Donor Derived Systemic Lupus Erythematosus (SLE) after Allogeneic Transplantation

Donor-related autoimmune diseases after hematopoietic cell transplantation (HCT) are not well characterized. Most post-transplant immune disorders come in the guise of GVHD or reactions by the recipient to therapy (pneumonitis).  The extreme minority are due to donor-related adoptive immunity. It is interesting that donor-derived auto-immune issues are not more common after HCT, suggesting the presence of identifiable risk factors.

A 30 year old male underwent myeloablative matched related allogeneic peripheral blood stem cell transplant for Philadelphia chromosome positive Acute Lymphoid Leukemia (ALL). Importantly, the patient did not have any history of rheumatologic disorders, and specifically no history of Systemic Lupus Erythematosus (SLE).  On D+10 he developed chest pain; echocardiogram showed a moderate pericardial effusion that resolved with NSAIDs therapy. He later (D+73) developed myalgia and poly-synovitis requiring significant doses of opioids for relief. Infectious work-up was negative. Autoimmune evaluation demonstrated anti-nuclear antibodies (ANA) with elevated anti-double stranded DNA (477 U (range 0 – 29U)) as well as anti-smooth muscle antibody (SM Ab – 77 U (0-19.9)).  SLE diagnosis was made by Rheumatology and the patient was started on hydroxy-chloroquine, celecoxib and prednisone. The patient responded and is currently on maintenance therapy. In retrospect, donor history was notable for chronic pain and labs showed a mild eosinophilia with an absolute eosinophil count of 530/µl.  Significantly, the donor was subsequently tested and diagnosed with SLE as well.

Donor screening for auto-immune diseases could be considered in those with suspicious findings on evaluation.  This case demonstrates a real threat to recipient morbidity after HCT due to a donor related immune issue. Further study is needed to understand the incidence, severity and pattern of autoimmune diseases after HCT.