Treatment of severe immune thrombocytopenia associated with systemic lupus erythematosus: 59 cases.
OBJECTIVE
To evaluate the response to treatment in a large cohort of patients with systemic lupus erythematosus (SLE) associated with autoimmune thrombocytopenia.


METHODS
Response to treatment was assessed retrospectively in 59 patients with SLE, either definite (n = 44) or incomplete (n = 15), associated with frank autoimmune thrombocytopenia (defined as platelet count < 50 x 10(9)/l). Response to treatment was classified as complete (CR: platelet count > 150 x 10(9)/l), partial (PR: platelet count > 50 x 10/l), or failure (FR) in the other cases.


RESULTS
Oral prednisone alone was used in 50 of the 59 patients (mean initial dose 1 mg/kg body weight/day). A response was obtained in 80% of cases (CR in 28. PR in 12) but only 11 (22%) had a sustained response (CR, n = 7; PR, n = 4). In contrast, combined treatment with prednisone and either danazol (n = 18) or hydroxychloroquine (n = 11) resulted in 50% (7 CR, 2 PR) and 64% (4 CR, 3 PR) longterm responses, respectively, allowing prednisone to be withdrawn or the dose tapered below 0.2 mg/kg body weight/day. High dose methylprednisolone pulses (n = 10) and intravenous immunoglobulin (IVIG) (n = 31) resulted in positive responses in 60% (4 CR, 2 PR) and 65% (12 CR, 8 PR) of cases, respectively, but the response was transient in each case. Splenectomy (n = 17) resulted in 65% longterm responses (10 CR, 1 PR). Only 2 longterm partial responses were obtained with the 22 immunosuppressant-containing regimens administered to 14 patients. At the end of the study. a response was observed in 52 (88%) patients [CR: 36 (61%), PR: 16 (27%)], mainly as a result of splenectomy or combined treatment with prednisone and either danazol or hydroxychloroquine.


CONCLUSIONS
Longterm remission was obtained in the majority of patients. The major treatments inducing remission were splenectomy and prednisone combined with danazol or hydroxychloroquine.
Arnal
Christophe
C
Service de Médecine Interne, Hôpital Henri Mondor, Assistance Publique Hôpitaux de Paris, Creteil, France.
Piette
Jean-charles
JC
Léone
Jean
J
Taillan
Bruno
B
Hachulla
Eric
E
Roudot-Thoraval
F
F
Papo
Thomas
T
Schaeffer
Annette
A
Bierling
Philippe
P
Godeau
Bertrand
B
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D003613:Danazol / Q000627:therapeutic use; D004333:Drug Administration Routes; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D007074:Immunoglobulin G / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy*; D012008:Recurrence; D012189:Retrospective Studies; D013156:Splenectomy / Q000009:adverse effects / Q000706:statistics & numerical data*; D016896:Treatment Outcome
D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D006886:Hydroxychloroquine; D003613:Danazol; D011241:Prednisone; D008775:Methylprednisolone