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Treatment of isolated severe immune hemolytic anaemia associated with systemic lupus erythematosus: 26 cases

Department of Internal Medicine, Hôtel Dieu, Lyon, France

M Ruivard

Department of Internal Medicine and Hematology, CHU de Clermont-Ferrand, Clermont-Ferrand, France

M Koenig

Department of Internal Medicine, Hôpital Nord, Saint-Etienne, France

A Woods

Department of Hematology, University Hospital, Edmonton, Canada

N Magy

Department of Internal Medicine, Hopital Jean-Minjoz, Besancon, France

J Ninet

Department of Internal Medicine, Hôpital Edouard Herriot, Lyon, France

H Rousset

Department of Internal Medicine, Centre Hospitalier Lyon-Sud, Pierre-Bénite, France

G Salles

Department of Hematology, Centre Hospitalier Lyon-Sud, Pierre-Bénite, France

C Broussolle

Department of Internal Medicine, Hôtel Dieu, Lyon, France

P Sève

Department of Internal Medicine, Hôtel Dieu, Lyon, France, pascal.seve{at}chu-lyon.fr

The aim of this study was to evaluate the response to treatment and the long-term outcome in a cohort of patients in whom severe autoimmune hemolytic anaemia (AHA) was the leading manifestation of systemic lupus erythematosus (SLE). Twenty-six women with severe isolated AHA were included. Corticosteroids were used as the initial treatment for all patients in our study. An initial response was obtained in all but one patient (96%). The overall recurrence rate was three per 100 person-years, with an expected recurrence-free proportion of 73% with a 180 months median follow-up. Seven patients (27%) experienced a relapse of AHA. We found a higher proportion of pleuritis in relapsing patients. Only three patients experienced multiple relapses despite splenectomy and several immunosuppressants. Steroid-sparing effect of hydroxychloroquine and azathioprine could not be assessed because most of the patients received these treatments for other reasons than AHA. Intravenous immunoglobulins induced transient response in three cases. Splenectomy was efficient to definitively control AHA in one patient but two patients quickly experienced relapses while one patient did not benefit. Five patients received immunosuppressants that induced only transient responses. Rituximab was long-term efficient in one case. In conclusion, severe AHA is a serious complication of SLE that warrants appropriate management. On the basis of our experience, the ideal treatment of isolated AHA should be oral corticosteroids in first-line treatment. Our study does not support an important role for splenectomy. Patients refractory to conventional therapy should be treated either with few toxic immunosuppressive drugs, danazol or rituximab.

Key Words: autoimmune hemolytic anaemia • systemic lupus erythematosus

Lupus, Vol. 15, No. 4, 223-231 (2006)
DOI: 10.1191/0961203306lu2292oa


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