Mayo Clinic proceedings
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Interstitial lung diseases (ILDs), a broad heterogeneous group of parenchymal lung disorders, can be classified into those with known and unknown causes. The definitions and diagnostic criteria for several major forms of ILDs have been revised in recent years. Although well over 100 distinct entities of ILDs are recognized, a limited number of disorders, including idiopathic pulmonary fibrosis, sarcoidosis, and connective tissue disease-related ILDs, account for most ILDs encountered clinically. ⋯ Serologic testing can be helpful in selected cases. Histopathologic findings procured through bronchoscopic or surgical lung biopsy are often needed in deriving a specific diagnosis. An accurate prognosis and optimal treatment strategy for patients with ILDs depend on an accurate diagnosis, one guided by recent advances in our understanding of the causes and pathogenetic mechanisms of ILDs.