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

Introduction: Light chain deposition disease (LCDD) rarely involves the lungs. We report clinical and radiologic findings of pulmonary LCDD. Methods: 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. Results: 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 18 mm (range, 5–68 mm). All 9 patients had solid nodules (3 had >10 nodules). Five patients had subsolid nodules. The median diameter of the largest solid nodules was 13 mm (range, 6–26 mm). 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). Conclusions: Pulmonary LCDD is characterized by cysts and nodules. The disease is associated with MALT lymphoma, especially in the setting of Sjögren syndrome.