A35-28 Constrictive Bronchiolitis Secondary to Paraneoplastic Autoimmune Multiorgan Syndrome: A Case Series

Introduction Constrictive bronchiolitis is a rare cause of obstructive pulmonary disease, most commonly seen in lung and bone marrow transplants. It can, however, occur in the general population secondary to environmental and autoimmune causes. Cases of constrictive bronchiolitis have also been found in conjunction with neoplastic processes and mucocutaneous disease, a constellation of findings called paraneoplastic autoimmune multiorgan syndrome (PAMS). We present two cases of patients who underwent transplant for constrictive bronchiolitis in the setting of a neoplastic background. Case 1 A 54 ear-old woman with history notable for oral lichen planus presented to clinic due to progressive dyspnea. Patient had been developing symptoms for a five month period. On presentation, she was found to have severe obstructive lung disease with an FEV1 of 500mL. Steroids and immunosuppressants trialed without success. She required supplemental oxygen and admission for hypercapnic respiratory failure. Imaging suspicious for air small airways disease; overall assessment concerning for constrictive bronchiolitis. Patient underwent transplant evaluation and received bilateral orthoptic lung transplants. Pathology of patient’s native lungs confirmed constrictive bronchiolitis. Course complicated by persistent right pleural effusion with high chest tube output. Workup revealing for pelvic mass with final diagnosis of abdominal follicular dendritic sarcoma, which has a known association with PAMS and lichen planus-like lesions. Patient recently underwent radiation and surgical resection with plans to see medical oncology for further management. Case 2 A 32 year-old woman who initially presented in her 20s with a rash to dermatology and was diagnosed with pemphigus. Patient had a diagnosis of common variable immunodeficiency and asthma but no other prior respiratory history. She underwent further workup and was found to have a retroperitoneal mass on imaging with subsequent diagnosis of Castleman’s. After resection and treatment with rituximab and IVIG she began to develop worsening dyspnea and hypoxemia which remained refractory to steroids. Testing showing an FEV1 of 490mL and imaging with concerns for constrictive bronchiolitis. Her disease progressed and patient underwent transplantation. Castleman’s has a strong association with PAMS and has a higher rate of skin and pulmonary manifestations compared to other neoplastic causes. Discussion In both cases, the patient developed severe bronchiolitis related to a known or unknown malignancy. Prognosis can be quite poor in this patient population but depends in large part on the underlying malignancy, with pulmonary involvement often irreversibly progressive. Transplant is often required but early identification and treatment can sometimes halt progression of the disease. This abstract is funded by: None