Chair Medical Sciences/Immunology Athens Academy Athens Greece.
Published: August 2020
AI Article Synopsis
Article Abstract
Respiratory complaints alone or in association with musculoskeletal complaints can be the predominant presenting feature of antisynthetase syndrome. Therefore, antibodies to cellular antigens should be evaluated in such clinical settings.
Objective: To summarize the clinical characteristics of 57 patients diagnosed with anti-glycyl tRNA synthetase (anti-EJ) positive antisynthetase syndrome (ASS), a subtype of anti-glycyl tRNA positive ASS, complicated by interstitial lung disease (ILD), and to investigate the factors asso-ciated with ILD recurrence.
Methods: A retrospective analysis was conducted on the clinical data of 57 anti-EJ positive ASS patientswho were treated at the First Affiliated Hospital of Nanjing Medical University from January 1, 2020 to June 30, 2024. The data collected included demographic information, clinical characteristics, laboratory test results, chest CT findings, and pulmonary function tests.
- The study aimed to examine the clinical and immune system characteristics of patients with anti-synthetase syndrome (ASS) who also have rheumatoid arthritis (RA) compared to those without RA.
- Out of 104 ASS patients with arthritis, 23.1% were diagnosed with RA and displayed notable differences such as higher rates of rapidly progressive interstitial lung disease, more tender and swollen joints, and increased bone erosion compared to the non-RA group.
- The overlap group (ASS with RA) also had significantly altered immune cell profiles, including decreased regulatory T cells and B cells, as well as higher levels of certain inflammatory markers, leading to a lower rate of complete clinical response.
Interstitial lung disease (ILD) frequently occurs in adults with idiopathic inflammatory myopathies, particularly those with antisynthetase syndrome and anti-MDA5 antibodies, manifesting in various severities from mild to rapidly worsening symptoms.
Common symptoms include lung issues along with myositis, skin lesions, arthritis, and Raynaud’s phenomenon, but 16-65% may have lung problems as the only indication.
Treatment typically involves immunosuppression, combining therapies for aggressive cases, and possibly lung transplantation for severe cases, although short-term mortality is notably high in rapidly progressive disease associated with anti-MDA5 antibodies.
Objectives: To explore the association between the extent of CT abnormalities by quantitative imaging analysis (QIA) and clinical/physiological disease parameters in patients with antisynthetase syndrome associated interstitial lung disease (ARS-ILD).
Methods: We analysed 20 patients with antisynthetase antibodies and active ILD enrolled in the Abatacept in Myositis-Associated Interstitial Lung Disease study. High-resolution chest CT was obtained at weeks 0, 24 and 48 and QIA scored the extent of ground glass (quantitative score for ground glass), fibrosis (quantitative score for lung fibrosis, QLF) and total ILD (quantitative ILD, QILD).
