In:
BioMed Research International, Hindawi Limited, Vol. 2022 ( 2022-5-2), p. 1-9
Kurzfassung:
Overlap myositis is a distinct subgroup of idiopathic inflammatory myositis (IIM) with various clinical phenotypes. The aim of this study was to determine the clinical, serological, and genetic features of systemic sclerosis (SSc)-IIM overlap patients. It was a retrospective study using clinical database of 39 patients, fulfilling both the criteria of SSc and IIM. 56.4% of the patients had limited cutaneous, 43.6% had diffuse cutaneous SSc, whereas 7.7% of the patients had dermatomyositis and 92.3% polymyositis. The two diseases occurred simultaneously in 58.97%, while 10.26% in myositis and 30.77% in scleroderma were initially diagnosed. The frequencies of organ involvement were interstitial lung disease 71.8%, dysphagia 66.7%, cardiac involvement 41%, pulmonary arterial hypertension (PAH) 30.8%, and renal involvement 12.8%, respectively. The presence of human leukocyte antigen HLA − DRB 1 ∗ 03 and DQA 1 ∗ 051 ∗ 01 alleles were significantly higher in the overlap patients than in healthy controls (82.35% vs. 27.54%; p 〈 0.0001 and 88.24% vs. 30.16; p 〈 0.0001 ). Certain clinical parameters, such as fever at diagnosis (41.67% vs. 7.41%, p = 0.0046 ), cardiac involvement (83.33% vs. 22.22%, p = 0.0008 ), subcutaneous calcinosis (41.66 vs. 11.11, p = 0.01146 ), and claw hand deformity (25% vs. 11.11%, p = 0.00016 ) were significantly associated with the presence of PAH. Upon comparison, the overlap patients and anti-Jo-1 positive antisynthetase patients showed similarities in terms of genetic results and major clinical features; however, SSc-IIM overlap patients could be distinguished with higher erythrocyte sedimentation rate (ESR) level, more frequent presence of Raynaud’s phenomenon ( p 〈 0.0001 ; OR: 20.00), dysphagia ( p 〈 0.0001 ; OR: 15.63), and infrequent livedo reticularis ( p 〈 0.01 ; OR: 0.11). SSc-IIM overlap myositis is a unique group within IIM-s possessing characteristic clinical features.
Materialart:
Online-Ressource
ISSN:
2314-6141
,
2314-6133
DOI:
10.1155/2022/6251232
Sprache:
Englisch
Verlag:
Hindawi Limited
Publikationsdatum:
2022
ZDB Id:
2698540-8
