What is Idiopathic Pulmonary Fibrosis?

Idiopathic Pulmonary Fibrosis
Histopathological and molecular correlates of idiopathic pulmonary fibrosis. a–c (hematoxylin and eosin) show increased magnifications of idiopathic pulmonary fibrosis in which areas of histologically normal lung is admixed with areas of active fibrosis (a, × 25), marked by serpentine glands lined with epithelia (b, × 50), which show nuclear atypia and multinucleation (c, arrow, × 400). Herpesvirus saimiri DNA was commonly detected in the nuclei of the epithelia of these serpentine glands (d, × 50) and much less evident in the admixed histologically normal lung (e, × 400; blue NBT/BCIP signal with pink counterstain). In comparison, herpesvirus saimiri DNA was not evident in cases of interstitial pneumonitis and fibrosis of known viral etiology (f, × 400 measles infection). Herpesvirus saimiri DNA was evident in the regenerating epithelia in idiopathic pulmonary fibrosis in the same cells when serial sections were probed for herpesvirus saimiri terminal repeat sequence (g, × 400) or the STP ORF 1 gene (h, × 400). Idiopathic pulmonary fibrosis is strongly associated with productive infection by herpesvirus saimiri. Folcik VA, Garofalo M, Coleman J, Donegan JJ, Rabbani E, Suster S, Nuovo A, Magro CM, Di Leva G, Nuovo GJ - Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc (2013). Not Altered. CC.

Idiopathic Pulmonary Fibrosis is a chronic progressive syndrome of the lungs with 3–5 years projected median survival time after diagnosis was made.

What is the Pathology of Idiopathic Pulmonary Fibrosis?

The pathology of idiopathic pulmonary fibrosis is: 

-Etiology: The cause of idiopathic pulmonary fibrosis is unknown.

-Genes involved: None. 

-Pathogenesis: The sequence of events that lead to idiopathic pulmonary fibrosis is unknown.

-Histology: The histology associated with idiopathic pulmonary fibrosis shows alveolar exudate, fibrosis in the alveoli, and the interstitial septal wall with inconstant inflammation.

How does Idiopathic Pulmonary Fibrosis Present?

Patients with idiopathic pulmonary fibrosis typically males are affected more frequently, and present at age range of 20 to 45 years. The symptoms, features, and clinical findings associated with idiopathic pulmonary fibrosis include a dry cough and slowly developing dyspnea, cor-pulmonale fingers clubbing.

How is Idiopathic Pulmonary Fibrosis Diagnosed?

Idiopathic pulmonary fibrosis is diagnosed by excluding all known causes of interstitial fibrosis.

How is Idiopathic Pulmonary Fibrosis Treated?

Idiopathic pulmonary fibrosis is treated medical care-, pirfenidone and nintedanib antifibrotic drugs slow the lung functions weakening.

What is the Prognosis of Idiopathic Pulmonary Fibrosis?

The prognosis of idiopathic pulmonary fibrosis is poor, death within 6 weeks to 6 months.

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Last updated on May 25, 2022