The Journal of Rheumatology 2002; 29:9 2000 From the Pediatric Rheumatology Unit, Department of Pediatrics, Children’s Institute, and the Division of Urology Clinic, University of São Paulo, São Paulo, Brazil. C.A.A. Silva, MD, PhD, Head, Pediatric Rheumatology Unit, Department of Pediatrics; J. Hallak, MD, PhD, Attending Physician, Division of Urology Clinic; F.F. Pasqualotto, MD, Postgraduate student, Division of Urology Clinic; M.F. Barba, MD, PhD, Attending Physician, Department of Pediatric Radiology; M.I. Saito, MD, PhD, Head, Adolescent Unit; M.H.B. Kiss, MD, PhD, Associate Professor of Pediatrics. Address reprint requests to Dr. C.A.A. Silva, Rua Raul Pompéia, 303/43-Pompéia, São Paulo SP, Brazil CEP 05025-010. E-mail: clovisaas@icr.hcnet.usp.br Submitted October 17, 2001; revision accepted March 7, 2002. Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of unknown etiology and is more preva- lent in women than in men (F:M = 5:1) 1 . The annual incidence of juvenile onset SLE in childhood is roughly 0.4 per 100,000. The prognosis for children and adolescents with SLE con- tinues to improve as better access to medical care and diag- nostic testing have provided better detection of children with SLE 2 . With antiinflammatory therapy as well as sophisticated pediatric care, 10-year survival rates are now found in over 80% of patients with SLE 3,4 . However, clinical attention has begun to shift away from the problems of cure and toward refinements in quality of life among the survivors. Despite the number of adolescents and young adults with SLE, few stud- ies have reported the gonadal function of these patients. Oligospermia or azoospermia in patients with SLE may have several causes including adolescence, the disease itself, drugs used to treat the illness, disease of the genital system 5,6 , hypothalamic-pituitary-axis dysfunction 7,8 , autoimmunity, activity of the disease 9 , and especially the drugs used as immunosuppressors, particularly cyclophosphamide 10-15 and chlorambucil 12-15 . We studied gonadal function in 4 male adolescents and young men with SLE. MATERIALS AND METHODS Patients. Four male patients who fulfilled 4 or more 1982 American College of Rheumatology (ACR) classification criteria for SLE 16 , followed at the Pediatric Rheumatology Unit of the Children’s Institute of our university hos- pital, were studied. Informed consent was obtained from all participants. Their actual ages ranged from 16 years and 9 months to 22 years and 10 months. Clinical evaluation. SLE disease activity and cumulative damage at the time of study entry were measured in all patients, using the SLE Disease Activity Index (SLEDAI) 17 and the Systemic Lupus International Collaborating Clinics/ACR (SLICC/ACR) Damage Index 18 . SLE activity was classified into 3 patterns: relapsing-remitting (RR), chronic active (CA), and long quiescent Gonadal Function in Male Adolescents and Young Males with Juvenile Onset Systemic Lupus Erythematosus CLOVIS A.A. SILVA, JORGE HALLAK, FABIO F. PASQUALOTTO, MÁRIO F. BARBA, MARIA I. SAITO, and MARIA H.B. KISS ABSTRACT. Objective. To evaluate gonadal function in male adolescents and young men with juvenile onset sys- temic lupus erythematosus (SLE). Methods. Four young men with SLE underwent clinical and laboratory evaluation, testicular ultrasound, follicle stimulating hormone, luteinizing hormone, prolactin, testosterone, and anti-sperm antibody determination. The semen analyses were performed according to the WHO guidelines and Kruger strict criteria. All patients were asked to provide 3 semen samples over a period of 2 months. A new sample was collected 6 months later. Results. The median disease duration was 6.6 years. The median age at initial ejaculation was 13.5 years. All 4 patients had severe disease with renal involvement (WHO class IV or V). The SLICC/ACR damage index at the time of study entry ranged between 0 and 3. The patients’ Tanner stage was P5G5; all reported normal erection and libido. Gonadal evaluation by thorough examination of the genitalia and ultrasound was normal. Anti-sperm antibodies were negative in all patients. Only one patient showed high FSH and LH levels. The initial and final semen evaluations of the 4 patients were abnor- mal (azoospermia, oligoastenoteratospermia, or teratospermia). One patient was receiving azathioprine and 2 were receiving cyclophosphamide at the time of study entry. Conclusion. Although these patients had normal sexual activity and normal external genitalia, their fer- tility was decreased based on the sperm abnormalities. Serial semen analyses in larger study populations will be necessary to clarify the degree and duration of sperm abnormalities in male patients with SLE in general. (J Rheumatol 2002;29:2000–5) Key Indexing Terms: LUPUS MALE GONADAL SEMEN HORMONE ADOLESCENT Personal non-commercial use only. The Journal of Rheumatology Copyright © 2002. All rights reserved.