Juvenile systemic lupus erythematosus (JSLE) and autoimmune hepatitis (AIH) are both autoimmune disorders that are rare in children and have a widespread clinical manifestation. A few case reports have shown a JSLE-AIH associated disorder. To our knowledge, this is the first study that simultaneously evaluated the prevalence of JSLE-AIH in a large JLSE and AIH population in groups of Hepatology and Rheumatology of a tertiary Paediatric University Hospital. In a 24-year period, 228 patients were diagnosed with JSLE (ACR criteria). In the same period, 252 patients were diagnosed with AIH according to the International Autoimmune Hepatitis Group. In this article, we present the demographic data, clinical features, laboratory exams and treatment of four children with both the diseases. The prevalence was 1.8% in JSLE population and was 1.6% in AIH population. The current median age was 15.5 years and three were females. In three of them, the diagnosis of AIH preceded JSLE. All of them had increased liver enzymes with a characteristic liver biopsy of AIH and responded to the combination of prednisone, azathioprine and antimalarial drugs. In conclusion, the presence of AIH-JSLE associated disorder was rarely observed. The liver biopsy could be necessary in patients with JLSE with a persistent increase of liver enzymes.

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Approximately 10% of cSLE had symptomatic PA at diagnosis, particularly endocrine autoimmune disorders and antiphospholipid syndrome. Lupus was characterized by a mild disease onset and MAS was infrequently evidenced. Further studies are necessary to determine if this subgroup of cSLE patients have a distinct genetic background with a less severe disease and a better long-term outcome.

Objectives To identify associations between mortality in cSLE patients and their characteristics: clinical and laboratory features, disease activity and damage scores, and treatment; to evaluate risk factors associated with mortality in cSLE; and to determine the most frequent causes of death in this group of patients. Methods We performed a multicenter retrospective cohort using data from 1,528 cSLE patients followed in 27 pediatric rheumatology tertiary centers in Brazil. Patients’ medical records were reviewed according to a standardized protocol, in which information regarding demographic and clinical features, disease activity and damage scores, and treatment were collected and compared between deceased cSLE patients and survivors. Univariate and multivariate analyses by Cox regression model were used to calculate risk factors for mortality, whereas survival rates were analyzed by Kaplan–Meier plots. Results A total of 63/1,528 (4.1%) patients deceased, 53/63 were female (84.1%), median age at death was 11.9 (9.4–13.1) years and median time interval between cSLE diagnosis and death was 3.2 (0.5–5.3) years. Sepsis was the main cause of death in 27/63 (42.8%) patients, followed by opportunistic infections in 7/63 (11.1%), and alveolar hemorrhage in 6/63 (9.5%) patients. The regression models resulted in neuropsychiatric lupus (NP-SLE) (HR = 2.56, 95% CI = 1.48–4.42) and chronic kidney disease (CKD) (HR = 4.33, 95% CI = 2.33–4.72), as risk factors significantly associated with mortality. Overall patient survival after cSLE diagnosis at 5, 10, and 15 years were 97%, 95.4%, and 93.8%, respectively. Conclusions This study confirmed that the recent mortality rate in cSLE in Brazil is low, but still of concern. NP-SLE and CKD were the main risk factors for mortality, indicating that the magnitude of these manifestations was significantly high.

Background Infections are an important cause of morbidity and mortality in juvenile systemic lupus erythematosus (JSLE). Invasive aspergillosis (IA) is a fungal infection caused by Aspergillus spp., usually affects the pulmonary tract, but can involve any organ or system. Association between IA and SLE was seldom described in adults and to our knowledge only two cases were reported in JSLE patients, including one from our Pediatric Rheumatology Group (1). However, the prevalence of IA in a large population of JSLE patients from a tertiary Pediatric Hospital was not reported. Objectives To evaluate the prevalence and report the demographic data, clinical findings, treatment regimens and outcome of JSLE patients at IA diagnosis. Methods From January 1983 to June 2011, 5,604 patients were followed at the Pediatric Rheumatology Unit from our University Hospital and 283 (5%) of them met the ACR classification criteria for SLE. IA was classified in “proven” and “probable” as previously defined. Proven IA demands isolation of Aspergillus spp. in tissue and probable IA requires isolation of Aspergillus spp. in direct test (culture, cytology or microscopy) or indirect test (detection of antigen or cell-wall component) (2). Results Six (2.1%) of our JSLE patients had IA, and were the only of the total population (n=5,604; 0.1%) followed at our service with this invasive fungal disease. One of them was previously reported (1) and five will be reported herein. Proven IA was observed in four cases and probable IA in one case. Four of them were of the female gender. The median age at JSLE diagnosis was 12 years (8-16) and the median interval between diagnosis of JSLE and IA was 6 months (1-38). All had pulmonary involvement and three of them had systemic involvement. The median SLEDAI-2K was 19 (7-22). Diagnosis of IA was performed by isolation of Aspergillus spp., two in bronchoalveolar lavage culture and by way of autopsy in the others. All of them were treated with corticosteroids and immunosuppressive drugs at IA diagnosis (azathioprine and intravenous cyclophosphamide). They all required pediatric intensive care unit with mechanical ventilation and antifungal therapy (fluconazole, amphotericin B and/or voriconazole), nonetheless none of them survived. Conclusions IA is a rare and severe opportunistic infection that may mimic JSLE manifestations in patients with active disease receiving immunosuppressive agents. This study reinforces the importance of early diagnosis and antifungal therapy, especially in critically ill patients. References Canova EG, Rosa DC, Vallada MG, et al. Invasive aspergillosis in juvenile systemic lupus erythematosus. A clinico-pathologic case. Clin Exp Rheumatol 2002; 20: 736. De Pauw B, Walsh TJ, Donnelly JP, et al. Revised definitions of invasive fungal disease from the European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group (EORTC/MSG) Consensus Group. Clin Infe...