Cryptogenic organizing pneumonia pathophysiology: Difference between revisions
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==Microscopic Pathology== | ==Microscopic Pathology== | ||
On microscopic histopathological analysis:<ref name="urlCryptogenic organising pneumonia | Radiology Reference Article | Radiopaedia.org">{{cite web |url=https://radiopaedia.org/articles/cryptogenic-organising-pneumonia-1 |title=Cryptogenic organising pneumonia | Radiology Reference Article | Radiopaedia.org |format= |work= |accessdate=}}</ref> | |||
*It is characterized by chronic mild interstitial inflammation without fibrosis | *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. | *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. | ||
Revision as of 17:55, 2 March 2018
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Cryptogenic Organizing Pneumonia Microchapters |
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Differentiating Cryptogenic organizing pneumonia from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Serge Korjian M.D.
Overview
Pathophysiology
- The organizing pneumonia pathogenesis model is very similar to the that of cutaneous wound healing.
- Following an insult, a recovery phase characterized by the organisation of inflammatory exudates with resulting intra-alveolar fibrosis ensues.
- Although the intra-alveolar fibrosis resembles that present in usual interstitial pneumonia (UIP), it is however not associated with irreversible fibrosis. In contrast, cryptogenic organizing pneumonia is characterized by it's favorable response to corticosteroid therapy.[1]
Pathophysiology
Pathogenesis
- It is understood that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
- [Pathogen name] is usually transmitted via the [transmission route] route to the human host.
- Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
- [Disease or malignancy name] arises from [cell name's, which are [cell type] cells that are normally involved in [function of cells].
- The progression to [disease name] usually involves the [molecular pathway].
- The pathophysiology of [disease/malignancy] depends on the histological subtype.
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 helps in fragmentation of fibrin.
- Activated fibroblasts differentiate into myofibroblasts which is 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 “mature” fibrotic buds clearly delineated inside the alveolar space. Inflammatory cells and fibrin deposits are no longer found in alveolar buds, which are mostly constituted by typical myofibroblasts (with cytoplasmic filaments orientated toward the cell axis), organized in concentric rings alternating with layers of collagen bundles. At this stage, the connective network consists of thin collagen-I fibers together with thinner fibrils of collagen and procollagen type III, and fibronectin.
In a fourth stage (resolution phase), this process resolves without significant sequelae, similar to reversible wound healing in the skin. The relative preservation of the alveolar basal laminae is considered to be required for the reversibility of the lesions.
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.
Associated Conditions
Gross Pathology
- On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
On microscopic histopathological analysis:[2]
- 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.
References
- ↑ Cordier JF (2006). "Cryptogenic organising pneumonia". Eur Respir J. 28 (2): 422–46. doi:10.1183/09031936.06.00013505. PMID 16880372.
- ↑ "Cryptogenic organising pneumonia | Radiology Reference Article | Radiopaedia.org".