[CIS PIDD] [cis-pidd] Renal transplantation in CVID

Tue Jun 9 02:32:53 EDT 2015

Dear Stan,

Consider Candida and Histoplasma, Bartonella and Coxiella.
Regards
Philipp

Dr. Philipp Henneke
Professor
Head Ped. Infectious Dis & Rheumatology
UNIVERSITY MEDICAL CENTER FREIBURG
Center for Chronic Immundefiency (CCI)
Center for Pediatrics & Adolesc. Medicine
phone assist/ office + 49(0)761270 77640
mobil +49 (0)162 285 2481
fax +49 (0) 761 270 77600
Breisacherstr. 117-2.OG, 79106 Freiburg

Am 09.06.15 07:35 schrieb "Prof. Dr. Klaus Warnatz" unter
<klaus.warnatz at uniklinik-freiburg.de>:

>Dear Stan, Dear Jack,
>
>I agree with Jack clinically it sounds like NRH the unusual findings
>however are the granulomatous lesions and the increasing liver enzymes,
>although this can happen. Jack, we don't usually apply immunosuppressive
>treatment to NRH since the evidence is rather low that this is helpful.
>Like Mel asked which drug du you usually apply? IN this concrete case
>however with the presence of granulomatous disease and elevated enzymes
>Stan you might consider. IN case of portal hypertension, splenomegaly and
>hypersplenism we have successfully applied TIPS (usually however after
>the first bleeding event or advanced state of varicosis) which not only
>reduced the portal vein pressure but also improved the hypersplenism. We
>have seen NRH more often in the context of inflammatory GI disease, are
>there any signs for this? I would also recommend to exclude an infectious
>origin of the liver disease if not already done including atypical
>mycobacterial disease (we have currently one patient which looked like
>NRH and had granulomatous disease T cell infiltration of the liver due to
>atypical mycobacteria). if these are excluded I would try treating the
>patient with systemic steroids also for the renal disease, since we have
>one patient with interstitial nephritis who stabilized nicely on steroids
>despite advanced renal insufficiency. I would not wait for the
>transplant. 
>In regard to the risk of immunosuppressive therapy yes you run the risk
>of increased infection, even opportunistic infection which you obviously
>need to control for. we add PjP prophylaxis in patients with high
>immunosuppression especially when the CD4 count drops under 200/µbut with
>the renal failure you have to probably go for pentamidine inhalation.
>these are my thoughts
>Jack, I would be very interested in your experience with
>immunosuppression in NRH
>best regards
>
>klaus
>
>
>Prof. Dr. med. Klaus Warnatz
>
>MEDICAL CENTER  UNIVERSITY OF FREIBURG
>Center for Chronic Immunodeficiency  CCI
>Department of Rheumatology and Clinical Immunology
>
>Breisacher Str. 117, 79106 Freiburg, Germany
>Tel. +49 761 270 77640 / FAX -71000 / Pager 12-7100
>klaus.warnatz at uniklinik-freiburg.de
>
>www.uniklinik-freiburg.de/cci
>
>
>________________________________________
>Von: Stan Ress <stan.ress at uct.ac.za>
>Gesendet: Dienstag, 9. Juni 2015 00:09
>An: CIS-PIDD
>Betreff: RE: [cis-pidd] Renal transplantation in CVID
>
>Thanks so much to Bodo Grimbacher, Mary Hogan, & John Routes for your
>very helpful replies.
>
>I think Jack's correct about the liver. I certainly assumed it was
>nodular regenerative hyperplasia based on my reading of this liver
>complication in CVID (Ward et al Clin & Exp Immunology: 2008, 153, 331-7,
>Fuss et al J. Clin Immunol 2013, 33(4) 748-58). The actual liver
>histology report described granulomatous inflammation with granulomas in
>the portal tract accompanied by an infiltrate of lymphocytes, histiocytes
>and eosinophils. There were necro-inflammatory foci in the lobule, also
>with inflammatory infiltrates. Reticulin stain showed foci of "focal
>nodular hyperplasia" with nodules of hyperplastic hepatocytes with liver
>cell plates two cells thick. Bile Sirius Red stain shows extensive
>pericellular fibrosis in zone 3. The inflamed portal tracts show fibrous
>expansion.
>
>So this fits NRH rather than FNH? And needs more aggressive
>immunosuppressive therapy?
>
>Thanks so much again.
>
>Stan
>--------------------------------------------------------------------------
>-----------
>Stanley Ress
>Emeritus Associate Professor of Medicine, UCT
>Specialist physician & Clinical Immunologist
>UCT Private Academic hospital,
>Anzio Road, Observatory,
>Cape Town, 7925
>South Africa
>TEL:INTERN. + 2721-4421966 or 4421816
>FAX:   "    + 2721-(0)865173095
>Cell: 0833115482
>email: stan.ress at uct.ac.za
>
