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732 Clr DEFICIENCY: PRESENTATION AS A MILD LUPUS LIKE SYNDROME

The congenital absence of early complement components may be associated with autoimmunity. We report the 4th patient deficient in Clr, a 14-Yr-old Puerto Rican female who presented with vague migrating arthralgia. She had a history of possible rheumatic fever and post varicella encephalitis but no unusual susceptibility to infection and no skin rash. She was normotensive and physical examination was normal. She had mild proteinuria (300 mg/24 Hr) and hematuria but no cylinduria. Creatinine clearance was normal. Renal biopsy showed mild mesangial proliferation and focal IgG and C3 basement membrane deposition. Immunoglobulins were elevated. Rheumatoid factor was present (1:640) and antibodies to Sm antigen were present (1:105) although antibodies to double stranded DNA were absent. High titers of immune complexes were detected by Raji cell assay. Total hemolytic complement was < 1%. Hemolytic C1 was < .1%. Other components were normal. The defect in C1 was found to be C1r by normal levels of C1q protein, 2/3 normal C1s protein, inability to correct the defect in hemolytic C1 with two C1r deficient sera, and no C1r protein. Thus, we confirm that absence of C1r may be associated with autoimmunity but the minimal clinical manifestations suggest a wider spectrum than previously appreciated.