[CIS-PAGID] CVID + LIP

[Klaus Warnatz]

Dear all,

Stephan just forwarded me the emails regarding treatment of interstitial 
lung disease in CVID.
With great interest I read tehj discussion since we are in a similar 
discussion in Freiburg.
I agree with Charlotte, that we have not enough evidence in the long 
term what and how well we are treating.
We have no experience on RTX in lung disease and CVID.  the infiltrates 
are usually mixed, very few BALTs, mostly granuloma with interstitial 
infiltration of lymphocytes what I understand from our pathologists and 
pulmonologists are not really LIP in the precise sense.
Patients with clinical symptoms we have had on steroids and Steroids 
with CSA most responded initially, during the course it seemed so that 
granuloma were coming and going (often not affecting lung function).
We therefore set up a prospective randomized placebo controlled trial 
which will start in the beginning of 2012 where we try to understand 
whether we can inhibit progress of preclinical interstitial lung disease 
in CVID with steroids alone. This trial targets a different question 
than the proposed trial by Jack since we want to rather work on early 
prevention of progress rather than treatment of clinically manifest disease.
The idea is that we identify CVID patients with ILD by CT, prove by 
biopsy and or BAL, at a time point when the patient has no clinical 
symptoms and no abnormalities in lung function, pO2 or DLCO. we 
randomize these patients and follow them for 4 years. The primary 
endpoint would be Abnormal lung function at rest or exercise as well as 
side effects.
Due to logistics we will be able to run this trial only in European 
centers but are very happy to share our experience as much as possible 
with yours. Please contact me if you would like any further information.

Looking forward to your ideas

Klaus

PS: By the way, Can I hereby apply to become included into the CIS-PAGID 
mailing list?

-- 
Prof. Dr. Klaus Warnatz

UNIVERSITÄTSKLINIKUM FREIBURG
University Freiburg-Medical Center
Centrum für Chronische Immundefizienz (CCI)
Abteilung für Rheumatologie und Klinische Immunologie


Tel +49-761-270-77640 / Fax -71000 / Pager: 12-7100

Breisacher Str. 117, 79106 Freiburg, Germany
klaus.warnatz at uniklinik-freiburg.de
http://www.uniklinik-freiburg.de