Antisynthetase syndrome

WikiDoc Resources for Antisynthetase syndrome
Articles
Most recent articles on Antisynthetase syndrome Most cited articles on Antisynthetase syndrome Review articles on Antisynthetase syndrome Articles on Antisynthetase syndrome in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Antisynthetase syndrome Images of Antisynthetase syndrome Photos of Antisynthetase syndrome Podcasts & MP3s on Antisynthetase syndrome Videos on Antisynthetase syndrome
Evidence Based Medicine
Cochrane Collaboration on Antisynthetase syndrome Bandolier on Antisynthetase syndrome TRIP on Antisynthetase syndrome
Clinical Trials
Ongoing Trials on Antisynthetase syndrome at Clinical Trials.gov Trial results on Antisynthetase syndrome Clinical Trials on Antisynthetase syndrome at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Antisynthetase syndrome NICE Guidance on Antisynthetase syndrome NHS PRODIGY Guidance FDA on Antisynthetase syndrome CDC on Antisynthetase syndrome
Books
Books on Antisynthetase syndrome
News
Antisynthetase syndrome in the news Be alerted to news on Antisynthetase syndrome News trends on Antisynthetase syndrome
Commentary
Blogs on Antisynthetase syndrome
Definitions
Definitions of Antisynthetase syndrome
Patient Resources / Community
Patient resources on Antisynthetase syndrome Discussion groups on Antisynthetase syndrome Patient Handouts on Antisynthetase syndrome Directions to Hospitals Treating Antisynthetase syndrome Risk calculators and risk factors for Antisynthetase syndrome
Healthcare Provider Resources
Symptoms of Antisynthetase syndrome Causes & Risk Factors for Antisynthetase syndrome Diagnostic studies for Antisynthetase syndrome Treatment of Antisynthetase syndrome
Continuing Medical Education (CME)
CME Programs on Antisynthetase syndrome
International
Antisynthetase syndrome en Espanol Antisynthetase syndrome en Francais
Business
Antisynthetase syndrome in the Marketplace Patents on Antisynthetase syndrome
Experimental / Informatics
List of terms related to Antisynthetase syndrome

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 development 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

Imaging Findings

As antisynthetase syndrome is associated with interstitial lung disease, and therefore, high resolution computed tomography (HRCT) plays an important role in its work up, as well as to monitor the disease activity. Radiographic features seen on HRCT at the initial diagnosis include traction bronchiectasis, ground glass opacities and reticulation.

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. The choice of immunosuppressive agent is dependent on the practitioner and is based on the potential risks of therapy as no agent is superior to the other.

Prednisone and azathioprine or mycophenolate mofetil may be used in patients with anti-synthetase syndrome related ILD. In addition to that, tacrolimus may be added for rapid disease control in more severe cases of ILD.

In patients with severe progressive or refractory ILD, rituximab may be added; while cyclophosphamide is reserved for those with acute respiratory distress syndrome.

The physician must also been on the lookout for other co-morbid conditions associated with antisynthetase syndrome i.e. pulmonary hypertension and screening for malignancies.