What is the management of spleen involvement in systemic lupus erythematosus (SLE)?

Management of Spleen Involvement in Systemic Lupus Erythematosus (SLE)

Spleen involvement in SLE should be managed according to the specific manifestation, with splenectomy reserved only as a last resort option for cases of severe, refractory thrombocytopenia that fail to respond to medical therapy. 1

Hematological Manifestations Related to Splenic Involvement

• Thrombocytopenia and autoimmune hemolytic anemia (AIHA) are the most common hematological manifestations in SLE that may involve the spleen 1
• Splenic calcifications may occur in SLE patients, presenting a unique pattern that can help differentiate from other conditions 2

Treatment Algorithm for Thrombocytopenia in SLE

First-line therapy:

• Moderate to high doses of glucocorticoids (GC) in combination with immunosuppressive (IS) agents 1
  • Initial therapy with pulses of intravenous methylprednisolone (1-3 days) is recommended 1
  • Follow with oral prednisone with quick tapering to maintenance doses ≤5 mg/day 3
• Immunosuppressive options to combine with GC:
  • Azathioprine (AZA) 1
  • Mycophenolate mofetil (MMF) 1
  • Cyclosporine (has the least potential for myelotoxicity) 1

Acute phase management:

• Intravenous immunoglobulin (IVIG) may be considered in:
  • Cases with inadequate response to high-dose GC 1
  • Situations where GC-related infectious complications need to be avoided 1

For refractory cases or relapses:

• Rituximab (RTX) should be considered for patients with:
  • Failure to reach platelet count >50,000/mm³ despite GC therapy 1
  • Relapses during GC tapering 1
• Cyclophosphamide (CYC) may also be considered in these cases 1

Last resort options:

• Thrombopoietin agonists 1
• Splenectomy - reserved only for cases that fail all medical therapies 1

Management of Autoimmune Hemolytic Anemia (AIHA)

• AIHA is less common than thrombocytopenia in SLE but follows the same treatment principles 1:
  • Glucocorticoids
  • Immunosuppressive drugs
  • Rituximab for refractory cases

Monitoring and Prevention

• Regular assessment of disease activity using validated indices 4
• All SLE patients should receive hydroxychloroquine (HCQ) as the backbone of treatment unless contraindicated 4, 5
• Dose of HCQ should not exceed 5 mg/kg real body weight to minimize risk of retinal toxicity 1
• Monitor for infections, as SLE patients have increased risk due to both disease and immunosuppressive therapy 4

Special Considerations

• Treatment of thrombocytopenia in SLE is typically lengthy and often characterized by relapses during GC tapering 1
• Autoimmune leukopenia is common in SLE but rarely needs treatment; careful workup is recommended to exclude other causes (especially drug-induced) 1
• The goal of treatment should be remission or low disease activity to prevent organ damage accrual 1
• Consider antiphospholipid antibody status when managing SLE patients with hematological manifestations, as this may influence treatment approach 1

Pitfalls and Caveats

• Avoid prolonged use of high-dose glucocorticoids due to risk of irreversible organ damage 1
• Risks of glucocorticoid therapy increase substantially with doses >7.5 mg/day prednisone equivalent 1
• Carefully assess adherence to drug treatment, especially hydroxychloroquine, as non-adherence is associated with higher flare rates 1
• Splenectomy should not be considered early in the treatment algorithm due to increased risk of infections and potential for post-splenectomy thrombosis 1