Pulmonary nodular and cystic light chain deposition disease: A retrospective review of 10 cases.

Abstract

Light chain deposition disease (LCDD) rarely involves the lungs. We report clinical and radiologic findings of pulmonary LCDD. We retrospectively identified patients with biopsy-proven pulmonary LCDD seen at Mayo Clinic (Rochester, Minnesota) from January 1997 through December 2018. Demographic, clinical, and imaging features were analyzed. We identified 10 patients with pulmonary LCDD (median age at diagnosis, 55 years; range, 39-77 years). Eight patients were women and 7 were never-smokers. Dyspnea (n=3) and chest pain (n=3) were the most common respiratory symptoms. Associated conditions included Sjögren syndrome (n=6), sarcoidosis (n=1), and limited scleroderma (n=1). Eight patients had mucosa-associated lymphoid tissue (MALT) lymphoma. Among the 9 patients with chest computed tomography (CT) images, 8 (89%) had cysts. Cysts were predominantly distributed in the lower lung and were round or oval. All patients had multiple cysts (5 patients had 1-5 cysts, 3 had >20 cysts). The median diameter of the largest cyst was 18mm (range, 5-68mm). All 9 patients had solid nodules (3 had >10 nodules). Five patients had subsolid nodules. The median diameter of the largest solid nodules was 13mm (range, 6-26mm). Positron emission tomography-CT images were available for 8 patients. The median maximum standardized uptake value of the most avid pulmonary nodule was 2.2 (range, 1.9-6.0). Two patients died during a median follow-up of 2.3 years (range, 0.5-9.9 years). Pulmonary LCDD is characterized by cysts and nodules. The disease is associated with MALT lymphoma, especially in the setting of Sjögren syndrome.