Sjögren Syndrome Presenting With Acute Fibrinous and Organizing Pneumonia: A Rare Association

Abstract

SESSION TITLE: Fellow Case Report Slide: Pulmonary Disorders SESSION TYPE: Affiliate Case Report Slide PRESENTED ON: Tuesday, October 25, 2016 at 04:30 PM - 05:30 PM INTRODUCTION: Acute fibrinous organizing pneumonia(AFOP) is an acute or subacute lung injury with dominant histological pattern of patchy intra-alveolar fibrin and organizing pneumonia(OP) that does not meet the histologic criteria for diffuse alveolar damage(DAD), OP, or eosinophilic pneumonia(EP).(1)This pattern lack hyaline membranes, eosinophils, granulomatous inflammation, extensive pneumonia or abscess.(1) In the literature, AFOP was associated with rheumatologic conditions but not Sjögren syndrome. CASE PRESENTATION: 75-year-old female with Sjögren syndrome presented with dyspnea, wheezing, fatigue and productive cough. Physical exam: tachypnea, hypoxemia, diffuse wheezes and rhonchi. Chest CT: bilateral multilobar consolidation. She had no clinical improvement on IV antibiotics. Bronchoscopy and transbronchial biopsy revealed focal areas of OP with intra-alveolar fibrin accumulation, reactive type II pneumocyte hyperplasia, focal acute and chronic inflammation consistent with the diagnosis of AFOP.(fig 1&2) Her symptoms promptly improved with steroids. DISCUSSION: AFOP is a new entity with <115 cases reported in the literature. It has been associated with rheumatologic disease, infections, drug reaction, environmental exposures, autoimmune disease and lung transplantation. There are only 8 cases associated with rheumatologic disease in the literature.(2) Tissue histopathology is characterized by intra-alveolar fibrin and OP with a patchy distribution. AFOP have histological pattern similar to DAD, OP and EP but has its own distinct histopathology. She fulfilled the 3 major criteria; intraalveolar fibrin organization, OP and patchy distribution. The pattern also fulfils two minor features; type II pneumocyte hyperplasia and presence of acute and chronic inflammation. The clinical course and radiologic findings are similar to that of OP. The mainstay treatment for AFOP is steroids; other immunosuppressants and antibiotics have also been used with varying results. CONCLUSIONS: We are reporting the first case report of AFOP associated with Sjögren syndrome that had a favorable outcome. Further studies and treatment modalities should be evaluated. Reference #1: Acute fibrinous and organizing pneumonia: a histological pattern of lung injury and possible variant of diffuse alveolar damage. Beasley MB et al. 2002. Arch Pathol Lab Med. pp 1064-1070. Reference #2: Life after acute fibrinous and organizing pneumonia: a case report of a patient 30 months after diagnosis and review of the literature. Kuza C et al. 2016. J Crit Care. pp 255-261. DISCLOSURE: The following authors have nothing to disclose: Adebayo Fasanya, Rihab Sharara, Christina DiCarlo, Raghukumar Thirumala, Mouhib Naddour No Product/Research Disclosure Information