A RARE ASTHMA MIMIC

Abstract

SESSION TITLE: Allergy and Airway SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: IgG4 related disease (IgG4-RD) is a recently identified systemic fibro-inflammatory disease associated with elevated circulating levels of IgG4. We are presenting a case of IgG4-RD with hilar mediastinal lymph node and bronchial wall inflammation presenting as an asthma exacerbation. CASE PRESENTATION: A 49 year old male presented to the pulmonary clinic for an evaluation of asthma after several emergency room visits for worsening dyspnea, dry cough and wheezing for 2 months. The patient had no prior history of respiratory issues, and reported that he had never smoked. The patient noted that his facial features appeared to be changing over the previous 4 months. The patient moved from the Democratic Republic of Congo 25 years ago, and currently is a chemistry teacher. The patient reports no recent travel, has no pets. Additionally, the patient denies any recent organic or inorganic fume or dust exposure, and reports no family history of asthma or respiratory issues. Bilateral firm parotid swelling and periorbital edema were prominent on examination making his face appear significantly different from a picture he took 4 months ago. Bilateral wheezes were noted in the lungs with prolonged expiratory phase. The patient’s pulmonary function tests (PFTs) revealed a severe obstructive defect with an FEV1 of 1.33, increased residual volume and a mild diffusion defect. A PET scan was administered to rule out lymphoma as a cause of parotid swelling. Marked enlargement with diffuse hypermetabolic activity was seen in the parotid, submandibular, sublingual, and lacrimal glands. Additionally, multiple mildly hypermetabolic enlarged mediastinal and hilar lymph nodes were observed. There was peribronchial thickening with scattered distal bronchiolar plugging. A biopsy of the submandibular gland revealed sclerosing sialadenitis with interlobular storiform fibrosis, and marked lymphoplasmacytic inflammation. The immunohistochemical findings were consistent with IgG4-RD. A serum IgG subclass analysis was done and showed increased IgG4 level upto 1070mg/dl. The patient was referred to rheumatology and was treated with Rituximab for 3 cycles. His parotid swelling, dysmorphic facial features resolved after the first treatment. So did his dyspnea and wheezing. His repeat PFTs normalized as well. DISCUSSION: IgG-RD is an immune mediated systemic fibro-inflammatory condition that affects almost any organ. Although the inflammation is responsive to steroids, it tends to recur. However, there is a good response to rituximab with less rate of recurrence. CONCLUSIONS: Pulmonary involvement can present as inflammatory pseudo tumors mimicking malignancies, consolidative infiltrates hilar mediastinal lymphadenopathy or bronchovascular inflammation. This patient's symptoms stemmed from bronchovascular inflammation, untreated progression to irreversible bronchovascular fibrosis is highly likely. Reference #1: Clinical Review of Pulmonary Manifestations of IgG4-related DiseaseSabrina N. Campbell, Edmundo Rubio, and A. Lukas LoschnerAnn Am Thorac Soc Vol 2014;11:1466–1475 DISCLOSURES: No relevant relationships by Arezou Khosroshahi, source=Web Response Research support relationship with Boehringer Ingelheim, Sanofi, Fibrogen, Biogen, Please note: >$100000 Added 05/31/2018 by Srihari Veeraraghavan, source=Web Response, value=Grant/Research Support No relevant relationships by Avanthika Wynn, source=Web Response