[CIS-PAGID] Pulmonary granuloma & CVID: Treament

[raas0027 at umn.edu]

Good Afternoon All:

Three adult patients all with newly diagnosed CVID (very low IgG; absent 
IgA; low IgM; all with some degree of memory B-cell derangement; one with 
low expression of Baff-R). All are starting on IgG.

All with pulmonary granulomatous disease; two confirmed with biopsy.

One with pulmonary "sarcoidosis" as initial diagnosis; CVID dx'd afterward. 
Has been on chronic prednisone to good effect but hoping to transition to 
non-steroid therapy.

As noted in previous PAGID discussions, anecdotal experience reported with 
hydroxychloroquine, azathioprine, CellCept, methotrexate in similar cases.


My thinking in these 3 patients is to treat initially with prednisone, 
taper and see if remission/improvement in granulomatous disease can be 
maintained with hydroxychloroquine (seems the most inocuous of choices and 
PAGID-ers out there have reported good outcomes).

Thoughts?

Anyone have standard of practice regarding how long to treat initally with 
prednionse before tapering? Dose of Plaquenil? Follow-up CTs?

Skip Plaquenil altogether?

As always, thanks for your input.

-Jason


-- 
Jason Raasch, MD

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

(Phone) 763.577.0008
(FAX)   763.5770192