>-----Original Message-----
>From: Routes, John [mailto:jroutes at mcw.edu]
>Sent: 08 June 2015 10:06 PM
>To: CIS-PIDD
>Subject: Re: [cis-pidd] Renal transplantation in CVID
>
>Stan
>Are you sure the liver pathology wasn't nodular regenerative
>hyperplasia-certainly has the clinical features of this? If so, then
>immunosuppressives would be needed as this is increasingly recognized as
>a potentially fatal complication of CVID 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: Office 414-266-6840
>Fax: 414-955-6487 (Clinical)
>Fax: 414-955-6323 (Laboratory)
>Email: jroutes at mcw.edu<mailto:jroutes at mcw.edu>
>
>From: Bodo Grimbacher
><bodo.grimbacher at uniklinik-freiburg.de<mailto:bodo.grimbacher at uniklinik-fr
>eiburg.de>>
>Reply-To: CIS-PIDD
><cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
>Date: Monday, June 8, 2015 at 2:59 PM
>To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
>Subject: Re: [cis-pidd] Renal transplantation in CVID
>
>Dear Stan,
>We have many patients with CVID and autoimmune features on
>immunosupressive treatment.
>If you take care of good trough levels and give antibiotics early and
>long enough, the bronchiectatic lung disease may not progress at all.
>Immunosupression may actually help the granulomatous complications seen
>in your patient.
>So it is a clear option for your patient.
>We do prefer steroids and MMF over the other immunosupressants though.
>Best, Bodo
>****************************************
>Univ.-Prof. Dr. med. B. Grimbacher
>
>Scientific-Director
>CCI-Center for Chronic Immunodeficiency
>UNIVERSITÄTSKLINIKUM FREIBURG
>Tel.: 0761 270-77731  Fax: -77744
>Engesserstraße 4, 79108 Freiburg
>bodo.grimbacher at uniklinik-freiburg.de<mailto:bodo.grimbacher at uniklinik-fre
>iburg.de>
>www.uniklinik-freiburg.de/cci
>
>Von: Stan Ress <stan.ress at uct.ac.za<mailto:stan.ress at uct.ac.za>>
>Antworten an: CIS-PIDD
><cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
>Datum: Monday 8 June 2015 21:42
>An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
>Betreff: [cis-pidd] Renal transplantation in CVID
>
>Dear Colleagues,
>
>I would be grateful for advice regarding further management in a
>complicated case of CVID.
>
>A 35 year-old lady was diagnosed with CVID in 2008 and commenced on IVIG.
>She has had a complicated course. In 2010  a chest CT scan showed
>bilateral bronchiectasis as a result of recurrent RTI's. She then
>developed hepatomegaly and progressive splenomegaly. Bone marrow
>trephines done at another centre in 2008 & 2010 showed normal T-and
>B-cell gene rearrangements, but she developed severe bicytopenia due to
>hypersplenism, and eventually required splenectomy in April 2014 when
>splenic size was 24 cm, platelet count < 80, WCC  < 1.5. US and MRI of
>liver indicated increased porta-venous shunting of blood with portal
>hypertension, liver biopsy reported features compatible with focal
>nodular hyperplasia (FNH) with T-cell infiltration but no B-cells
>present. Liver enzymes then progressively increased post-splenectomy
>(alkaline phosphatase & gamma GT especially), this has improved on low
>dose prednisone. However, her renal function had also deteriorated and
>simultaneous renal biopsy (at the time of splenectomy)  was reported as
>showing interstitial nephritis.
>
>She is managed on SC Immunoglobulin replacement, her current renal
>function has been stable with serum urea of 22 mmol/L  (2.8-7.2),
>creatinine of 371 umol/L (estimated GFR of 12 ml/min). Her nephrologist
>is exploring the possibility of a live related renal transplant and asks
>about the experience in this regard. While the QOL on haemodialysis would
>be less good than following a renal transplant, my concern is for the
>effect of immune suppression on an already immunocompromised patient who
>also has bronchiectasis.
>
>We would appreciate any advice and would also be grateful to hear of any
>other experience with regard to transplantation in CVID patients.
>
>Many thanks.
>
>Stan
>-------------------
>Stanley Ress
>Emeritus Associate Professor of Medicine, UCT Specialist physician &
>Clinical Immunologist UCT Private Academic hospital, Anzio Road,
>Observatory, Cape Town, 7925 South Africa TEL:INTERN<tel:INTERN>. +
>2721-4421966 or 4421816
>FAX:   "    + 2721-(0)865173095
>Cell: 0833115482
>email: stan.ress at uct.ac.za<mailto:stan.ress at uct.ac.za>
>
>
>________________________________
>UNIVERSITY OF CAPE TOWN
>
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