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.


CONCLUSIONS
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.
Cervera
H
H
Rheumatic Diseases Unit, Hospital de Especialidades, Mexico City, Mexico.
Jara
L J
LJ
Pizarro
S
S
Enkerlin
H L
HL
Fernandez
M
M
Medina
F
F
Fraga
A
A
Miranda
J M
JM
D000328:Adult; D000743:Anemia, Hemolytic / Q000150:complications*; D001327:Autoimmune Diseases / Q000150:complications*; D003613:Danazol / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy* / Q000601:surgery; D008875:Middle Aged; D011446:Prospective Studies; D012074:Remission Induction; D013156:Splenectomy; D013577:Syndrome; D013921:Thrombocytopenia / Q000150:complications*
D003613:Danazol