[CIS-PAGID] CVID + LIP

[Routes, John]

Friends/Colleagues
All of our patients had biopsies (all but 1 an open lung biopsy)--the infiltrate consisted of LIP, granuloma and follicular bronchiolitis—the phenotype in all cases at MCW included T cells and B cells (as well as macs)--fibrosis in longstanding cases---we monitored chest CT as well as PFTS by plethysmography----we not only had improvements in CT findings, but  PFTs----my experience my be different and acknowledge the very real possibility of referral bias, but the cases I have had demonstrate a slow diminution of pulmonary function if not treated----when at National Jewish, I tried a couple patients with rituxin alone and got some improvement, but not to the extent of combination chemotherapy---

For the last 18 months I have been in contact with Genentech (they might say pestered) and based on our CIS abstract/and newer data, in the last week have provided me with a letter of intent to supply rituxin for a study—(essential as the cost would be prohibitive otherwise) ===-I am in preliminary discussions with people at the NIH to see if they would fund a multicenter clinical study----what I would suggest is that get interested parties together to discuss the best possible protocols----I strongly believe we need tissue in all cases to correlate pathological findings with PFTs and CT findings------we (Nicki Chase, James Verbsky and I----Nicki especially deserves a TON of credit here) have collaborated with pathology and radiology at MCW to come up with a scoring system for HRCT scans and path----so I think we would be set to have everything be blindly read at one center and scored pre and post Rx---

If you are interested in participating in such a trial, give me an email at jroutes at mcw.edu

thanks!

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
9000 W. Wisconsin Ave.
Milwaukee, WI  53226-4874

Phone: 414-456-4802; 414-266-6997
Fax: 414-456-6487 (Clinical)
Fax: 414-456-6323 (Laboratory)
Email: jroutes at mcw.edu




________________________________
From: "Cunningham-Rundles, Charlotte" <charlotte.cunningham-rundles at mssm.edu>
Reply-To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Date: Fri, 22 Jul 2011 20:25:13 -0500
To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>, "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Subject: Re: [CIS-PAGID] CVID + LIP

Hi,

 it is true that we need a new idea for these patients.    However,   I am thinking that we need to be sure that we are treating more X ray findings. So many have nodules and lymphoid infiltrates, but not the decompensation that Terri's patient had. So I think I would rather follow these folks on a macrolide and busesonide perhaps and not do more unless diffusion becomes abnormal or deteriorates .

Then, I,d like to know if the infiltrates are a lot of  B cells or not.   Rituxan makes a lot of sense here.  as for azathioprine, not sure over the longer haul on that.     We had two lymphomas on pts on methotrexate for autoimmunity.   Short run, anything works. Long run, well, that does need a trial, but also similar patients with biopsy data on the infiltrates, hopefully with cell staining.


Charlotte

Charlotte Cunningham-Rundles MD PhD



-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org on behalf of Terri Tarrant
Sent: Fri 7/22/2011 2:32 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] CVID + LIP

Moving forward prospectively or historical case compilation? We're game either way.
Terri Tarrant

Sent from my iPhone


On Jul 22, 2011, at 2:15 PM, "Nelson, Robert P Jr" <ronelson at iupui.edu> wrote:


> What about considering a mutlicenter clinical trial so we could contibute our patients to an organized effort?  Bob

> ________________________________________

> From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] on behalf of Fleisher, Thomas (NIH/CC/DLM) [E] [TFleishe at cc.nih.gov]

> Sent: Friday, July 22, 2011 10:32 AM

> To: Routes, John; pagid at list.clinimmsoc.org

> Subject: Re: [CIS-PAGID] CVID + LIP

>

> Sorry for copying all on a simple question to Jack.

>

> Thomas A. Fleisher, M.D.

> Chief, Department of Laboratory Medicine

> NIH Clinical Center

> 301 496-5668 (T)

> 301 402-1612 (F)

>

> -----Original Message-----

> From: Routes, John [mailto:jroutes at mcw.edu]

> Sent: Friday, July 22, 2011 8:32 AM

> To: pagid at list.clinimmsoc.org

> Subject: Re: [CIS-PAGID] CVID + LIP

>

> We have treated over ten patients with combination chemotherapy consisting of rituxin and azathioprine with outstanding results no infections and in many cased complete or near complete clearing.

>

> Sent from my Verizon Wireless BlackBerry

>

> ________________________________

> From: Terri Tarrant <tarra002 at gmail.com>

> Sender: "pagid-bounces at list.clinimmsoc.org" <pagid-bounces at list.clinimmsoc.org>

> Date: Fri, 22 Jul 2011 06:16:47 -0500

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

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

> Subject: Re: [CIS-PAGID] CVID + LIP

>

> Yes, we have 3 cases that responded well (2 with CVID and 1 idiopathic LIP).  We used the Eisenberg protocol of 1 g IV day 0 and day 14 with 100 mg IV solumedrol pre-med.  We did not use in combination with other drugs.  We did not see an increase in infections.  All patients needed redosing at 9-12 month intervals.

>

> Terri Tarrant

>

> On Thu, Jul 21, 2011 at 10:07 PM, Church, Joseph <JChurch at chla.usc.edu<mailto:JChurch at chla.usc.edu>> wrote:

> Colleagues:

>

> A 60yo female with long term CVID has had lymphoid interstitial pneumonitis that was biopsy documented in 2005.

>

> She had developed worsening of symptoms over the past 6 months.

>

> She is trying to avoid increasing her prednisone, currently at 10mg/d, because of coronary artery disease and hypertension; she could not tolerate CellCept.

>

> Has anyone tried Rituximab in this setting?

>

> Joe Church, MD

> Children's Hospital Los Angeles

>

>

>

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> --

> Terri Tarrant, MD

> Assistant Professor of Medicine

> Thurston Arthritis Research Center

> Lineberger Cancer Center Member

> CB # 7280, 3300 Manning Dr.

> Chapel Hill, NC 27599

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