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.
Highlights
Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune connective tissue diseases characterized by chronic muscle inflammation resulting in progressive symmetrical muscle weakness with elevated serum levels of muscle enzymes, electromyographic abnormalities, and characteristic mononuclear inflammatory infiltrates in muscle biopsy specimens
The organ involvement of the disease was not different in Human leukocyte antigen (HLA)-DRB1∗0301 positive or negative patients who were positive to the anti-Jo-1 antibody; initial creatine kinase (CK) level was lower in HLA-DRB1∗0301 positive patients
We found that HLA-DRB1∗03 positivity was associated with lower initial CK level in patients with anti-Jo-1 positivity; no other clinical parameters were influenced by the presence or absence of HLA-DRB1∗03 genotype (Table 1)
Summary
Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune connective tissue diseases characterized by chronic muscle inflammation resulting in progressive symmetrical muscle weakness with elevated serum levels of muscle enzymes, electromyographic abnormalities, and characteristic mononuclear inflammatory infiltrates in muscle biopsy specimens. Autoantibodies are of great importance for the diagnosis of many systemic autoimmune rheumatic diseases, including IIMs. Myositis-associated autoantibodies (MAAs) are those that appear in myositis overlap syndromes and in other connective tissue diseases, which correlate with certain clinical and/or pathophysiological conditions of myositis [2,3,4,5,6,7,8]. The myositis-specific autoantibodies (MSAs) are useful markers for clinical diagnosis, classification, and prediction of the prognosis of the IIM. Presence of anti-Jo-1 defines a distinct clinical phenotype, antisynthetase syndrome (ASS), which is characterized by poor prognosis, and multiple organ involvement, such as myositis, interstitial lung disease (ILD), arthritis, Raynaud’s phenomenon, mechanic’s hand, skin rashes, and fever [12]. New classification criteria for IIM were developed [13], where the presence of anti-Jo-1 antibody plays an important role in the scoring system with the highest score point
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