What rheumatological diseases are reported after transplant as part of treatment for Sickle Cell Disease (SCD)?

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Rheumatological Diseases Reported After Transplantation in Sickle Cell Disease

Vaso-occlusive pain episodes are the most commonly reported rheumatological complication after transplantation in patients with sickle cell disease, occurring in up to 64% of patients, particularly in association with corticosteroid use for immunosuppression. 1

Post-Transplant Rheumatological Complications

Common Complications

  • Vaso-occlusive pain episodes:

    • Reported in 64% of SCD patients following renal transplantation 1
    • Strongly associated with corticosteroid exposure used in post-transplant immunosuppression regimens
    • May lead to decline in renal function when occurring after renal transplantation

  • Avascular necrosis (AVN):

    • Common complication in SCD patients, particularly affecting hip and shoulder joints 2
    • Can be exacerbated post-transplant due to immunosuppressive therapy, especially corticosteroids
    • In patients with coexisting SCD and rheumatoid arthritis, 4 out of 7 patients developed AVN 2

Immunological Complications

  • Graft-versus-host disease (GVHD):

    • Significant risk following hematopoietic stem cell transplantation (HSCT)
    • More common with alternative donors than matched sibling donors 1
    • Can present with rheumatological manifestations including arthritis, myositis, and scleroderma-like features
    • Severe chronic GVHD has been reported in heavily-transfused end-stage adult SCD patients 1

  • Graft rejection:

    • Reported in 23% of SCD patients following renal transplantation 1
    • Can lead to inflammatory responses with joint manifestations

Management Considerations

Immunosuppression Regimen Selection

  • Corticosteroid use:

    • Guidelines specifically advise judicious use of corticosteroids in post-transplant immunosuppression regimens due to the established relationship between steroid exposure and vaso-occlusive pain in SCD patients 1
    • Consider steroid-sparing or minimizing regimens when possible

  • Alternative immunosuppression:

    • For HSCT, antibodies should be routinely used for GVHD prevention in non-sibling transplants 1
    • In renal transplantation, carefully balance immunosuppression needs against risk of triggering SCD complications

Viral Infection Risk and Management

  • Virus-specific T-cell therapy:
    • Effective for prevention and treatment of viral infections post-transplant in SCD patients
    • 80% of patients with active viremia achieved remission of at least one target virus 3
    • 85.7% of patients receiving prophylactic VSTs remained virus-free 3
    • Low rate of severe GVHD development (15.4%) with VST therapy 3

Special Considerations for Different Transplant Types

Renal Transplantation

  • Despite risks, renal transplantation is recommended over dialysis for SCD patients with end-stage renal disease 1
  • One-year survival rate after renal transplantation is approximately 88% 1
  • Careful monitoring for pain episodes is essential, as they occurred in 64% of patients post-renal transplant 1

Hematopoietic Stem Cell Transplantation

  • Preferred stem cell source is bone marrow or umbilical cord blood from matched sibling donors 1
  • HSCT should be considered early in the disease course, preferably at pre-school age when possible 1
  • For patients without matched sibling donors, alternative donor transplantation should be considered within clinical trials 1

Coexisting Rheumatoid Arthritis and SCD

  • Rheumatoid arthritis is rarely reported among SCD patients 2
  • In a case series of 7 patients with both conditions:
    • Average age at RA diagnosis was 33.3±12.6 years
    • Most were women (5/7) and RF/anti-CCP positive (6/7)
    • Treatment with standard antirheumatic drugs (including methotrexate and biologics) did not increase risk of SCD complications 2

Clinical Pitfalls and Caveats

  1. Distinguishing pain sources: Differentiating between vaso-occlusive crisis, GVHD-related pain, and other rheumatological conditions can be challenging post-transplant.

  2. Steroid-induced complications: Careful monitoring is required when using corticosteroids, as they can trigger vaso-occlusive crises in SCD patients 1.

  3. Infection risk: Immunosuppression increases risk of infections that can mimic rheumatological conditions; virus-specific T-cell therapy can be considered to reduce this risk 3.

  4. Monitoring for AVN: Regular assessment for early signs of avascular necrosis is essential, particularly in patients receiving corticosteroids.

  5. Balancing disease control and transplant success: The goal of immunosuppression must be balanced against the risk of triggering SCD complications.

References

1
Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

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This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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