Danazol for systemic lupus erythematosus with refractory autoimmune thrombocytopenia or Evans' syndrome.

OBJECTIVE: To determine the efficacy of danazol for refractory autoimmune thrombocytopenia or Evans' syndrome complicating systemic lupus erythematosus (SLE). METHODS: We studied 16 consecutive patients with SLE and corticosteroid refractory autoimmune thrombocytopenia; 3 patients had coexisting autoimmune hemolysis (Evans' syndrome). Five patients had undergone splenectomy. Danazol was commenced at 200 mg/day, and increased stepwise (maximum 1200 mg/day) until benefit or toxicity was observed. After remission the danazol dose was gradually reduced to 200-400 mg/day. RESULTS: All 16 patients achieved a complete remission (platelet count >100 x 10(9)/l, hematocrit >39%) 2 months after starting danazol (range 6 weeks-8 months). Remission persisted during continued danazol therapy (mean followup 18.2 months, range 2-49 months). One patient with Evans' syndrome required discontinuation of danazol because of jaundice and biopsy proven minimal hepatic necrosis: hemolysis recurred after discontinuation of danazol. CONCLUSION: Danazol is effective for the treatment of autoimmune thrombocytopenia or Evans' syndrome complicating SLE irrespective of splenectomy status. Longer followup will be needed to determine whether the remission persists after withdrawal of danazol.

Renal biopsy in systemic lupus erythematosus: significance of glomerular thrombosis. Analysis of 108 cases.

We investigated the frequency and distribution of glomerular thrombosis (GT) in 108 renal biopsies of lupus patients and correlated this finding with the presence of anticardiolipin antibodies (ACLA). GT was present mainly in the diffuse proliferative form. The activity index was higher in those patients with GT (12.9 +/- 4.7 vs 5.4 +/- 4.1, P < 0.01). The more severe histologic features, necrosis and extracapillary proliferation were also related with GT. In 18 cases with repeated biopsy the best predictors for the subsequent development of glomerular sclerosis were fibrinoid necrosis (P < 0.01), glomerular infiltration (P < 0.01) and an activity index of 10 or more (P < 0.05). GT also showed to be an important prognostic factor for sclerosis, although no statistically significant. ACLA were investigated in 36 patients at the time of renal biopsy. There were nine positive cases and in three of them this finding was related to GT. We can conclude that GT is a relevant feature showing active lupus nephritis and that it is not related to the presence of ACLA.

Rheumatic manifestations in human immunodeficiency virus positive and negative individuals: a study of 2 populations with similar risk factors.

OBJECTIVE: Assess the impact of human immunodeficiency virus (HIV) infection on the onset of rheumatic manifestations in HIV+ patients, and to compare them with a control HIV- group with similar risk factors. METHODS: We prospectively studied 74 consecutive HIV+ patients, looking for clinical and laboratory findings of rheumatic manifestations and compared them with 72 control subjects with similar risk factors for HIV who tested negative for HIV. RESULTS: Rheumatic manifestations were more frequently observed in the HIV+ group than the HIV-group (p < 0.001): Arthralgias were found in 34 (45%), arthritis in 8 (10%), and Reiter's syndrome in 6 (8%). Laboratory findings revealed rheumatoid factor in 16 (21%) HIV+ vs 2 (2%) in HIV-, antinuclear antibodies in 13 (17%) HIV+ vs 0 in HIV-, IgG anticardiolipin antibodies in 70 (94%) HIV+ vs 7 (9%) in HIV- (p < 0.001). Hyperuricemia was found in 31 HIV+ patients (41%), and hypouricemia in 4 (5%), compared with none in the HIV- group (p < 0.0001). Neoplasia were identified in 13 HIV+ patients, in 7 associated with hyperuricemia and 3 with hypouricemia. Of interest, 2 patients had urate abnormalities before the diagnosis of neoplasia. CONCLUSIONS: Our study suggests that rheumatic manifestations are more prevalent in HIV+ patients. In advanced HIV infection, hypo and hyperuricemia may be considered markers of neoplasia.

PIP: This study was conducted to assess the impact of HIV infection on the onset of rheumatic manifestations in HIV+ patients and to compare them with a control HIV- group with similar risk factors. 74 consecutive HIV+ patients were therefore studied prospectively, with researchers looking for clinical and laboratory findings of rheumatic manifestations. These cases were compared against 72 control subjects with similar risk factors for HIV who tested negative for HIV. The results suggest that rheumatic manifestations are more prevalent in HIV+ patients. Further, in advanced HIV infection, hypo and hyperuricemia may be considered markers of neoplasia. Specifically, rheumatic manifestations were found more frequently in the HIV+ group than in the HIV- group: arthralgia were found in 45%, arthritis in 10%, and Reiter's syndrome in 8%. Rheumatoid factor was found in 21% of the HIV+ group and 2% in the HIV- group, antinuclear bodies in 17% of the HIV+ group versus none in the HIV- group, and IgG anticardiolipin antibodies in 94% of the HIV+ group and 9% of the HIV- group. Hyperuricemia was observed in 31 HIV+ patients and hypouricemia in 4, compared with none in the HIV- group. Neoplasia were observed in 13 HIV+ patients. Two patients had urate abnormalities before the diagnosis and neoplasia.