Cryptogenic organizing pneumonia pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Serge Korjian M.D.

Pathophysiology

Pathogenesis

Various steps in the pathogenesis of cryptogenic organizing pneumonia are: 1) Injury phase - The early phase of cryptogenic organizing pneumonia.

• It is characterized by the deposition of plasma proteins in the alveolar lumen.
• Mechanism of early phase is an imbalance between coagulation and fibrinolytic cascade and activation of coagulation process which leads to fibrin deposition.

2) Proliferating phase - The second stage of the cryptogenic organizing pneumonia in which there is a formation of fibroinflammatory buds.

• Macrophages and inflammatory cells help in fragmentation of fibrin.
• Activated fibroblasts differentiate into myofibroblasts which are migrating through gaps of the basal lamina.
• Inflammatory cells and fibrin are progressively replaced by aggregated fibroblasts/myofibroblasts intermixed with a loose connective matrix tissue rich in collagen (especially collagen I), fibronectin, procollagen type III and proteoglycans.
• Alveolar epithelial cells proliferate, restoring the continuity of the alveolar-capillary membrane and the integrity of the alveolar unit.

3) Mature phase - The third stage is characterized by the formation of “mature” fibrotic buds.

• In alveolar buds, there are myofibroblasts, organized in concentric rings alternating with layers of collagen bundles.

4)Resolution phase - The fourth stage, this stage usually resolves if there is the preservation of alveolar basal laminae.

Genetics

• [Disease name] is transmitted in [mode of genetic transmission] pattern.
• Genes involved in the pathogenesis of [disease name] include [gene1], [gene2], and [gene3].
• The development of [disease name] is the result of multiple genetic mutations.

Gross Pathology

• On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Microscopic Pathology

On microscopic histopathological analysis:

• It is characterized by chronic mild interstitial inflammation without fibrosis.
• There is the formation of buds of granulation tissue which is made of fibrous tissue (Masson bodies), mononuclear cells and foamy macrophages, in the distal airspaces which cause secondary bronchiolar occlusion due to the presence of the inflammatory process.