[CIS-PAGID] Pulmonary granuloma & CVID: Treament

[Charlotte Cunningham-Rundles@mssm.edu]

This is not simple and I am not sure there is any good way to get this
resolved. It is probably like other granulomatous diseases, only controlled.
The CT will never clear up anyway. In my view, in the lungs, it does spell
more trouble, as Jack says, because of the scarring and inflammation. But as
it can¹t be eliminated, I¹d use  chronic low dose steroids and placquenil (
200 am and pm)  if the DLCO and lung functions are ok and stable, then move
into anything else if they are not.  We also use a macrolide in all cases
for the anti-inflam and maybe antimicrobial effects, and also inhaled
budesonide.  We have used remicade with no benefit, but I admit that it was
late in the day and maybe not useful because of that.

If you get a new idea or something is helpful, let everyone know!

Charlotte


On 5/24/11 3:32 PM, "raas0027 at umn.edu" <raas0027 at umn.edu> wrote:


> 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

> 


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