We report the case of an 80-year-old Caucasian man with PL-12 antibody positive antisynthetase syndrome. He presented with progressive dyspnoea and weight loss, later developing dysphagia, mild proximal muscle weakness and mild sicca symptoms. Investigations revealed interstitial lung disease, inflammatory myopathy and an immunology profile consistent with PL-12 antisynthetase syndrome. Prednisolone and cyclophosphamide resulted in a significant improvement of all his symptoms.
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http://dx.doi.org/10.1136/bcr-2018-226119 | DOI Listing |
Case Rep Pulmonol
January 2025
Prisma Health, University of South Carolina-School of Medicine, Columbia, South Carolina, USA.
Diffuse alveolar hemorrhage (DAH) is a potentially life-threatening condition which can present with hemoptysis, diffuse alveolar infiltrates, anemia, and hypoxic respiratory failure. Antisynthetase syndrome (AS) is a rare autoimmune disorder most often characterized by nonerosive arthritis, proximal muscle weakness with elevated muscle enzymes, Raynaud's phenomenon, hyperkeratosis of the digits (mechanic's hands), and interstitial lung disease. According to large population studies, AS has an annual incidence of 0.
View Article and Find Full Text PDFJ Rheumatol
January 2025
Floranne C. Ernste MD, Division of Rheumatology, Mayo Clinic College of Medicine and Science, Rochester, MN, USA.
Objective: Population-based epidemiology studies about antisynthetase syndrome (ASSD) are lacking. Our aims were to determine the incidence and prevalence of ASSD and assess malignancy risk among patients following ASSD diagnosis.
Methods: A retrospective, population-based cohort of adults with incident ASSD residing in Olmsted County, Minnesota, in 1998-2019 was assembled.
Arthritis Res Ther
January 2025
Rheumatology Department, Hospital Universitario Reina Sofía, Universidad de Córdoba, Instituto Maimónides de Investigación Biomédica de Córdoba (IMIBIC), Córdoba, Spain.
Objective: To evaluate the main outcomes of disease activity and their association with other measures of activity, damage, and quality of life in patients with idiopathic inflammatory myopathy (IIM) according to time since diagnosis and positivity to antisynthetase autoantibodies (ASAs).
Methods: Cross-sectional multicenter study within the Spanish Myo-Spain registry. Cases were classified as incident (≤ 12 months since diagnosis) and prevalent.
Front Pharmacol
December 2024
Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, Taiyuan, China.
CAR-T cell therapy, a cutting-edge cellular immunotherapy with demonstrated efficacy in treating hematologic malignancies, also exhibits significant promise for addressing autoimmune diseases. This innovative therapeutic approach holds promise for achieving long-term remission in autoimmune diseases, potentially offering significant benefits to affected patients. Current targets under investigation for the treatment of these conditions include CD19, CD20, and BCMA, among others.
View Article and Find Full Text PDFBeijing Da Xue Xue Bao Yi Xue Ban
December 2024
Department of Rheumatology and Immunology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China.
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.
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