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

[Bodo Grimbacher]

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 thoughŠ
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
www.uniklinik-freiburg.de/cci

Von:  Stan Ress <stan.ress at uct.ac.za>
Antworten an:  CIS-PIDD <cis-pidd at lyris.dundee.net>
Datum:  Monday 8 June 2015 21:42
An:  CIS-PIDD <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 RTI¹s. 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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