Pediatric rheumatology

Association of anti-TPM4 autoantibodies with vasculopathic cutaneous manifestations in juvenile dermatomyositis

Rheumatology (Oxford)Published Cross-sectional study

Authors' conclusion

Anti-TPM4 autoantibodies are detected frequently in children with JDM and are novel myositis-associated autoantibodies. Their presence correlates with vasculopathic and other cutaneous manifestations of JDM that may be indicative of more refractory disease.

Study context

Population
Children with JDM, polyarticular JIA and healthy children
Participants
63 / 50 / 40
Exposure
JDM vs polyarticular JIA (pJIA) vs healthy children (HC); JDM with vs without anti-TPM4 autoantibodies
  • JDMn = 63
  • pJIAn = 50
  • HCn = 40reference
Outcome
Presence of anti-TPM4 autoantibodies (ELISA) and correlation with clinical features of JDM
Methodological detailsELISA, Western blotting, TPM4 protein expression in cultured normal human dermal microvascular endothelial cells (ECs) shown by Western blotting
  • ELISA
  • Western blotting
  • TPM4 protein expression in cultured normal human dermal microvascular ECs shown by Western blotting

Main finding

As reported in the abstract

Anti-TPM4 autoantibodies detected in plasma

JDM vs pJIA: P < 0.0001 · JDM vs HC: P < 0.0001

Observational data: associations, not effects.

Further findings

Clinical features associated with anti-TPM4 autoantibodies in JDM

Cutaneous ulcers (53%)
P = 0.02
Shawl sign rash (47%)
P = 0.03
Mucous membrane lesions (84%)
P = 0.04
Subcutaneous edema (42%)
P < 0.05

Treatment associated with anti-TPM4 autoantibodies in JDM

Use of intravenous steroids and IVIG therapy (significant correlation)
both P = 0.01
Higher total number of medications received with anti-TPM4 autoantibodies
P = 0.02

Original publication

Association of anti-TPM4 autoantibodies with vasculopathic cutaneous manifestations in juvenile dermatomyositis. Rheumatology (Oxford). Published 5 May 2023. doi:10.1093/rheumatology/kead203

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