Causes of anemia in SLE patients include chronic inflammation, hemolysis, blood loss, renal insufficiency, myelodysplasia, hypersplenism, or marrow aplasia.

A white blood cell count lower than 4500/µL has been reported in 50% of patients. This could be due to lymphopenia, neutropenia, or both and may indicate active SLE but can also be caused by infections, medications, or hypersplenism.

Up to 50% of SLE patients present with mild thrombocytopenia associated with immune platelet destruction or antiphospholipid antibody syndrome. Severe thrombocytopenia should raise the suspicion of thrombotic thrombocytopenic purpura. Other common causes of low platelet count are hypersplenism, drugs, infections, and bone marrow aplasia.

MANAGEMENT AND TREATMENT

The treatment of SLE is tailored to the severity of individual organ involvement and to the degree of disease activity. All patients should be instructed to avoid exposure to ultraviolet light and wear sunscreen at all times. Regular visits to a rheumatologist, even during periods of inactivity, are indicated. Traditional cardiovascular risks should be identified in every patient and measures to correct them should be implemented.

Antimalarial agents are indicated in all SLE patients, not just for the treatment of cutaneous SLE, serositis, and arthritis but also for their immunomodulating and antithrombotic effects. Low-dose aspirin should also be part of the “baseline” treatment of every patient with SLE.

Corticosteroids are the mainstay of treatment for most of the lupus flares. Dosing and route of administration depends on the type and severity of organ involvement.

Corticosteroid toxicity is a major problem in SLE, and the clinician should try to taper the dose as soon as clinically possible. The doses of corticosteroids vary widely from a few milligrams per day to 1 g of Solu-Medrol for 3 to 5 days intravenously in cases of life-threatening or organ-threatening lupus. Protection from osteoporosis is very important, and so is protection against Pneumocystis jiroveci pneumonia in patients prescribed moderate and high doses of corticosteroids for prolonged periods of time.

Additional immunosuppressants are frequently used for their corticosteroid-sparing effects and for more severe disease. Drugs in this group include azathioprine, methotrexate, cyclophosphamide, mycophenolate mofetil, and cyclosporine. Potential toxicities make close monitoring mandatory. Contraception is mandatory owing to the teratogenic risk of these medications. Pregnant women with severe active lupus can be treated with high doses of glucocorticoids and azathioprine, and the fetus should be delivered at the earliest time that is deemed safe. Mycophenolate mofetil has emerged as an alternative to cyclophosphamide in some patients with lupus nephritis because studies have shown comparable efficacy and fewer side effects.

Belimumab is a fully human monoclonal antibody that specifically recognizes and inhibits the biologic activity of B-lymphocyte stimulator (BLyS), a factor important for the survival of B lymphocytes. Two studies (BLISS-76 and BLISS-52) have evaluated the efficacy and safety of belimumab plus standard of care in patients with active SLE. Belimumab resulted in a significant improvement in disease activity measures by week 52 and helped reduce the prednisone dose while increasing the time to the first disease flare and the risk of severe disease flare compared with placebo.

Several new agents are under investigation. Some of these drugs target T lymphocyte co-stimulatory activation (cytotoxic T-lymphocyte–associated protein-4 [CTLA-4]), production of reactive oxygen species (N-acetylcysteine), aberrant activation of mTOR (rapamycin), spleen tyrosine kinase (Syk inhibitor), type 1 interferons (anti–IFN-α monoclonal antibody), and IL-6 (tocilizumab).