Anti‐Jo1 autoantibodies, from clinic to the bench

Galindo-Feria, A. S.; Horuluoglu, Begum; Lundberg, Ingrid E.

doi:10.1002/rai2.12035

Cited by 7 publications

(1 citation statement)

References 124 publications

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“…ASyS represents a subtype that is characterized by autoantibodies targeting aminoacyl transfer ribonucleic acid synthetases, myositis, interstitial lung disease and arthritis. Multiple studies indicate a possible role of anti-Jo-1 autoantibodies in the pathogenesis of ASyS [ 12 ]. For example, a correlation between anti-Jo-1 antibody levels and disease severity has been reported [ 13 , 14 ] suggesting B-cell/antibody-directed treatment approaches for ASyS to reduce circulating autoantibodies.…”

Section: Discussionmentioning

confidence: 99%

Secondary immune-mediated thrombotic thrombocytopenic purpura in idiopathic inflammatory myopathy: a case-based review

et al. 2022

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Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a potentially fatal acquired thrombotic microangiopathy syndrome that frequently develops in the context of infectious diseases or systemic autoimmune conditions including connective tissue diseases. We report the case of a 42-year-old female suffering from severe iTTP associated with anti-Jo-1 positive antisynthetase syndrome, which was successfully treated with combination therapy of intravenous immune globulin, rituximab and plasma exchange. Based on a systematic review of the literature, two additional cases of idiopathic inflammatory myopathy-associated iTTP (secondary iTTP) were identified. In conclusion, iTTP may be a rare complication of IIM that clinicians should consider in cases of marked thrombocytopenia. Further work-up of this finding should include a peripheral blood smear (schistocyte count) and ADAMTS13 activity. The concomitant manifestation of these autoimmune conditions may require intensive immunosuppressive therapy.

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Section: Discussionmentioning

confidence: 99%

Secondary immune-mediated thrombotic thrombocytopenic purpura in idiopathic inflammatory myopathy: a case-based review

et al. 2022

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Anti‐PL‐12 anti‐synthetase syndrome manifesting with multiple digital ischemia: Case report & review of the literature

Doumeth,

Petrinec,

Chaudhary

et al. 2024

Clinical Case Reports

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Key Clinical MessageAcute digital ischemia is a rare manifestation of anti‐synthetase syndrome in the absence of Raynaud's phenomenon. A high index of suspicion may result in early diagnosis and better clinical outcomes.AbstractA 61‐year‐old male patient was admitted to the hospital for worsening arthralgias with morning stiffness lasting hours, as well as left sided headaches, and jaw pain while eating. He had significant weight loss and subjective fever at home. Multiple fingers and toes were noted to be ischemic. His laboratory workup was pertinent for significantly elevated inflammatory markers, and mild Creatinine kinase elevation. Chest imaging and later lung biopsy were notable for organizing pneumonia. Conventional angiogram showed evidence of significant digital disease without collaterals. Subsequent autoimmune screening tests with extended myositis‐specific and myositis‐associated panels revealed a strongly positive anti‐PL‐12 antibody and moderately positive anti‐ SSA‐52KD IgG ab. After ruling out infectious etiologies and malignancy, anti‐synthetase syndrome (ASyS) diagnosis was considered in the presence of ischemic digits, organizing pneumonia, polyarthralgia, constitutional symptoms, increased inflammatory markers and positive antibodies. The patient was treated with high dose prednisone and mycophenolate mofetil along with amlodipine and sildenafil for digital vasodilation. Acute digital ischemia may be the first manifestation of ASyS with ILD. A high index of suspicion is warranted for early diagnosis and better outcomes.

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Healthcare utilization and unmet needs of patients with antisynthetase syndrome: An international patient survey

Weiss,

Holzer,

Muehlensiepen

et al. 2023

Rheumatol Int

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Antisynthease syndrome (ASSD) is a rare, complex and understudied autoimmune disease. Internet-based studies can overcome barriers of traditional on-site research and are therefore very appealing for rare diseases. The aim of this study was to investigate patient-reported symptoms, diagnostic delay, symptoms, medical care, health status, working status, disease knowledge and willingness to participate in research of ASSD patients by conducting an international web-based survey. The multilingual questionnaire was created by an international group of rheumatologists and patients and distributed online. 236 participants from 22 countries completed the survey. 184/236 (78.0%) were female, mean age (SD) was 49.6 years (11.3) and most common antisynthetase antibody was Jo-1 (169/236, 71.6%). 79/236 (33.5%) reported to work full-time. Median diagnostic delay was one year. The most common symptom at disease onset was fatigue 159/236 (67.4%), followed by myalgia 130/236 (55.1%). The complete triad of myositis, arthritis and lung involvement verified by a clinician was present in 42/236 (17.8%) at disease onset and in 88/236 (37.3%) during the disease course. 36/236 (15.3%) reported to have been diagnosed with fibromyalgia and 40/236 (16.3%) with depression. The most reported immunosuppressive treatments were oral corticosteroids 179/236 (75.9%), followed by rituximab 85/236 (36.0%). 73/236 (30.9%) had received physiotherapy treatment. 71/236 (30.1%) reported to know useful online information sources related to ASSD. 223/236 (94.5%) were willing to share health data for research purposes once a year. Our results reiterate that internet-based research is invaluable for cooperating with patients to foster knowledge in rare diseases.

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Anti‐Jo1 autoantibodies, from clinic to the bench

Cited by 7 publications

References 124 publications

Secondary immune-mediated thrombotic thrombocytopenic purpura in idiopathic inflammatory myopathy: a case-based review

Secondary immune-mediated thrombotic thrombocytopenic purpura in idiopathic inflammatory myopathy: a case-based review

Anti‐PL‐12 anti‐synthetase syndrome manifesting with multiple digital ischemia: Case report & review of the literature

Healthcare utilization and unmet needs of patients with antisynthetase syndrome: An international patient survey

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