TY - JOUR T1 - Limited GPA and Alpha-1 Antitrypsin Deficiency in a Pediatric Patient JF - The Journal of Rheumatology JO - J Rheumatol SP - 543 LP - 544 DO - 10.3899/jrheum.180979 VL - 46 IS - 5 AU - ELAINE S. XIE AU - NIKHIL PAI AU - MICHELLE BATTHISH Y1 - 2019/05/01 UR - http://www.jrheum.org/content/46/5/543.abstract N2 - Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis affecting small to medium-sized blood vessels. The most frequent presenting symptoms are nonspecific constitutional symptoms, followed by pulmonary, renal, and upper respiratory tract symptoms1. Alpha-1-antitrypsin (A1AT) deficiency is the most common genetic cause of liver disease in children2. We present a case of concurrent GPA and A1AT deficiency in a pediatric patient. Consent for this case report was obtained from the patient’s family. Because this is a case report, a waiver from ethics review was granted by the Hamilton Integrated Research Ethics Board.A previously healthy 3-year-old female was referred to pediatric rheumatology for subglottic stenosis. She had presented numerous times to the emergency department over several months with recurrent episodes of cough, nasal congestion, shortness of breath, and stridor. Oral dexamethasone produced dramatic symptomatic improvement. Unfortunately, she experienced ongoing congestion and stridor between steroid courses.History of presenting illness was negative for rash, joint pain/swelling, hematuria, or constitutional symptoms. Past medical history was unremarkable. Family history was positive for childhood asthma in her mother, and systemic lupus erythematosus in … Address correspondence to Dr. M. Batthish, Division of Rheumatology, Department of Pediatrics, McMaster University, 1280 Main St. West, Hamilton, Ontario L8N 3Z5, Canada. E-mail: batthim{at}mcmaster.ca ER -