Antisynthetase Syndrome

Abstract

Autoantibodies to eight of the aminoacyl-transfer RNA synthetases-the most well-recognized of which is anti-histidyl (Jo-1)-have all been implicated in the pathogenesis of antisynthetase syndrome (AS). AS is characterized by varying degrees of interstitial lung disease, myositis, arthropathy, fever, Raynaud's phenomenon, and mechanic's hands, and the morbidity and mortality of the disease are usually linked to the pulmonary findings. The value of a lung biopsy in AS cannot be overemphasized, as it serves to describe the underlying etiology of the interstitial lung disease, guide therapy, and estimate prognosis. Muscle disease shares many clinical features of polymyositis, yet histologically, the inflammatory involvement resembles that of dermatomyositis. Because inflammatory arthritis mimics rheumatoid arthritis, AS should be considered in atypical cases. Corticosteroids are the mainstay of acute therapy, although treatment often requires immunosuppressant medications such as cyclophosphamide, azathioprine, mycophenolate mofetil, cyclosporine, tacrolimus, or rituximab.

Inflammatory Myopathies with Cutaneous Involvement: from Diagnosis to Therapy

“…The clinical and laboratory features of anti-synthetase syndrome of nonerosive arthritis, interstitial lung disease, Raynaud's phenomenon, mechanic's hand cutaneous lesions, carpal tunnel syndrome, and antisynthetase antibodies (anti Jo-1, PL-7; PL-12; OJ, EJ, KS and ZO) are found in sera. 53,54 …”

Antisynthetase syndrome: a case report

“…It has a reported prevalence of 1.5 per 100,000 populations. The hallmark of ASS are antibodies against aminoacyl-transfer RNA synthetase of which the most frequent is the anti-Jo-1 antibody which is present in approximately 80% of cases (1). Less commonly encountered antibodies in ASS are including anti-PL-7 and anti-EJ (2).…”

“…NSIP is the most frequently reported histological pattern in ASS and the presence of fibrosis (as opposed to cellular infiltrates) is an adverse predictor for response to immunosuppressive therapies (1-3). Joint symptoms are reported in around 60% of patients with ASS and are more common in anti-Jo-1 positive ASS patients however they are non-specific and often mimic more common inflammatory arthropathies such as rheumatoid arthritis (1). Myositis is reported in 30-60% of ASS patients and anti-Jo-1 positivity can predate clinical myositis (1).…”

“…Myositis is reported in 30-60% of ASS patients and anti-Jo-1 positivity can predate clinical myositis (1). Most treatment regimens for ASS describe a combination of glucocorticosteroids and other immunosuppressant such as cyclophosphamide with pulmonary disease response dictating treatment duration (1).…”

“…Antisynthetase syndrome (ASS) is quite a rare condition and early diagnosis is the key in management (1)(2)(3).…”