Antisynthetase syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: M. Hassan, M.B.B.S

Synonyms and keywords:

Overview

Antisynthetase syndrome, an uncommon autoimmune disorder, is characterized by antibodies against aminoacyl-transfer ribonucleic acid synthetases, with various combinations of three cardinal manifestations of interstitial lung disease (ILD), polymyositis, and arthritis. Other features of antisynthetase syndrome may include pyrexia of unknown origin, mechanic's hand (hyperkeratotic skin changes along the lateral surfaces of the digits) and Raynaud's phenomenon.

Till date, nine different anti-aminoacyl t-RNA synthetase (ARS) antibodies have been identified to play a role in the developement of antisynthetase syndrome.

Historical Perspective

In 1990, Marguerie et al and coworkers first described antisynthetase syndrome as a triad of polymyositis, interstitial lung disease (ILD), and the presence of myositis-specific autoantibodies to aminoacyl-transfer ribonucleic acid synthetases.

Differentiating Antisynthetase syndrome from other Diseases

Antisynthetase syndrome may be misdiagnosed as idiopathic interstitial lung disease (ILD) or inflammatory myopathy. This is due to the rarity and lack of awareness of the disease, as well as due to the lack of

Most cases of AS are either misdiagnosed as idiopathic ILD or inflammatory myopathy not only due to the lack of awareness of the disease entity but also due to the lack of facilities for detection of anti-ARS antibodies.

Diagnosis

Diagnostic Criteria

In 2010, Connors et al proposed a formal criteria for the diagnosis of antisynthetase syndrome. This criteria proposed that all patients must have a presence of an anti-aminoacyl tRNA synthetase antibody in addition to one or more of the following clinical features: mechanic’s hands, Raynaud’s phenomenon, myositis, ILD, arthritis, and/or unexplained fever.

In 2011, Solomon et al proposed alternative criteria, which is more strict. This criteria requires the presence of anti-aminoacyl tRNA synthetase antibody plus two major or one major and two minor criteria.

Major criteria includes: Interstitial Lung Disease (not attributable to another cause), and Polymyositis or dermatomyositis.

Minor criteria includes: arthritis, Raynaud’s phenomenon, and mechanic’s hands.

Connors et al (2010) criteria
Required: Presence of an anti-aminoacyl tRNA synthetase antibody
PLUS one or more of the following clinical features: 1) Raynaud’s phenomenon 2) Arthritis 3) Interstitial lung disease 4) Fever (not attributable to another cause) 5) Mechanic’s hands (thickened and cracked skin on hands, particularly at fingertips)

Solomon et al (2011) criteria
Required: Presence of anti-aminoacyl tRNA synthetase antibody
PLUS two major or one major and two minor criteria: Major: 1) Interstitial Lung Disease (not attributable to another cause) 2) Polymyositis or dermatomyositis Minor: 1) Arthritis 2) Raynaud’s phenomenon 3) Mechanic’s hands

Treatment

Medical Therapy

Various immunosuppressive agents, such as azathioprine, mycophenolate mofetil, and/or tacrolimus, are used in the treatment of antisynthetase syndrome. Additionally, corticosteroids may be added for management of the myositis and pulmonary manifestations of anti-synthetase syndrome.