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Table 1 Environmental and clinical associations of autoantibody subgroups

From: Adult and juvenile dermatomyositis: are the distinct clinical features explained by our current understanding of serological subgroups and pathogenic mechanisms?

Myositis-specific autoantibodya

Antibody target

Environmental/genetic associations

Frequency in juvenile IIM (%)

Juvenile disease features

Frequency in adult IIM (%)

Adult disease features

Jo1, PL-7, PL-12, EJ, OJ, KS, Ha, Zo [39]

tRNA synthetases: catalyse amino acid binding to their cognate RNAs [39]

Onset in spring [74, 75]

1-5 [43, 44]

Rare [40, 41]

40 [39]

Anti-synthetase syndrome [39, 41]

Mi2

Nucleosome remodelling deacetylase complex (NuRD) [39]

UV light exposure [76]

4-10 [39, 43, 46]

Classic DM, mild disease [43, 46]

20 [39, 45]

Classic DM, mild disease [39]

P155 - TIF1γ/TRIMM33 (transcriptional intermediary factor 1)

Nuclear factor: acts as ubiquitin ligase for SMAD4. SMAD4 contributes to TGFβ signalling, suppressing cell growth [68]

Juvenile disease trend towards late winter birth [77]

23 [50]

Severe cutaneous disease [50]

13-21 [47]

Severe cutaneous disease.

Malignancy in 50% [48, 49]

P140 - NXP2/MJ (nuclear matrix protein 2)

Nuclear transcriprion factor involved in activation and localisation of tumour suppressor gene p53 [69]

In adults high prevalence found in Italian cohort [55]

11-23 (personal data, [52, 53]

Severe disease. Calcinosis [52, 53]

1.6-30 [50, 51]

Rare in most adult populations

Possibly malignancy

Possibly calcinosis [54, 55]

P140 - MDA5 (melanoma differentiation associated gene 5)

Cyoplasmic RNA helicase involved in inate immunity viral infection response [56]

Increased prevalence in Eastern Asia. Rising frequency in central Japan [56–58, 78]

7 (personal data)

RP-ILD - in Japanese cohort Ulceration [62]

13-35 [56, 58]

Amyopathic DM Characteristic cutaneous findings, ulceration RP-ILD [56, 58]

SAE (sumo-activating enzyme)

Involved in post-transcriptional modification [39, 63]

 

<1% (personal data)

Very rare

8.4 [63]

Initially amyopathic disease

Dysphagia [63]

SRP (signal recognition peptide)

Cytoplasmic protein involved protein recognition and translocation [39]

Onset in autumn [74, 75]

 

Rare [43, 44, 66]

5 [39]

Necrotising myopathy [64, 65]

  1. aJo1, histydly tRNA synthetase; PL-7, threonyl tRNA synthetase; Pl-12, alanyl tRNA synthetase; EJ, glycl tRNA synthetase; OJ, isoleucyl tRNA synthetase; KS, asparaginyl tRNA synthetase; Ha, tyrosyl tRNA synthetase; Zo, phenylalanyl tRNA synthetase. DM, dermatomyositis; IIM, idiopathic inflammatory myopathy; RP-ILD, rapidly progressive interstitial lung disease; TGF, transforming growth factor.