[PAGID] Agamm with inclusion body myosistis

[raas0027 at umn.edu]

Hello everyone,

Any suggestions regarding therapies for inclusion body myosisits? Although 
there are a number of small trials with numerous agents (steroids, IVIG, 
azathioprine, TNF antagonisits, cytoxan, etc) none appear particularly 
effective.

The patient is a 65 y.o. gentleman with agammaglobulinemia (CD19 absolute 
number is ZERO, Btk analysis without obvious mutation). 10 years ago dx'd 
with lymphoma (CD20+, interesting) tx'd with CHOP+rituximab. Remains in 
remission.

Dx'd with 'polymyositis' based on clinical picture and muscle biopsy. 
Continued weakness and muscle atrophy despite steroids (oral and IV), high 
dose IVIG, azathioprine and Cellcept. This prompted repeat muscle biopsy 
which was consistent with inclusion body myositis.

Out of curiositiy, any anecdotal cases of inclusion body myositis in 
primary immune deficiency patients? [this is where the USIDNET registry 
will be helpful...]

Thanks for your thoughts.

-Jason


-- 
Jason Raasch, MD

Midwest Immunology Clinic
15700 37th Ave N
Suite 110
Plymouth, MN 55446

(Phone) 763.577.0008
(FAX)   763.5770192