Major infections in a cohort of 120 patients with juvenile-onset systemic lupus erythematosus

•The most common major infections in jSLE are skin and soft tissue infections, pneumonias and urinary tract infections with fever.•Major infections are responsible for considerable morbidity in jSLE.•jSLE activity and its treatment contribute to the risk of major infection.•Judicious use of immunosuppressive drugs is essential to reduce major infections.

In order to describe the incidence and characteristics of major infections in juvenile-onset systemic lupus erythematosus (jSLE), we studied a cohort of 120 patients (51% Hispanic and 28% African American, 49% with renal involvement and 12% with neuropsychiatric manifestations). There were 101 major infections affecting 44 patients (37%) for an incidence of 169/1000 patient-years of follow-up. Active disease at jSLE diagnosis, renal involvement, neuropsychiatric manifestations, higher cumulative dose of prednisone, and treatment with cyclophosphamide or mycophenolate mofetil were all associated with major infection (p < 0.05). By logistic regression, the combined effect of treatment with cyclophosphamide and cumulative dose of prednisone was associated with major infection (p = 0.04). Two patients died, one due to cytomegalovirus infection. Major infection was associated with damage (p = 0.004). In conclusion, in a large cohort of jSLE patients, major infections were common, were associated with active disease and its treatment, and resulted in noteworthy morbidity.