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Table 1 Clinical Features Associated with Myositis Specific Autoantibodies

From: Myositis autoantibodies in a racially diverse population of children with idiopathic inflammatory myopathies

Myositis Specific Autoantibody

Anti-p155/140 [8]

Anti-MJ

Anti-synthetase (including anti-Jo-1)

Anti-Mi-2

Anti-MDA-5 [9]

Autoantigen Target

TIF-1

NXP-2

Histidyl-tRNA synthetase

Mi-2

CADM140

Frequency

18–30%

15–20%

2–4%

2–10%

6% (UK) – 38% (Japan)

JIIM subgroup (predominant)

JDM

JDM > JPM

JDM > Overlap CTD > JPM

JDM

JDM

Median age at onset (years)

7 (4–10)

6 (4–8)

14 (10–14)

11 (6–12)

6 (4–10)

Race/ethnicity

White

White

White>Black

Hispanic

Variable, case reports in Japan

Skin

Classic rash, photosensitive rash, cutaneous ulceration

Classic rash

Mechanics hands, Raynauds

Classic rash

Palmar papules, cutaneous ulceration

Weakness

Moderate

Severe

Mild/moderate

Mild

May be amyopathic

Muscle enzyme elevation

Low

Moderate

Moderate

High

Mild

Additional musculoskeletal features

Muscle atrophy

Muscle cramps, joint contractures

Arthritis/ arthralgias

Less Common

Arthritis

Major organ involvement

Less common

Dysphagia, GI ulceration

Interstitial lung disease

Less common

Interstitial lung disease

Complications

Calcinosis (30%), lipodystrophy

Highest rate of calcinosis (47%)

Less calcinosis (10%), lipodystrophy (33%)

Calcinosis (18%)

Calcinosis (27%)

Prognosis

Chronic course, low mortality

Chronic course, 1/3 monocyclic, low mortality

Highest risk of mortality

Best prognosis

Poor prognosis in Japan cohort

References

4,5,7,11

4,5,7

4,5,7

4,7

6,7, 9,10,12

  1. Abbreviations: Anti-TIFI, anti-p155/140, Anti-transcription intermediary factor 1 gamma, Anti-NXP2, anti-mj, Anti-nuclear matrix protein 2, anti- MDA5 anti-melanoma differentiation associated protein 5