Clinical profile and treatment outcomes in antisynthetase syndrome: a tertiary centre experience

Abstract

Objectives The aim was to describe the clinical profile and outcomes in patients with antisynthetase syndrome (ASS) from a tertiary care centre. Methods The clinical data and investigations of all patients classified as ASS by Connors criteria over 5 years were recorded, and they were followed up prospectively. The median (interquartile range) was used for descriptive statistics. Clinical variables between the Jo-1 and non-Jo-1 groups and between patients with and without anti-Ro52 antibodies were compared using the χ2 test. Survival analysis was done using the log rank test. Results The 28 patients (23 females) had a median age of 42.5 (34.8–52.3) years, with a disease duration of 1.75 (0.6–3.8) years at diagnosis, and had a follow-up of 2 (0.25–4.25) years. Seronegative arthritis was seen in 23 of 28 patients. Non-specific interstitial pneumonia was seen in 19 patients with interstitial lung disease (ILD). Antibodies to Jo-1 (n = 17) were more frequent than non-Jo-1 antibodies (n = 11; five anti-PL-12, four anti-PL-7 and two anti-EJ). There was no significant difference in the prevalence of myositis (P = 0.07) or ILD (P = 0.11) between groups. Anti-Ro52 antibodies were more frequently found in the non-Jo-1 group (P = 0.006, ϕ = 0.51). A partial or complete improvement with treatment was seen in three-quarters of the patients. Five patients succumbed to the illness. Better survival was seen in the Jo-1 group (P = 0.05). Conclusion The most typical presenting manifestation of ASS in our cohort was isolated seronegative arthritis. Non-specific interstitial pneumonia was the commonest ILD pattern. Patients with antibodies to Jo-1 had better survival compared with non-Jo-1. The non-Jo-1 aminoacyl-transfer RNA synthetases had a strong association with anti-Ro52 antibodies.

Dissociating Autoantibody Responses against Ro52 Antigen in Patients with Anti-Synthetase or Anti-MDA5 Antibodies

“…Based on a targeted literature review using PubMed, previous studies utilized EIA or LIA alone for detecting anti-Ro52 antibodies and reported the prevalence of anti-Ro52 antibodies as 21.4-65.9% in patients with anti-synthetase antibodies [13][14][15][16][17][18][36][37][38][39][40][41] (Supplementary Table S1) and 31.5-74.7% in those with anti-MDA5 antibodies [13,[19][20][21][22][23][24] (Supplementary Table S2), respectively, which are compatible with the prevalence in the present study.…”

“…Table S2: Previous studies reporting the prevalence and clinical significance of anti-Ro52 antibodies in patients with anti-MDA5 antibodies. References [13][14][15][16][17][18][19][20][21][22][23][24][36][37][38][39][40][41] are cited in the Supplementary Materials. Informed Consent Statement: Informed consent was obtained from all subjects involved in the study.…”

A Review of Antisynthetase Syndrome-Associated Interstitial Lung Disease

“…• Anti-Jo-1 (Anti-Histidyl tRNA Synthetase) Anti-Jo-1 is the most common ASAb, occurring in roughly 30-60% of ASyS patients depending on the cohort studied [47,49,73,74]. It is associated more frequently with polyarticular arthritis and muscle involvement compared to the other ASAbs [47,56,75].…”

Idiopathic inflammatory myopathies: one year in review 2022

“…According to real-life data from the Indian MyoCite cohort (116), methotrexate (MTX) is the most employed drug, particularly in children, in whom it is prescribed as first-line treatment in 90% of cases. Azathioprine (AZA) and mycophenolate mofetil (MMF), as well as CYC and RTX, are preferred in the case of ILD and ASS (117). Anti-CD20 agents have the highest retention rate relapse-free survival (116).…”

“…Anti-CD20 agents have the highest retention rate relapse-free survival (116). In ASS, MTX is generally restricted to managing arthritis, and AZA is preferred in the case of myositis (117). Among synthetic immunosuppressants, MMF is an inactive drug hydrolysed to mycophenolic acid (MPA).…”