Background Juvenile-onset systemic lupus erythematosus (JSLE) is multisystemic, autoimmune disease that is characterised by widespread immune dysregulation, formation of autoantibodies and immune complexis, resulting in inflammation of blood vessels and connective tissue with potential damage to variety of organs. Antinuclear antibodies (ANA) are present in the sera of 95-98% children with JSLE. Anti-double stranded DNA antibodies (Anti-dsDNA) are highly specific for JSLE and are present in about 61-93% children with active disease, especially active nephritis. Anti-Smith antibodies are also highly specific for JSLE, but they are detected in only about 50% of patients. As a part of disease presentation, many children diagnosed with JSLE have skin and oral lesions. Mucocutaneous lesions, as defined by the American College of Rheumatology (ACR) in 1997, include malar rash, discoid rash, photosensitivity and oral ulcers. In order to avoid delay in diagnosis it is very important to understand mucocutaneous signs and symptoms of lupus in children.
Objectives To characterize mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus and to study it's associations with autoantibodies.
Methods A single-center cohort study of all patients with newly diagnosed JSLE seen over an 10-year period was performed. All patients fulfilled the clinical and laboratory criteria of the American College of Rheumatology (ACR). Serum autoantibody profiles and extensive list of mucocutaneous manifestation were evaluated.
Results A total of twelve patients during period of January 2005 to January 2015 were identified. All had positive ANA, Direct Coombs test, significantly reduced C3 and C4 and high titres of Anticardiolipin antibodies IgG. Eleven patients (91%) had high titers of ANTIdsDNA. Two patients (16.6%) was identified as having discoid lupus and it was corelated with negative sera ENA- 6 profile. Two children (16,6%) had positive Anti SSA/Anti SSB and it was correlated with clinical findings of livedo reticularis. Nine patients (74%) had malar rash and eight (66%) had oral ulcers, but we were not able to demonstrate specific correlation with autoantibody profiles in this subset of patients.
Conclusions Patients with JSLE commonly present with mucocutaneous manifestations, and it is therefore important to recognize the lesions to make an accurate diagnosis. In the consecutive cohort of patients with JSLE we have identified association of specific autoantibody to mucucotaneous lesions that may have clinical, therapeutic and prognostic implications.
Chiewchengchol D, Murphy R, Morgan T, Edwards SW, Leone V, Friswell M, et al. Mucocutaneous manifestations in a UK national cohort of juvenile-onset systemic lupus erythematosus patients. Rheumatology (Oxford) 2014;53:1504.
Chiewchengchol et al. Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature. Pediatric Rheumatology 2015, 13:1
Disclosure of Interest None declared
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