Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 9-21: Diffuse Interstitial Pneumonias + Key Features Download Section PDF Listen +++ ++ Clinical presentation is similar to that of other idiopathic interstitial pneumonias Median survival is approximately 3 years + Clinical Findings Download Section PDF Listen +++ ++ Onset at age 55–60 with slight male predominance Presents as insidious dry cough with months to years of exertional dyspnea Clubbing is present at diagnosis in 25–50% Pulmonary function tests: restrictive pattern and decreased diffusion capacity Antinuclear antibodies (ANA) and rheumatoid factor (RF) are positive in 25% of patients without documented collagen vascular disease + Diagnosis Download Section PDF Listen +++ ++ Chest radiographic findings Decreased lung volumes with linear or reticular bibasilar or subpleural opacities Normal in 2–10% at diagnosis High-resolution CT scan shows minimal ground-glass and variable honeycombing changes Biopsy shows nonuniform distribution of fibrosis, with loss of type I pneumocytes and proliferation of alveolar type II cells Usual interstitial pneumonia can be diagnosed with 90% confidence in patients age > 65 with classic features, avoiding biopsy + Treatment Download Section PDF Listen +++ ++ Nintedanib and pirfenidone appear to reduce rate of decline in lung function