Progressive Pulmonary Cystic Disease in Autoimmune Hepatitis and Rheumatoid Arthritis: A Rare Association.

Pulmonary cystic lung disease is an uncommon manifestation of systemic autoimmune disorders. Rheumatoid arthritis (RA) is most often associated with interstitial lung disease, whereas pulmonary involvement in autoimmune hepatitis (AIH) is rare. We describe an 82-year-old woman with longstanding RA and AIH who demonstrated progressive bilateral pulmonary cystic disease over a 15-year period, complicated by acute hypoxemic respiratory failure and the new identification of a pulmonary arteriovenous malformation. Interval imaging revealed progression of thin-walled pulmonary cysts and fibrotic changes compared with prior studies. This case highlights a rare overlap of autoimmune conditions associated with cystic lung disease and emphasizes the importance of longitudinal imaging, medication review, and multidisciplinary management in patients with complex autoimmune disease presenting with dyspnea.