Snapshot A 42-year-old Nigerian female presents to the emergency room with shallow, rapid breathing. She worked in a glass manufacturing facility for 20 years prior to immigrating to the United States. Her sister reports that she has had breathing difficulty for the last 5-10 years that is getting progressively worse over time. Introduction Also known as interstitial lung disease Group of disorders characterized by inflammation and fibrosis of the interalveolar septum Chronic injury can be idiopathic in some cases Known etiologies include pneumoconioses asbestosis, silicosis, and the like collagen vascular disease granulomatous disorders drug side effects bleomycin and busulfan radiation exposure hypersensitivity disorders chronic infections (TB) eosinophilic pulmonary syndromes Presentation Symptoms shallow, rapid breathing shortness of breath dyspnea on exertion chronic, non-productive cough Physical exam cyanosis crackles/rales inspiratory wheezes digital clubbing RHF Evaluation Diagnosis is highly clinical but complemented by classic CXR findings CXR diagnostic gold standard may appear reticular, nodolar, or as ground-glass severe disease shows cystic spaces in the lung periphery, or "honeycombing" PFTs decreased TLC, FVC, and DLCO normal FEV1 / FVC ratio Labs serum markers for connective tissue disease may be obtained in suspected clinically Differential COPD, asthma, bronchiectasis, pneumonia Treatment Non-operative supportive management indicated in all cases avoid further exposure to causative agents ambulatory/supplemental O2 when SaO2 <90% corticosteroids and other anti-inflammatory/immunosuppressive agents may be indicated in inflammatory conditions (i.e. collagen vascular disease) Operative lung transplantation may be indicated for survival in most advanced disease Prognosis, Prevention, and Complications Prognosis poor to fair, as disease is irreversible and often progressive if not managed appropriately Prevention treatment of underlying causes is the only way to prevent progression of disease unknown etiologies make prevention difficult to impossible Complications irreversible nature of disease may lead to rapid, uninhibited progression and death