@article {Arnal75, author = {Christophe Arnal and Jean-charles Piette and Jean L{\'e}one and Bruno Taillan and Eric Hachulla and F Roudot-Thoraval and Thomas Papo and Annette Schaeffer and Philippe Bierling and Bertrand Godeau}, title = {Treatment of severe immune thrombocytopenia associated with systemic lupus erythematosus: 59 cases.}, volume = {29}, number = {1}, pages = {75--83}, year = {2002}, publisher = {The Journal of Rheumatology}, abstract = {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= 11) resulted in 50\% (7 CR, 2 PR) and 64\% (4 CR, 3 PR) longterm responses, respectively, allowing prednisone to be withdrawn or the dose tapered below 0.2 mg/kg body weight/day.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. CONCLUSION: Longterm remission was obtained in the majority of patients. The major treatments inducing remission were splenectomy and prednisone combined with danazol or hydroxychloroquine.}, issn = {0315-162X}, URL = {https://www.jrheum.org/content/29/1/75}, eprint = {https://www.jrheum.org/content/29/1/75.full.pdf}, journal = {The Journal of Rheumatology} }