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Review
. 2011 Jan-Feb;37(1):100-9.
doi: 10.1590/s1806-37132011000100015.

Myositis-related interstitial lung disease and antisynthetase syndrome

[Article in English, Portuguese]
Joshua Solomon  1 , Jeffrey J Swigris, Kevin K Brown
Affiliations
Review

Myositis-related interstitial lung disease and antisynthetase syndrome

[Article in English, Portuguese]
Joshua Solomon et al. J Bras Pneumol. 2011 Jan-Feb.

Abstract

In patients with myositis, the lung is commonly involved, and the presence of anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies marks the presence or predicts the development of interstitial lung disease (ILD). A distinct clinical entity-antisynthetase syndrome-is characterized by the presence of anti-ARS antibodies, myositis, ILD, fever, arthritis, Raynaud's phenomenon, and mechanic's hands. The most common anti-ARS antibody is anti-Jo-1. More recently described anti-ARS antibodies might confer a phenotype that is distinct from that of anti-Jo-1-positive patients and is characterized by a lower incidence of myositis and a higher incidence of ILD. Among patients with antisynthetase syndrome-related ILD, the response to immunosuppressive medications is generally, but not universally, favorable.

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Figures

Figure 1
Figure 1
Slice through the lower lung zones from an HRCT scan of a patient with antisynthetase syndrome and interstitial lung disease showing reticular and patchy ground-glass opacities in the lung bases.
Figure 2
Figure 2
Lung biopsy from a patient with antisynthetase syndrome and interstitial lung disease showing cellular interstitial infiltrate and organizing pneumonia (arrow).
Chart 1
Chart 1
Proposed criteria for the diagnosis of antisynthetase syndrome.
Chart 2
Chart 2
Anti-aminoacyl-tRNA synthetase antibodies and associated clinical phenotypes.
Chart 3
Chart 3
Treatment of patients with antisynthetase syndrome and interstitial lung disease.
See this image and copyright information in PMC

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MeSH terms

Supplementary concepts