Although the etiology and pathogenesis are unknown, the lung damage is thought to be caused by the release of toxic oxygen species and proteases by polymorphonuclear neutrophils Mean age at onset is 50 years. Both sexes are equally affected and there is no correlation with tobacco smoking There are no known risk factors CLINICAL FEATURES The onset is acute and the most common presenting symptoms are cough (100%), dyspnea (80-100%) and fever. Patients often report a prodromal flu-like illness (joint and muscle pain, fever, chills and malaise) Physical examination reveals tachypnea and peripheral cyanosis. In 50% of cases fine rales can be heard throughout the lung fields All patients have reduced DLCO and a restrictive pattern. Moderate to severe hypoxemia, at times refractory to oxygen therapy, rapidly ensues. Rapid progression to respiratory failure similar to acute respiratory distress syndrome (ARDS) is common Vourlekis JS. Acute interstitial pneumonitis. Case series and review of the literature. Medicine 2000, 79: 369 PATHOLOGY The histologic features of AIP are those of diffuse alveolar damage (DAD): • Acute (exudative) phase, rarely biopsied: hyaline membranes, an expression of acute epithelial damage, line the alveolar walls. The alveolar septa show edema and varying amounts of acute and chronic inflammatory infiltrate. Thromboses of small and medium-size arterioles are common • Organizing (proliferative) phase: proliferation of myofibroblasts that migrate from the interstitium to the alveolar spaces: the hyaline membranes (A) are resorbed and organized within the alveolar septa, which become thickened (). Proliferation of hyperplastic type II pneumocytes restores the alveolar epithelium • Chronic (fibrotic) phase: dense fibrosis with possible distortion of lung architecture Definition Etiology and pathogenesis Epidemiology Risk factors History Physical findings Basic lesions Pulmonary function tests Diffuse Lung Diseases Maffessanti & Dalpiaz AIP ☯ Diffuse (alveoli and alveolar septa) Histopathologic differential diagnoses: • DAD associated with infections: granulomas, viral inclusions, necrotic foci, abscesses; identification of the microorganism with special stains • DAD superimposed on UIP (accelerated UIP): associated with the characteristic UIP pattern • DAD due to other causes: not idiopathic but secondary to other causes (shock, trauma, physical or chemical causes, etc.) • OP: predominantly intraalveolar foci of fibroblastic organization, intense inflammatory infiltrate, bronchiolar involvement (not constant)