Journal article
Association of anti-TPM4 autoantibodies with vasculopathic cutaneous manifestations in juvenile dermatomyositis
Rheumatology, Vol.62(11), pp.3757-3762
05/05/2023
DOI: 10.1093/rheumatology/kead203
PMCID: PMC10629777
PMID: 37144941
Abstract
Objectives
AECAs are detected in multiple forms of vasculitis or vasculopathy, including JDM. High levels of tropomyosin alpha-4 chain (TPM4) gene expression in cutaneous lesions and TPM4 protein expression in some endothelial cells (ECs) have been proven. Furthermore, the presence of autoantibodies to tropomyosin proteins have been discovered in DM. We therefore investigated whether anti-TPM4 autoantibodies are an AECA in JDM and are correlated with clinical features of JDM.
Methods
The expression of TPM4 protein in cultured normal human dermal microvascular ECs was investigated by Western blotting. Plasma samples from 63 children with JDM, 50 children with polyarticular JIA (pJIA) and 40 healthy children (HC) were tested for the presence of anti-TPM4 autoantibodies using an ELISA. Clinical features were compared between JDM patients with and without anti-TPM4 autoantibodies.
Results
Autoantibodies to TPM4 were detected in the plasma of 30% of JDM, 2% of pJIA (P < 0.0001) and 0% of HC (P < 0.0001). In JDM, anti-TPM4 autoantibodies were associated with the presence of cutaneous ulcers (53%; P = 0.02), shawl sign rash (47%; P = 0.03), mucous membrane lesions (84%; P = 0.04) and subcutaneous edema (42%; P < 0.05). Anti-TPM4 autoantibodies significantly correlated with the use of intravenous steroids and IVIG therapy in JDM (both P = 0.01). The total number of medications received was higher in patients with anti-TPM4 autoantibodies (P = 0.02).
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.
Details
- Title: Subtitle
- Association of anti-TPM4 autoantibodies with vasculopathic cutaneous manifestations in juvenile dermatomyositis
- Creators
- Rie Karasawa - St. Marianna University School of MedicineKazuo Yudoh - St. Marianna University School of MedicineToshiko Sato - St. Marianna University School of MedicineMegumi Tanaka - St. Marianna University School of MedicineSara E Sabbagh - Medical College of WisconsinWilly A Flegel - National Institutes of Health Clinical CenterAndrew L Mammen - National Institute of Arthritis and Musculoskeletal and Skin DiseasesJames N Jarvis - University at Buffalo, State University of New YorkLisa G Rider - National Institute of Environmental Health SciencesBita ArabshahiApril BinghamVictoria CartwrightRodolfo CurielMarietta M DeGuzmanBarbara Anne EberhardBarbara S EdelheitTerri H FinkelWilliam HannanMichael HenricksonAdam M HuberAnna JansenSteven J KleinBianca LangCarol B LindsleyGulnara MamyrovaFrederick W MillerStephen R MitchellKabita NandaPayam Noroozi FarhadiMurray H PassoDonald A PersonTova RonisAdam SchiffenbauerBracha ShahamMatthew L StollSangeeta H SuleIra N TargoffScott A VogelgesangRita VolochayevJennifer C WargulaPamela WeissChildhood Myositis Heterogeneity Study Group
- Resource Type
- Journal article
- Publication Details
- Rheumatology, Vol.62(11), pp.3757-3762
- Publisher
- Oxford University Press (OUP)
- DOI
- 10.1093/rheumatology/kead203
- PMID
- 37144941
- PMCID
- PMC10629777
- ISSN
- 1462-0324
- eISSN
- 1462-0332
- Language
- English
- Date published
- 05/05/2023
- Academic Unit
- Immunology; Internal Medicine
- Record Identifier
- 9984802192902771
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