Table 1

Environmental and clinical associations of autoantibody subgroups

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]


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.

Tansley et al. Arthritis Research & Therapy 2013 15:211   doi:10.1186/ar4198