Childhood-Onset COPA Syndrome Recognized Retrospectively in the Context of Polyarticular Juvenile Idiopathic Arthritis and Rheumatoid Arthritis

COPA syndrome is a very rare autoinflammatory disorder manifesting with childhood-onset arthritis and pulmonary and renal disease, of which awareness may remain lacking. We present the case of a twenty-year-old male patient seen in the Young Adults with Rheumatic Disease clinic. Initially diagnosed...

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Main Authors: Roko P. A. Nikolic, Cristina Moran Toro
Format: Article
Language:English
Published: Wiley 2023-01-01
Series:Case Reports in Rheumatology
Online Access:http://dx.doi.org/10.1155/2023/3240245
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author Roko P. A. Nikolic
Cristina Moran Toro
author_facet Roko P. A. Nikolic
Cristina Moran Toro
author_sort Roko P. A. Nikolic
collection DOAJ
description COPA syndrome is a very rare autoinflammatory disorder manifesting with childhood-onset arthritis and pulmonary and renal disease, of which awareness may remain lacking. We present the case of a twenty-year-old male patient seen in the Young Adults with Rheumatic Disease clinic. Initially diagnosed with seropositive polyarticular juvenile idiopathic arthritis, the patient’s early childhood complaints of fatiguability, paroxysmal dyspnea, and pneumonia-like episodes were long to be felt unrelated to his arthritis. Upon transition to adult rheumatology care, a thorough review of the patient’s history prompted imaging which revealed interstitial lung disease. Restrictive spirometry and genetic testing confirmed the retrospective diagnosis of COPA syndrome.
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spelling doaj-art-882f6b0c98334601896fe6b18afed12e2025-08-20T03:07:16ZengWileyCase Reports in Rheumatology2090-68972023-01-01202310.1155/2023/3240245Childhood-Onset COPA Syndrome Recognized Retrospectively in the Context of Polyarticular Juvenile Idiopathic Arthritis and Rheumatoid ArthritisRoko P. A. Nikolic0Cristina Moran Toro1Department of MedicineDivision of RheumatologyCOPA syndrome is a very rare autoinflammatory disorder manifesting with childhood-onset arthritis and pulmonary and renal disease, of which awareness may remain lacking. We present the case of a twenty-year-old male patient seen in the Young Adults with Rheumatic Disease clinic. Initially diagnosed with seropositive polyarticular juvenile idiopathic arthritis, the patient’s early childhood complaints of fatiguability, paroxysmal dyspnea, and pneumonia-like episodes were long to be felt unrelated to his arthritis. Upon transition to adult rheumatology care, a thorough review of the patient’s history prompted imaging which revealed interstitial lung disease. Restrictive spirometry and genetic testing confirmed the retrospective diagnosis of COPA syndrome.http://dx.doi.org/10.1155/2023/3240245
spellingShingle Roko P. A. Nikolic
Cristina Moran Toro
Childhood-Onset COPA Syndrome Recognized Retrospectively in the Context of Polyarticular Juvenile Idiopathic Arthritis and Rheumatoid Arthritis
Case Reports in Rheumatology
title Childhood-Onset COPA Syndrome Recognized Retrospectively in the Context of Polyarticular Juvenile Idiopathic Arthritis and Rheumatoid Arthritis
title_full Childhood-Onset COPA Syndrome Recognized Retrospectively in the Context of Polyarticular Juvenile Idiopathic Arthritis and Rheumatoid Arthritis
title_fullStr Childhood-Onset COPA Syndrome Recognized Retrospectively in the Context of Polyarticular Juvenile Idiopathic Arthritis and Rheumatoid Arthritis
title_full_unstemmed Childhood-Onset COPA Syndrome Recognized Retrospectively in the Context of Polyarticular Juvenile Idiopathic Arthritis and Rheumatoid Arthritis
title_short Childhood-Onset COPA Syndrome Recognized Retrospectively in the Context of Polyarticular Juvenile Idiopathic Arthritis and Rheumatoid Arthritis
title_sort childhood onset copa syndrome recognized retrospectively in the context of polyarticular juvenile idiopathic arthritis and rheumatoid arthritis
url http://dx.doi.org/10.1155/2023/3240245
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