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Review
. 2021 Mar;9(5):433.
doi: 10.21037/atm-20-5175.

Dermatomyositis autoantibodies: how can we maximize utility?

Luqman Mushila Hodgkinson  1 , Tiffany Tingshuen Wu  1 , David Franklin Fiorentino  1
Affiliations
Review

Dermatomyositis autoantibodies: how can we maximize utility?

Luqman Mushila Hodgkinson et al. Ann Transl Med. 2021 Mar.

Abstract

The past 15 years has seen significant advances in the characterization of myositis-specific autoantibodies (MSAs) and their associated phenotypes in patients with dermatomyositis (DM). As more careful studies are performed, it is clear that unique combinations of clinical and pathological phenotypes are associated with each MSA, despite the fact that there is considerable heterogeneity within antibody classes as well as overlap across the groups. Because risk for interstitial lung disease (ILD), internal malignancy, adverse disease trajectory, and, potentially response to therapy differ by DM MSA group, a deeper understanding of MSAs and validation and standardization of assays used for detection are critical for optimizing diagnosis and treatment. Like any test, the diagnostic sensitivity and specificity of assays for various MSAs is not perfect. Currently tests for MSAs are helpful at minimum for a clinician to assess relative risk or contribute to diagnosis and perhaps counsel the appropriate patient about what to expect. With international standardization and larger studies it is likely that more antibody tests will make their way into formal schemata for diagnosis and actionable risk assessment in DM. In this review, we summarize key considerations for interpreting the clinical and pathologic associations with MSA in DM and identify critical gaps in knowledge and practice that will maximize their clinical utility and utility for understanding disease pathogenesis.

Keywords: Dermatomyositis (DM); autoantibodies; immunoassay.

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Conflict of interest statement

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/atm-20-5175). The series “Rheumatologic Skin Disease” was commissioned by the editorial office without any funding or sponsorship. DFF served as the unpaid Guest Editor of the series. The authors have no other conflicts of interest to declare.

Figures

Figure 1
Figure 1
Wide range of prevalence of major MSAs in various studies. Anti-TIF-1γ: low report: 7% (Japan, anti-TIF-1γ/anti-TIF-1α, IP) (16); high report: 41% (USA, IP) (86). Anti-MDA-5: low report: 0% (Hungary, IP) (87); high report: 37% (China, ELISA) (88). Anti-NXP-2: low report: 2% (Japan, IP) (42); high report: 30% (Italy, IP) (89). Anti-SAE-1/2: low report: 2% (Japan, IP) (90); high report: 10% (UK, IP) (48). Anti-Mi-2: low report: 2% (Japan, IP) (16); high report: 30% (India, LIA) (91). Anti-Jo-1: low report: 9% (Italy, LIA) (92); high report: 30% (USA, African Americans, IP) (93). MSAs, myositis-specific autoantibodies; IP, immunoprecipitation; ELISA, enzyme-linked immunosorbent assay.
Figure 2
Figure 2
Wide range of prevalence of malignancy in various studies. Anti-TIF-1γ: low report: 18% (USA, IP) (6); high report: 100% (Japan, anti-TIF-1γ/anti-TIF-1α, IP) (70). Anti-MDA-5: low report: 7% (China, ELISA) (77); high report: 29% (ELISA and immunoblot) (78). Anti-NXP-2: low report: 17% (Europe, IP) (18); high report: 38% (Japan, IP) (42). Anti-SAE-1/2: low report: 18% (UK, IP) (100); high report: 50% (Japan, ELISA and IP) (115). Anti-Mi-2: low report: 0% (Japan, IP) (116); high report: 6% (USA, ELISA) (37). Anti-Jo-1: low report: 0% (Hungary, immune serology otherwise unspecified) (117); high report: 15% (Japan, IP) (114). IP, immunoprecipitation; ELISA, enzyme-linked immunosorbent assay.
Figure 3
Figure 3
Wide range of prevalence of ILD in various studies. Anti-TIF-1γ: low report: 0% (Spain, IP, method of evaluating ILD unspecified) (118); high report: 20% (Japan, IP, chest radiography and high-resolution CT) (70). Anti-MDA-5: low report: 50% (USA, ELISA, pulmonary fibrosis seen on chest radiography or high-resolution CT) (74); high report: 100% (Japan, IP, chest radiography and high-resolution CT) (70). Anti-NXP-2: low report: 0% (Japan, IP, standard clinical criteria) (42); high report: 7% (USA, IP, percentage of FVC) (97). Anti-SAE-1/2: low report: 18% (UK, IP, high resolution CT scan) (100); high report: 71% (Japan, IP, method of evaluating ILD unspecified) (90). Anti-Mi-2: low report: 0% (India, LIA, high-resolution CT) (91); high report: 6% (Brazil, LIA, high-resolution CT) (127). Anti-Jo-1: low report: 56% (Japan, IP, chest radiograph and high-resolution CT) (114); high report: 86% (USA, ELISA, abnormalities on chest radiograph or high-resolution CT or biopsy) (128). ILD, interstitial lung disease; IP, immunoprecipitation; CT, computed tomography; LIA, line immunoblot assay.
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