THU0517 Invasive Fungal Infections Survey in 852 Childhood-Onset Systemic Lupus Erythematosus: A Multicenter Cohort

Abstract

BackgroundThe majority of infections in childhood-onset systemic lupus erythematosus (cSLE) patients are caused by virus and bacteria, and less frequently by opportunistic agents, such as fungi.1 However, studies evaluating solely invasive fungal infections (IFI) in cSLE patients are restricted to case reports or case series1 without any systematic evaluation of the possible associated risk factors and outcomes in pediatric lupus population.ObjectivesTo study the prevalence, risk factors and mortality of IFI in cSLE patients.MethodsA retrospective multicenter cohort study was performed in 852 cSLE patients from 10 Pediatric Rheumatology services of São Paulo state, Brazil. An investigator meeting was held and all participants received database training. IFI were diagnosed according to EORTC/MSG Consensus Group (proven, probable and possible).2 Demographic data, clinical, laboratorial, SLEDAI-2K, SLICC/ACR-DI, treatment and outcome were also evaluated.ResultsIFI were recorded in 33/852 (3.9%) cSLE patients. Proven IFI was evidenced in 22 cSLE patients, probable IFI in 5 and possible IFI in 6. The most frequent types of IFI were candidiasis (n=20) and aspergillosis (n=9). The median of disease duration was lower (1.0 vs. 4.7 years, p<0.0001), with a higher current SLEDAI-2K [19.5 (0-44) vs. 2 (0-45), p<0.0001] and current prednisone dose [50 (10-60) vs. 10 (2-90) mg/day, p<0.0001] in patients with IFI compared to those without IFI. The frequency of death was higher in the former group (51% vs. 6%, p<0.0001). Logistic regression analysis revealed that SLEDAI-2K (OR=1.108; 95%CI=1.057-1.163; p<0.0001), current prednisone dose (OR=1.046; 95%CI=1.021-1.071; p<0.0001) and disease duration (OR=0.984; 95%CI=0.969-0.998; p=0.030) were independent risk factors for IFI (R2 Nagelkerke 0.425).ConclusionsThis was the first study that characterized IFI in a large cSLE population. We identified that disease activity and glucocorticoid use were the main risk factors for these life-threatening infections, mainly in early disease course and with a high rate of fatal outcome.ReferencesSilva MF, Ribeiro AS, Fiorot FJ, et al. Invasive aspergillosis: a severe infection in juvenile systemic lupus erythematosus patients. Lupus 2012;21:1011-6.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 Infect Dis 2008;46:1813-21.Disclosure of InterestNone declared

Childhood-onset systemic lupus erythematosus: early disease manifestations that the paediatrician must know

“…Lymphopenia (<1500 cells/mm 3 ) and leukopenia (white blood cell count <4000/mm 3 ) at cSLE diagnosis occur in 43% and in 27%, respectively, and may be associated with disease activity, drug therapy, and current infections [1,3,8,10]. Autoimmune hemolytic anemia and immune thrombocytopenia (<100,000/mm 3 ) may be the initial presentation and occur at cSLE diagnosis in 21% and 15%, respectively [10].…”

“…The most important risk factors associated with infections at cSLE onset are related to this systemic disease (leukopenia, neutropenia, lymphopenia, disease flare, and hypocomplementemia), concomitant primary immunodeficiency syndrome, and immunosuppressive treatment [1]. The most frequent infection sites in cSLE patients are skin and soft tissue, pneumonia, and urinary tract infections [1,8]. A recent study reported invasive fungal infections in 33 of 852 (3.9%) cSLE patients, particularly candidiasis and invasive aspergillosis.…”

“…A recent study reported invasive fungal infections in 33 of 852 (3.9%) cSLE patients, particularly candidiasis and invasive aspergillosis. Invasive fungal disease was observed at disease onset with high disease activity score (median SLE Disease Activity Index 2000 was 28) in 6 of 33 cSLE patients [8].…”

“…Serum levels of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) are sensitive markers of chronic inflammation and acute infections in cSLE patients [8,10]. Studies have also found inconsistent correlations between CRP levels and SLE disease activity [10,21].…”

“…Studies have also found inconsistent correlations between CRP levels and SLE disease activity [10,21]. In addition, ESR and CRP levels were higher in patients with severe fungal infections compared to those without these complications [8].…”