The occurrence of systemic lupus erythematosus (SLE) is reported in 2 pairs of male siblings. Since the occurrence of idopathic SLE is uncommon in the male, these cases provide additional support to the hypothesis that genetic factors play an important role in pathogenesis. Of additional interest in these patients was the varied clinical manifestations of disease between brothers, suggesting that additional factors govern the clinical expression of this disorder.The increasing evidence that systemic lupus erythematosis (SLE) is, at least in part, genetically determined, is supported by familial occurrence of the disease. Dubois gathered 66 cases from among 30 families in 1966 (1). Subsequently, 8 cases in 4 additional families have been reported (2-S), making a total of 74 cases in 34 families.Because of the potential error in studies of this kind, as shown by O'Brien (6), in denoting a true increased familial incidence, reports of concordance for SLE in twins are of special importance (2-3,7-11). In a similar way the occurrence of SLE among male siblings provides additional evidence for the importance of genetic factors since.the sex incidence in SLE is predominantly female, accounting for approximately 90% of cases (12). In the present report 2 sets of male siblings with SLE are described.
CASE REPORTS Family ACase 1. Patient RA was admitted to the Yale-New Haven Hospital in 1959, at age 14, because of a 2-month history of arthritis. Evaluation revealed fusiform swelling of wrists and interphalangeal joints, generalized lymphadenopathy, enlargement of liver and spleen and dependent edema. Laboratory studies demonstrated a positive LE cell preparation and antinuclear antibody test, a hematocrit of 23%, white blood cell count of 5350/cu mm, serum albumin of 1.9 g% and serum globulin of 6.6 g%. Renal involvement was indicated by elevated levels of nonprotein nitrogen, 43 to 67 mg% (normal: <45), 3+ proteinuria and microscopic hematuria. Subsequent to treatment with cortisone and chloroquine his symptoms resolved although photosensitivity and mild hypertension were noted.Five years later, 1965, a renal biopsy was performed because of persistent proteinuria and demonstrated a mild diffuse proliferative glomerulonephritis. After the institution of prednisone, 40 mg/day, he experienced a generalized seizure associated with elevated blood pressure, 180/120 mmHg. The dose of prednisone was reduced and discontinued in 1968 when aseptic necrosis of the right hip was discovered. Hydroxychloroquine, begun in 1967, 400 mg/day, has been continued until the present time.During the past 7 years he has remained free of symptoms and has been employed full time. Laboratory studies