Background Patients diagnosed with juvenile-onset systemic lupus erythematosus (jSLE) often have skin and oral lesions as part of their presentation. It is suggested, in literature, that mucocutaneous involvement in jSLE may be associated with some antibodies and systemic disease and often require treatment with systemic immunosuppressive drugs in order to achieve adequate disease control.
Objectives To assess mucocutaneous manifestations in jSLE and to study it's associations with clinical and immunological characteristics.
Methods Retrospective observational study was performed including consecutive patients with jSLE (disease onset before 16 years of age) followed in our Paediatric Rheumatology Unit. Clinical, demographic and laboratory characteristics were retrospectively collected by consulting the medical records. All patients fulfilled the clinical and laboratory criteria of the American College of Rheumatology (ACR). Patients with mucocutaneous manifestations were compared with others using Student t-test, Mann-Whitney test, Chi-square or Fisher test. (SPSS 23.0). Significance level was set as <0.05.
Results 38 patients were included, 92.1% (35) were female, with a mean age at diagnosis of 12.9 ± 3 years. Median period between onset of symptoms and diagnosis of SLE was 0.3 [0–1.2] years and median duration of follow-up was 14 [0.75–26].
84.3% had mucocutaneous manifestations, (78.9% malar rash, 5.2% discoid rash, 70.3% photossensibility and 39.5% ulcers). 78.9% of the patients also had musculoskeletal symptoms, 63.2% haematological manifestations, 50% had renal involvement, 21% serositis and 13.2% neuropsychiatric involvement.
Patients with mucocutaneous involvement had more frequently renal involvement (58% vs 14.2%, p=0.001), haematological manifestations (70.9% vs 28.5%, p=0.02), positive lupus anticoagulant (13.8% vs 4.3%, p=0.012), positive coombs test (59% vs 14.2%, p=0.04) and positive anti-SSa (35.4% vs 3.3%, p=0.04). SLEDAI and SLICC scores were comparable between the two groups.
Analysing by type of skin involvement, patients with malar rash had also more frequently haematological involvement (76.7% vs 12.5%, p=0.002) and it was associated with the presence of anti-SSa (33.3% vs 12.5%, p=0.04).
Patients with photossensibility had more frequently positive anti-SSa (34.5% vs 11.1%, p=0.029).
Two patients (5.2%) had discoid lupus and 15 (39.5%) had ulcers but it was not associated with any other clinical or immunological manifestation.
Conclusions Mucocutaneous are frequent manifestations in jSLE, and they are associated with active disease occurring frequently in patients with also haematological and renal manifestations. Anti-Ssa antibody is important in these patients as we have demonstrated it's association with some particularly skin manifestations.
Disclosure of Interest None declared