PMID- 35547355
OWN - NLM
STAT- MEDLINE
DCOM- 20220517
LR  - 20220716
IS  - 2314-6141 (Electronic)
IS  - 2314-6133 (Print)
VI  - 2022
DP  - 2022
TI  - Clinical, Serological, and Genetic Characteristics of a Hungarian 
      Myositis-Scleroderma Overlap Cohort.
PG  - 6251232
LID - 10.1155/2022/6251232 [doi]
LID - 6251232
AB  - 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) - DRB1 *03 and DQA1 *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.
CI  - Copyright (c) 2022 Katalin Szabo et al.
FAU - Szabo, Katalin
AU  - Szabo K
AUID- ORCID: 0000-0002-0617-1703
AD  - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, 
      Moricz Zs. krt. 22, 4032 Debrecen, Hungary.
FAU - Bodoki, Levente
AU  - Bodoki L
AUID- ORCID: 0000-0002-4934-6032
AD  - Division of Rheumatology, Faculty of Medicine, University of Debrecen, Moricz Zs. 
      krt. 22, 4032 Debrecen, Hungary.
FAU - Nagy-Vincze, Melinda
AU  - Nagy-Vincze M
AUID- ORCID: 0000-0003-0316-3828
AD  - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, 
      Moricz Zs. krt. 22, 4032 Debrecen, Hungary.
FAU - Beldi, Tibor
AU  - Beldi T
AUID- ORCID: 0000-0003-4972-1370
AD  - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, 
      Moricz Zs. krt. 22, 4032 Debrecen, Hungary.
FAU - Vincze, Anett
AU  - Vincze A
AUID- ORCID: 0000-0003-3654-9268
AD  - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, 
      Moricz Zs. krt. 22, 4032 Debrecen, Hungary.
FAU - Zilahi, Erika
AU  - Zilahi E
AUID- ORCID: 0000-0002-7376-8921
AD  - Department of Laboratory Medicine, Faculty of Medicine, University of Debrecen, 
      Nagyerdei krt. 98, 4032 Debrecen, Hungary.
FAU - Varga, Jozsef
AU  - Varga J
AUID- ORCID: 0000-0001-5797-9625
AD  - Department of Medical Imaging, Division of Nuclear Medicine, University of 
      Debrecen, Debrecen, Hungary.
FAU - Szucs, Gabriella
AU  - Szucs G
AUID- ORCID: 0000-0002-0958-9531
AD  - Division of Rheumatology, Faculty of Medicine, University of Debrecen, Moricz Zs. 
      krt. 22, 4032 Debrecen, Hungary.
FAU - Danko, Katalin
AU  - Danko K
AUID- ORCID: 0000-0002-8729-3947
AD  - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, 
      Moricz Zs. krt. 22, 4032 Debrecen, Hungary.
FAU - Griger, Zoltan
AU  - Griger Z
AUID- ORCID: 0000-0002-1371-7911
AD  - Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, 
      Moricz Zs. krt. 22, 4032 Debrecen, Hungary.
LA  - eng
PT  - Journal Article
DEP - 20220502
PL  - United States
TA  - Biomed Res Int
JT  - BioMed research international
JID - 101600173
RN  - 0 (HLA-DRB1 Chains)
SB  - IM
MH  - *Deglutition Disorders
MH  - HLA-DRB1 Chains
MH  - Humans
MH  - Hungary
MH  - *Myositis/genetics
MH  - Retrospective Studies
MH  - *Scleroderma, Localized
MH  - *Scleroderma, Systemic/complications/genetics
PMC - PMC9085307
COIS- The authors declare that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2022/05/14 06:00
MHDA- 2022/05/18 06:00
PMCR- 2022/05/02
CRDT- 2022/05/13 11:12
PHST- 2022/02/23 00:00 [received]
PHST- 2022/04/12 00:00 [accepted]
PHST- 2022/05/13 11:12 [entrez]
PHST- 2022/05/14 06:00 [pubmed]
PHST- 2022/05/18 06:00 [medline]
PHST- 2022/05/02 00:00 [pmc-release]
AID - 10.1155/2022/6251232 [doi]
PST - epublish
SO  - Biomed Res Int. 2022 May 2;2022:6251232. doi: 10.1155/2022/6251232. eCollection 
      2022.