CHRONIC EOSINOPHILIC PNEUMONIA IN A PATIENT WITH AUTOIMMUNE MENINGOENCEPHALITIS

Abstract

SESSION TITLE: Diffuse Lung Diseases SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/22/2019 3:45 PM - 4:45 PM INTRODUCTION: Glial fibrillary acidic protein (GFAP) encephalomyelitis is a newly described form of autoimmune meningoencephalitis that is characterized by GFAP-specific antibodies.1 This condition has been associated with a high incidence of malignancy, but to date there are no known associated pulmonary diseases. CASE PRESENTATION: A 58-year-old man with a history of GFAP encephalomyelitis was referred for evaluation of symmetric, upper-lobe predominant ground-glass infiltrates (Figure 1). He reported only minimal cough. For GFAP he was being treated with mycophenolate mofetil, prednisone, and appropriate prophylactics. He was completing a prednisone taper at his initial presentation. He underwent bronchoscopy with bronchoalveolar lavage (BAL). Cytology and infectious studies were negative. There were 44 total nucleated cells (TNC), 20% of which were eosinophils. Pulmonary function tests (PFTs) showed no obstruction or restriction, but the DLCO was reduced at 59% predicted. He had normal autoimmune markers, serum protein electrophoresis, and free light chains. He returned for a 3-month follow-up with worsening cough and dyspnea. He had new restriction on PFTs (TLC 64% predicted, FVC 58% predicted). A repeat chest CT showed significant progression of his prior infiltrates (Figure 2) and mild lymphadenopathy. In the interim, he had discontinued prednisone, but remained on pneumocystis prophylaxis. A urinalysis, creatine kinase, aldolase, and hypersensitivity pneumonitis panel were within normal limits. He underwent bronchoscopy with BAL and transbronchial biopsies. Cytology and infectious studies were negative. There were 42 TNC, 34% of which were eosinophils. Pathology showed organizing pneumonia with eosinophils. He was treated with 60mg of prednisone with a planned taper and prescribed oxygen. He noted significant improvement after re-starting steroids and at 3-month follow-up, he was taking 20mg of prednisone and had no pulmonary limitations. A chest CT showed significant improvement (Figure 3) and PFTs demonstrated improvement (TLC to 85% predicted, FVC 91% predicted). DISCUSSION: The patient's elevated eosinophil count in his BAL, evidence of eosinophils with concurrent organizing pneumonia on transbronchial biopsies, and rapid response to steroids is most consistent with chronic eosinophilic pneumonia. Although his neurologic disease did not have a concurrent exacerbation with his lung disease, the co-occurrence of these two rare conditions suggests a potential association between them. CONCLUSIONS: This represents the first reported case of chronic eosinophilic pneumonia associated with GFAP meningoencephalomyelitis. Further study into an association between these two entities is warranted Reference #1: Fang B, McKeon A, Hinson SR, et al. Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Novel Meningoencephalomyelitis. JAMA neurology. 2016;73(11):1297-1307. DISCLOSURES: No relevant relationships by Cassandra Braun, source=Web Response No relevant relationships by Gregory Stroh, source=Web Response