[CIS PIDD] [cis-pidd] SLE, C4 deficiency and HSCT

[Kobrynski, Lisa]

I was consulted on a young girl with autoimmune disease (Dermatomyositis, SLE with lupus nephritis) who has complete absence of C4.

We have not been able to get her autoantibody driven disease under control despite Methotrexate, systemic corticosteroids, mycophenolate, 2 courses of Retuximab and high dose IVIG.  She developed type I diabetes mellitus (acanthosis, elevated Hgb A1c) but then developed insulin resistance with marked hypoglycemia, despite stopping exogenous insulin.  Anti-insulin receptor antibodies are very elevated (on immunoblot).  She has now undergone 5 courses of plasma pheresis and still has significant hypoglycemia.  Repeat anti-insulin ab tests are pending.

The main consequence of her C4 deficiency seems to be the development of lupus, otherwise she has not had systemic infections.  Renal disease is consistent with lupus nephritis with IgG, IgA and IgM and C3 and C1q deposits and modestly elevated BUN and Cr.



Are there any other monoclonal ab therapies that might be useful?  (Belimumab?)

Additional immunosuppression?

What is the role of HSCT or mesenchymal SCT in these patients?  Would allogeneic transplant be better given her underlying C4 deficiency?



I appreciate any thoughts of comments you have on this patient.



Sincerely,



Lisa Kobrynski, MD, MPH
Associate Professor of Pediatrics
Marcus Professor of Immunology
Section, Allergy/Immunology
Emory University
Atlanta, GA

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