To report a case of triple association of juvenile systemic lupus erythematosus (SLE), juvenile dermatomyositis and urticarial vasculitis as well as a review of the relevant literature. A 12-year-old male patient diagnosed with overlap syndrome between SLE and juvenile dermatomyositis since 2004 evolved with erythematous plaques, which were compatible with an urticarial rash. Clinical, laboratory and histopathological findings indicated a diagnosis of urticarial vasculitis. The patient previously had a C1q deficiency. Using the established treatment with methylprednisolone (1 g/day for 3 days), increasing doses of deflazacort and introduction of a dapsone, as well as mycophenolate mofetil regimen, with the suspension of azathioprine resulted in complete resolution of skin lesions. Urticarial vasculitis can present in various diseases. In SLE, presentation of urticarial vasculitis in children is rarely found. The triple association of juvenile-onset SLE, juvenile dermatomyositis and urticarial vasculitis is unusual, and this is the first case described in literature.
The novel finding of elevated CRH expression solely in the affected skin deep dermis supports the notion of a cutaneous local dysfunction of the CRH-POMC axis in the pathogenesis of cutaneous SLE lesions.
ObjectivesTo analyse the impact of the concomitant use of corticosteroids on the treatment of patients with long-term rheumatoid arthritis in different clinical outcomes.MethodsA cross-sectional study was carried out in a sample of 100 patients with rheumatoid arthritis attended at specialised centres in rheumatology in Florianópolis. Validated instruments were used to evaluate the disease activity (DAS-28 VHS, DAS-28 PCR, SDAI and CDAI), functional ability (HAQ-DI) and quality of life (SF-12 and SF-6D). Statistical analysis was performed using qui-square test (Pearson), Fisher’s exact test, Student’s t-test, seeking to study the association between the independent variables and the use of corticosteroids.ResultsThe mean disease duration of the patients was 15.1±8.7 years, which of, 25% used corticosteroids, with an average dose of 6.7±3.9 mg/day. The mean of the physical component summary (PCS) of SF-12 increased significantly by 1.87 points (47.72±3.23 vs 45.85±2.52 p=0.013) in patients who used corticosteroids, on the other hand, in the mental component summary (MCS) we observed a reduction of the mean by 1.5 points (57.91±3.68 vs 56.41±2.98 p=0.046) in these patients. In the association between the use of corticosteroids with ICADs, HAQ and SF-6D no differences were found independent of the synthetic and/or biological DMARD used.ConclusionsUse of corticosteroids in different treatment strategies is not associated with an increase in the frequency of clinical remission in patients with rheumatoid arthritis. In patients using corticosteroids there was a better quality of life evaluated by the PCS, but not by the MCS.Disclosure of InterestNone declared
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