Evans Syndrome at Childhood-Onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Study.

BACKGROUND: Evans syndrome (ES) in childhood-onset systemic lupus erythematosus (cSLE) patients has been rarely reported and limited to small populations. PROCEDURES: A retrospective multicenter cohort study (Brazilian cSLE group) was performed in 10 Pediatric Rheumatology services including 850 patients with cSLE. ES was assessed at disease diagnosis and defined by the combination of immune thrombocytopenia and autoimmune hemolytic anemia. RESULTS: ES was observed in 11 of 850 (1.3%) cSLE patients. The majority of them had hemorrhagic manifestations (91%) and active disease (82%). All patients with ES were hospitalized and none died. Comparisons of cSLE patients with and without ES at diagnosis revealed similar frequencies of female gender, multiorgan involvement, autoantibodies profile, and low complement (P > 0.05). Patients with ES had a lower frequency of malar rash (9% vs. 53%, P = 0.003) and musculoskeletal involvement (18% vs. 69%, P = 0.001) than those without this complication. The frequencies of intravenous methylprednisolone (82% vs. 43%, P = 0.013) and intravenous immunoglobulin use (64% vs. 3%, P < 0.0001) were significantly higher in the ES group, with similar current prednisone dose between groups (1.1 [0.76-1.5] vs. 1.0 mg/kg/day [0-30], P = 0.195). CONCLUSIONS: Our large multicenter study identified ES as a rare and severe initial manifestation of active cSLE with good outcome. Diagnosis is challenging due to the lack of typical signs and symptoms of lupus and the requirement to exclude infection and primary immunodeficiency.

Condyloma acuminatum by human papilloma virus infection in childhood-systemic lupus erythematosus patients.

INTRODUCTION: Infections are frequent in childhood-systemic lupus erythematosus (C-SLE) patients, including human papillomavirus (HPV). HPV infection may cause genital and anal warts named condyloma acuminatum (CA). To our knowledge, none case was reported and the prevalence of CA in C-SLE population was not performed. CASE REPORTS: From January 1983 to May 2012, 5,682 patients were followed at the Pediatric Rheumatology Unit from of our University Hospital and 289 (5%) of them met the American College of Rheumatology classification criteria for C- SLE. Four (1.4%) of our female patients had CA. The median age at diagnosis was 13 years. Three of them were sexually active and all of them had active disease and had high risk HPV anogenital warts. Pap smears showed low-grade squamous intraepithelial lesion, guided biopsies identified chronic cervicitis, vulvar, vaginal, anal and/or cervix intraepithelial neoplasia. All of them were under corticosteroids and immunosuppressive drugs. The visible genital warts lesions were eradicated. DISCUSSION: Our patients requires rigorous gynecologic follow-up due to the severe anogenital dysplasia. HPV vaccine should be indicated in all C-SLE prior to sexual activity.