Objective Our objective was to review the rate of autoimmune cytopenia observed in lupus patients either at the inaugural diagnosis of lupus or during an already known lupus disease; try to correlate it with the precipitating factors and evaluate their outcome.
Design and Method This is a retrospective study of 9 years in an adult unit of internal medicine including 108 lupus adult patients. Anti-nuclear antibodies (ANA) were present in all patients, with antibodies to double stranded DNA (anti dsDNA)±anti nucleosome antibodies.
It concerns 94 women and 14 men. 35 cases diagnosed after the age of 50.
Results The autoimmune cytopenia was observed in 21 patients (16 women and 5 men).
The revelation modes varied between: exclusive neutropenia (6 cases), exclusive thrombocytopenia(6 patients)and pancytopenia (9 patients).
The association with antiphospholipid antibodies was noted in 6 patients, 2 cases had in addition insignificant titre of cold agglutinin, 1 case with a Sjögren syndrom, 2 cases associated with rheumatoid arthritis, 1 case with sclerosing cholangitis, 1 patient with cryofibrinogenemia and 2 cases with multiple autoimmunity disease. Only 1 woman had vitamin B12 deficiency.
The entry into the disease was revealed by a thrombotic microangiopathy (TMA) in one case and another woman by HELLP syndrom. A bone marrow aspirate was performed in a number of patients and eliminated a central cause.
Treatment with HYDROXYCHLOROQUINE and corticosteroids was given to all patients.
Immunosuppressive therapy, intravenous Immunoglobulin, thrombopoetin agonists and anti-CD20 were used in some cases. Cyclic antibiotics and Granocyte-colony stimulating factor were given to a patient with profound neutropenia and repeated severe infections. Plasma exchanges were performed in the TMA patient. The outcome was generally favourable for most patients and did not present a risk for survival. The death occurred in 2 patients due to other causes (mesenteric ischemia in one and bladder cancer in another).
Conclusions The autoimmune cytopenia in lupus is a source of diagnostic wandering, especially when it inaugurates the clinical presentation. The best knowledge of the mechanisms makes it possible to adapt the appropriate treatment. These cytopenias in our series did not constitute a pejorative element and were always of good prognosis due to the early management.
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