From the Guidelines
Yes, systemic lupus erythematosus (SLE) can cause hemolytic anemia, specifically autoimmune hemolytic anemia (AIHA), as stated in the 2019 update of the EULAR recommendations for the management of SLE 1. This occurs when the immune system produces autoantibodies that attack and destroy red blood cells, leading to anemia. The treatment of AIHA in SLE follows the same principles as other hematologic manifestations, with first-line treatment consisting of moderate to high doses of glucocorticoids (GC) in combination with immunosuppressive (IS) agents such as azathioprine, mycophenolate mofetil, or cyclosporine, and rituximab may be considered in cases of inadequate response or relapses 1. Some key points to consider in the management of AIHA in SLE include:
- The use of GC and IS agents to reduce the production of autoantibodies and decrease the destruction of red blood cells
- The consideration of rituximab in cases of inadequate response or relapses
- The potential need for blood transfusions in severe cases with significant anemia
- The importance of careful work-up to exclude other causes of anemia, especially drug-induced 1. While another study discusses the treatment of severe acute lupus-related hemolytic anaemia, the most recent and highest quality study, the 2019 EULAR recommendations, provides the most up-to-date guidance on the management of AIHA in SLE 1.
From the Research
Systemic Lupus Erythematosus (SLE) and Hemolytic Anemia
- Systemic Lupus Erythematosus (SLE) is a multisystem disorder characterized by the production of numerous autoantibodies, some of which have pathogenic consequences and result in considerable morbidity 2.
- SLE can cause hemolytic anemia, with warm autoimmune hemolytic anemia (AIHA) being a hematologic disorder that can occur in patients with SLE 3, 4, 5.
Incidence of AIHA in SLE Patients
- The incidence of AIHA in adult SLE patients is approximately 3%, while in pediatric SLE patients it is around 14% 3.
- AIHA can occur as the initial presentation of SLE, although this is extremely rare 4.
Clinical Associations and Outcomes
- Patients with SLE and AIHA are more likely to have elevated titers of IgG anticardiolipin antibodies and thrombosis 5.
- AIHA at the onset of SLE is independently associated with renal involvement, thrombocytopenia, and possibly thrombotic episodes during follow-up 5.
- The recurrence rate of AIHA in SLE patients is low among treated patients 5.
Treatment of AIHA in SLE Patients
- Treatment of AIHA in SLE patients can be challenging, and patients may require novel therapeutic modalities, including combinations targeting plasma cell, T- and B-cell proliferation 3.
- Rituximab, a chimeric monoclonal anti-CD20 antibody, has been used to treat steroid-resistant AIHA in SLE patients 2.
- Oral prednisolone has also been shown to improve hematological abnormalities in SLE patients with AIHA 6.