All of the following statement are true regarding idiopathic pulmonary fibrosis, except:
Idiopathic Pulmonary Fibrosis (also known as cryptogenic fibrosing alveolitis): Grossly, the pleural surfaces of the lung are cobblestoned as a result of the retraction of scars along the interlobular septa. The cut surface shows firm, rubbery white areas of fibrosis, which occurs preferentially in the lower lobes the subpleural regions, andalong the interlobular septa. Histologically,the hallmark is patchy interstitial fibrosis. The earliest lesions contain exuberant fibroblastic proliferation (fibroblastic foci). With time these areas become more collagenous and less cellular. Prognosis - poor Treatment - Immunosuppresive drugs; Lung transplant is the only definitive therapy. Newer drugs useful in IPF are: Nintedanib and Pirfenidone Masson bodies are seen in Cryptogenic Organising Pneumonia.
Pleural surfaces developed "cobblestoned" appearance
Areas of fibrosis, occur preferentially in the lower lobes and subpleural regions
Fibroblastic foci are the earliest lesion
Masson bodies are characteristic
Interstitial lung diseases