[CIS-PAGID] Pulmonary granuloma & CVID: Treament

[Routes, John]

Jason
We  see/treat alot of these patients---few questions---1. how was the biopsy
obtained?  In my experience, a transbronchial biopsy  does not get
sufficient tissue to make a definitive diagnosis----we always get an open
lung biopsy to exclude lymphoma, BOOP or other causes of ILD in CVID
patients. 2) What was the histology of the infiltrate, including phenotype
of the inflammatory cells? 3) Is there diffuse adenopathy with splenomegaly?

If this is granulomatous/lymphocytic interstitial lung disease (GLILD)--I
have not had good luck with prednisone---rarely clears the lung disease and
when you have a reasonable response, you need to maintain the patient on too
high dose of prednisone and cannot taper leading to unacceptable side
effects---We have used rituximab and azathioprine in 10-12 patients and have
had great results, with complete or near complete clearing of the
infiltrates and improved PFTs---we had a poster at the recent CIS meeting
that summarized our experience so it should be in the Journal of Clinical
Immunology pretty soon---happy to discuss in more detail if it would be of
help
Jack


John M. Routes, MD
Chief, Section of Allergy and Clinical Immunology
Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics
Department of Pediatrics
Children's Hospital of Wisconsin
Medical College of Wisconsin




> From: "raas0027 at umn.edu" <raas0027 at umn.edu>

> Reply-To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>

> Date: Tue, 24 May 2011 14:32:57 -0500

> To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>

> Subject: [CIS-PAGID] Pulmonary granuloma & CVID: Treament

> 

> 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