[PAGID] NEMO colitis

[Stephan Ehl]

Dear Colleagues,

a 2 year old boy with NEMO deficiency (full EDA-ID phenotype with 
pneumocystis, 2xinvasive staph, 2x enterococcal meningitis) was 
transplanted 8 months ago from his HLA-identical carrier sister 
(symptomatic with uveitis and aphtous stomatitis). He did not have 
colitis before the SCT, the procedure went well and he is now a full 
chimera.

One months ago he started to develop steroid-responsive colitis 
(requiring about 1 mg/kg for good control) with a histological picture 
of severe erosive inflammation with extensive intraepithelial 
neutrophils. No evidence of GvHD or infectious (eg. atyp. MB) disease.

We anticipated this complication due to the persisting epithelial NEMO 
deficiency, but are not sure about an appropriate (steroid-sparing) 
treatment concept. We would appreciate input concerning the following 
questions:
1. steroids and antibiotics?
2. sulfasalazine/azathioprine?
3. biologicals?
4. prognosis?

Jordan, you told me previously that you have seen colitis develop after 
SCT in NEMO - was the time frame similar? was it also independent of GvHD?

Thanks for your help,

Stephan

Prof. Dr. Stephan Ehl
Immunologie/Rheumatologie

UNIVERSITAETSKLINIKUM FREIBURG
Zentrum für Kinderheilkunde und Jugendmedizin
Mathildenstrasse 1, 
79106 Freiburg

Tel +49 761 270-4309 (4301) / Fax -4599
stephan.ehl at uniklinik-freiburg.de
http://www.uniklinik-freiburg.de/kinderklinik/live/forschung/immunologie.html