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A3087 - Multiorganic Autoimmune Paraneoplastic Syndrome: Constrictive Bronchiolitis Associated with Paraneoplastic Pemphigus in Follicular Lymphoma
Author Block: M. P. Caicedo1, D. Cañon2, C. Castillo3; 1Pulmonology, Fundacion Santa Fe de Bogota, Bogotá, Colombia, 2Pathology, Fundacion Santa Fe de Bogota, Bogotá, Colombia, 3Internal Medicine, Fundacion Santa Fe de Bogota, Bogotá, Colombia.
INTRODUCTION:
Constrictive bronchiolitis as part of the paraneoplastic autoimmune multiorgan syndrome (PAMS) is a rare but often fatal disease, due to its progressive nature and lack of response to treatments.
CASE :
A 39-year-old male, formerly a high-performance athlete with a diffuse large-cell non-Hodgkin's lymphoma diagnosed and treated eight years before, considered in remission, with no history of smoking, collagen diseases, or exposure to toxics, who presents with progressive dyspnea for the last two years. In the last month he complained of resting dyspnea, orthopnea, dry cough and weight loss. Additionally, multiple blister lesions on oral mucosa and lower limbs were diagnosed as pemphigus vulgaris one year ago. During the admission, he was found to be malnourished, with clinical signs of advanced chronic respiratory failure, some blister lesions in oral mucosa, and old scars in both lower limbs. He was hypercapnic, pulmonary function tests showed severe airway obstruction and air trapping that did not improve with bronchodilators and indicated rapid worsening compared to previous spirometries; he had not responded to previous systemic corticosteroid management. A suspicion of paraneoplastic syndrome was raised and biopsies performed six months ago at another hospital were reviewed confirming the diagnosis of pemphigus vulgaris in oral mucosa; and follicular grade 1 lymphoma with no evidence of diffuse component in a mediastinal node. Bone marrow biopsy at our hospital also showed minimal infiltration by lymphoma. Considering the coexistence of active hematological disease, pemphigus and probable constrictive bronchiolitis, PAMS was diagnosed and due to the progressive and severe respiratory deterioration and low performance status he was offered palliative care and died three weeks later. A limited post-mortem autopsy confirmed the diagnosis of constrictive bronchiolitis.
DISCUSSION:
This case reminds us that constrictive bronchiolitis is a rare disease that initially can mimic other more benign airway obstructive diseases, but when is part of PAMS is often refractory to treatment and lethal. PAMS is a rare syndrome with multiple manifestations that can compromise the skin, lungs, kidneys and muscles. (1) Originally described in 1990 and commonly associated with lymphocytic disorders non-Hodgkin's lymphoma, chronic lymphocytic leukemia, Castleman's disease (2). This autoimmune disorder is characterized by IgG autoantibodies against the proteins of desmosomes and hemidemosomes, transmembrane proteins (desmoglein) and an unidentified 170-kd antigen generating loss of cell-cell junctions, including bronchial epithelial involvement. (3,4), that evolve to submucosal fibrosis and bronchiolar obliteration.