Background Systemic lupus can affect multiple organs and it is associated with significant morbidity and mortality. Hematological manifestations in lupus are common and autoimmune hemolytic anemia (AHA) is a SLE classification criterion as well thrombocytopenia and leucopenia. It’s usually related with the more severe cases of SLE.
Objectives To analyze the frequency of hemolytic anemia in a Brazilian cohort of 1000 patients with SLE and the clinic manifestations that might be related to hemolytic anemia as their clinic evolution
Methods We reviewed retrospectively all cases of AHA from our lupus cohort of 1000 cases followed up between 1974 and 2011. Clinical data used in this study were extracted from the medical records of Hospital das Clínicas of State University of Campinas, Brazil. Hemolytic anemia was defined according to the American College of Rheumatology criteria for the classification of SLE and was not considered when secondary to drugs. We classified AHA in 75 patients according to severity in mild to moderate (women’s hemoglobin 7 g/dL - 11 g/dL and men’s hemoglobin 7 g/dL – 13 g/dL) or severe (≤7 g/dL); time of occurrence when at onset of SLE (first 6 months) or during the course of SLE (after 6 months) and frequency: a single episode or recurrent episodes. We compared patients with and without hemolytic anemia concerning to different clinical manifestations of the disease
Results Hemolytic anemia was present in 112 patients (11,2%) of all cases of SLE. The mean age of the patients was 27.13 years.There were 105 women and 7 men; 77 Caucasian and 35 non -Caucasian. Lupus patients with hemolytic anemia had more pleuritis (P=0,029), nephritis (P=0,012), leucopenia (P=0,001) and thrombocytopenia (P<0.001) and anti-dsDNA antibodies (P=0,008). Patients with Hemolytic anemia died more than patients without this manifestation (P=0,009). In 75 patients we observed AHA as a single episode in 64 (85%), recurrent episodes in 11 (15%) and at onset of SLE in 53 cases (71%) and in the evolution in 22 (29%). AHA was mild to moderate in 38,5% and severe in 61,5%
Conclusions Hemolytic anemia occurred in 11,2% of SLE, usually severe, at the onset of the disease and in a single episode. Pleuritis, nephritis, other cytopenias and Anti-DNA antibodies were strongly related to AHA. The higher death rate (18%) when compared to the patients without AHA (10.4%) was assigned to the multi systemic involvement, with high percentage of nephritis and other severe manifestations.
Disclosure of Interest G. Costallat Grant/Research support from: FAPSEP 2011/02907-8, S. Appenzeller Grant/Research support from: FAPESP 2008/02917-0; Conselho Nacional Pesquisa Desenvolvimento-Brasil CNPq (300447/2009-4), L. Costallat: None Declared