP group. Corticosteroids were effective in 94% of the patients in the AFOP group; however, recurrences were more frequent, and a higher corticosteroid dose was needed during recurrence. Conclusions AFOP might be an early phase of a histologic pattern associated with known etiologies. In addition, it could be a marker indicating intense inflammatory diseases with a tendency of recurrence.


2017 ◽  
Author(s):  
Lawrence A Ho ◽  
Bridget F Collins ◽  
Ganesh Raghu

Idiopathic lung diseases are diffuse parenchymal lung diseases that are grouped under the term interstitial lung disease (ILD). The term interstitial (or interstitium) is, however, misleading as the term interstitium refers to the microscopic anatomic space between the basement membranes of the endothelial and epithelial cells. The pathologic processes involved in these diseases, however, are not limited to the interstitium and can affect other elements of the gas exchange units as well as bronchiolar lumen, terminal bronchioles, pulmonary parenchyma, and pleural and vascular spaces. Since there are potentially hundreds of agents and clinical situations that are associated with ILD, a simplified grouping scheme includes seven main entities: ILD associated with (1) occupational and environmental factors (inhalation cause), (2) collagen vascular diseases, (3) granulomatous lung disease of known and unknown causes (eg, hypersensitivity pneumonitis [HP], sarcoidosis), (4) inherited diseases, (5) iatrogenic/drug induced, (6) certain specific entities (eg, pulmonary Langerhans cell histiocytosis [PLCH], lymphangioleimyomatosis, and (7) idiopathic interstitial pneumonia (IIP). As idiopathic pulmonary fibrosis (IPF) is a subgroup of IIP, this review focuses on the clinical features and management of the major IIPs, and IPF is discussed more in the review, “Idiopathic Pulmonary Fibrosis,” found elsewhere in this publication. This review also focuses on HP as it is a key disease in the differential diagnosis of the IIPs. Figures depict chest radiographs, high resolution computed topography (HRCT) scans, and histopathologic features of various ILDs. Tables list the American Thoracic Society (ATS) and the European Respiratory Society (ERS) classification of IIPs, and common antigens associated with HP and their potential sources. This review contains 22 highly rendered figures, 2 tables, and 156 references.


Sign in / Sign up

Export Citation Format

Share Document