[CIS PIDD] (no subject)

[Sorensen, Ricardo]

Hi everybody, 

I would like to ask you for advise on a patient with  a severe
systemic-onset Juvenile rheumatoid arthritis (JRA).  JRA is not defined
as an imunodeficiency jet, but it certainly is a severe immunological
disorder. The questions is: does anybody have experience and/or advise
on performing a stem cell transplant for this condition.

The patient is a 14 female with early onset JRA at age 2.  She has
received initially treatment with steroids and methotrexate with partial
response.  Enbrel (etanercept) treatment did not decrease her active
disease. IVIG infusions, another TNF blocker, remicade (infliximab),
IL-1 blocker, kineret (anakinra), IL-6 blocker (actemra), and even
orencia (abatacept) were  tried by our rheumatologist without success.
Finally, in the last 2 months she received a total of 3 infusions of
rituxan (rituximab) at 3 week intervals, again without significant
improvement. Currently, the patient is in a wheelchair, consuming a
significant dose of steroids but  still having a very active disease.
In summary this patient has an early onset,  severe refractory
active-destructive systemic-JRA.  

We wonder if she might benefit from stem cell transplantation.  If we
consider that this is really an immunodeficiency with an as jet
undetected molecular defect as suggested by the early once and
refractory course of the disease,  an autologous BMT probably would not
work? 

Any advise about additional diagnosis and treatment of this patients
would be much appreciated.

 

Thanks,

 

Ricardo U Sorensen, M.D.

For  Abraham Gedalia,M.D.

Professor of Pediatrics

Head, Division of Pediatric Rheumatology

LSU Health Sciences Center and Children's Hospital

New Orleans,Louisiana

Office: (504) 896-9385

E-Mail: agedal at lsuhsc.edu

 

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