Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSPFRANCA, Camila M. P.CAVALCANTE, Erica G.RIBEIRO, Aline S. M.OLIVEIRA, Guilherme T.LITVINOV, NadiaSILVA, Clovis A.2013-07-302013-07-302012ACTA REUMATOLOGICA PORTUGUESA, v.37, n.3, p.276-279, 20120303-464Xhttps://observatorio.fm.usp.br/handle/OPI/1373Introduction: Histoplasmosis is an infection caused by dimorphic fungus, Histoplasma capsulatum, and it has not been reported in juvenile systemic lupus erythematosus (JSLE) patients, particularly progressive disseminated histoplasmosis (PDH) subtype. Case report: We reported herein a 14-year old girl who was diagnosed with JSLE. Six months later, she had abdominal distension and received prednisone, hydroxychloroquine and azathioprine. Computer tomography evidenced hepatosplenomegaly and multiple mesenteric, mediastinal and retroperitoneal enlarged lymph nodes, forming large conglomerates at the mesentery, suggestive of lymphoproliferative disorder. After 10 days, she had acute surgical abdominal, and underwent a laparotomy and intestinal perforation and conglomerates of lymph nodes were observed. The jejunum biopsy showed perforated acute enteritis with hemorrhage and necrosis, and Grocott staining identified Histoplasma sp. and the culture showed a heavy growth of Histoplasma capsulatum. At that moment liposomal amphotericin B (1.0 mg/Kg/day) was introduced. Despite this treatment she died due to septic shock eight days later. Diffuse Histoplasma capsulatum was evidenced at autopsy. Conclusion: We reported a severe opportunistic infection in JSLE patient with adenopathy and multiple intestinal perforations. This study reinforces the importance of early diagnosis and antifungal therapy, especially in patients with these uncommon clinical manifestations.engrestrictedAccessHistoplasmosisJuvenile systemic lupus erythematosusChildrenOpportunistic infectiongastrointestinal histoplasmosisimmunodeficiencyperforationlymphomadiseasesarticleCopyright MEDFARMA-EDICOES MEDICAS, LDARheumatology