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.
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 Infect Dis 2008; 46:1813-1821.
Disclosure of Interest None Declared