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Severe clinical course of systemic lupus erythematosus in the first year of life

Rheumatology Unit, Department of Pediatrics, University of Padua, Italy zulian{at}pediatria.unipd.it

FR Pluchinotta

Pediatric Rheumatology Fellow, Department of Pediatrics, University of Padua, Italy

G Martini

Rheumatology Unit, Department of Pediatrics, University of Padua, Italy

L Da Dalt

Emergency Medicine Unit, Department of Pediatrics, University of Padua, Italy

G Zacchello

Nephrology Unit, Department of Pediatrics, University of Padua, Italy

Systemic lupus erythematosus (SLE) very rarely occurs before the age of 5. Herein we describe the clinical features of infantile SLE (iSLE) with onset during the first year of life. The clinical and laboratory characteristics of iSLE patients followed at the Department of Pediatrics of Padua were analyzed. They were combined with those collected from the literature by performing a systematic literature search on PubMed using the following keywords: SLE, infant, laboratory, therapy, and outcome. A total of 13 patients with iSLE, 2 from our Institution and 11 from the literature, are included in this review. Seven (53.8%) were females and 6 were males (46.2%). The age at disease onset ranged from 6 weeks to 11 months. In comparison with juvenile systemic lupus erythematosus (jSLE), iSLE showed a higher prevalence of positive family history for autoimmune diseases, systemic symptoms at presentation, internal organs involvement, and shorter time between symptoms onset and diagnosis. Anemia and thrombocytopenia were present in the majority of the patients at diagnosis, whereas leukopenia was rarely observed. The overall prognosis in iSLE was very poor: 5/13 infants died between 2 and 31 months after the onset, and 5/13 had severe disease course with residual organ damage. SLE can start as early as during the first year of life and is more severe than in the later age groups.

Key Words: child • infant • outcome • systemic lupus erythematosus

Lupus, Vol. 17, No. 9, 780-786 (2008)
DOI: 10.1177/0961203308090992


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