Effect of Genetic and Laboratory Findings on Clinical Course of Antisynthetase Syndrome in a Hungarian Cohort

Author:

Szabó Katalin1ORCID,Bodoki Levente1ORCID,Nagy-Vincze Melinda1ORCID,Vincze Anett1,Zilahi Erika2ORCID,Szodoray Peter3,Dankó Katalin1,Griger Zoltán1ORCID

Affiliation:

1. University of Debrecen, Faculty of Medicine, Division of Clinical Immunology, Móricz Zs. krt. 22, 4032 Debrecen, Hungary

2. University of Debrecen, Faculty of Medicine, Department of Laboratory Medicine, Nagyerdei krt. 98, 4032 Debrecen, Hungary

3. Institute of Immunology, Rikshospitalet, Oslo University Hospital, Sognsvannsveien 20, 0372 Oslo, Norway

Abstract

The aim of this study was to determine the clinical, serological, and genetic features of anti-Jo-1 positive antisynthetase patients followed by a Hungarian single centre to identify prognostic markers, which can predict disease phenotypes and disease progression. It was a retrospective study using clinical database of 49 anti-Jo-1 positive patients. 100% of patients exhibited myositis, 73% interstitial lung disease, 88% arthritis, 65% Raynaud’s phenomenon, 43% fever, 33% mechanic’s hand, and 12% dysphagia. We could detect significant correlation between anti-Jo-1 titer and the CK and CRP levels at disease onset and during disease course. HLA DRB1⁎03 positivity was present in 68.96% of patients, where the CK level at diagnosis was significantly lower compared to the HLA DRB1⁎03 negative patients. HLA DQA1⁎0501-DQB1⁎0201 haplotype was found in 58.62% of patients, but no significant correlation was found regarding any clinical or laboratory features. Higher CRP, ESR level, RF positivity, and the presence of fever or vasculitic skin lesions at the time of diagnosis indicated a higher steroid demand and the administration of higher number of immunosuppressants during the follow-up within anti-Jo-1 positive patients. The organ involvement of the disease was not different in HLA-DRB1⁎0301 positive or negative patients who were positive to the anti-Jo-1 antibody; however, initial CK level was lower in HLA-DRB1⁎0301 positive patients. Distinct laboratory and clinical parameters at diagnosis could be considered as prognostic markers.

Funder

Debreceni Egyetem ÁOK Research Fund

Publisher

Hindawi Limited

Subject

General Immunology and Microbiology,General Biochemistry, Genetics and Molecular Biology,General Medicine

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