[CIS PIDD] [cis-pidd] Chronic norovirus enteritis, update and Q

[Sullivan, Kathleen]

Concur with Klaus that immune suppression (I love rapamycin) seems to be a reasonable approach.  We would also consider RItuximab even though there are no peripheral blood B cells, if there are any B cells in the tissue.

Kate
Kate Sullivan, MD PhD
Wallace Chair 
Chief of Allergy Immunology
ARC 1216 CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363


> On Dec 31, 2014, at 1:33 AM, Mikko Sepp�nen <mikko.seppanen at hus.fi> wrote:
> 
> Dear all, 
> 
> we have now attempted to our most affected patient all other in Listserve-suggested means of therapy (IFN, IFN+ escalated riba, the latter has caused further diarrhea and vomiting) except daily p.o. IgG , to no avail. IFN+ escalated riba for 2.5 weeks did not clear norovirus. Due to the side effects and further weight loss caused by it, the patient cannot continue this approach. 
> 
> Q: Robert Sokolic suggested 5ml/day of Gamunex, mixed with orange juice. Was a 5% or 10% prep used at the time?
> I seem to recall that someone had recently used this approach successfully as well. Their dose?
> 
> Patient case in more detail: 
> 
> The patient is 31-y young recently married female, 174 cm tall, weighs only 40 kg now (!!!), has CVID of unknown genetic origin (WES: no known genes). 
> 
> CVID noted after ITP --> ITP+AINP (Evans)and  S.aureus sepsis 2004, recurrent LRTIs since 1998. Bronchiectasis already when diagnosed, HRCT mild follicular bronchiolitis. 
> 
> EGD- and IC-scopy performed a year ago, after following celiac diet no active cellular inflammation which You could treat with immunomodulators, however severe malabsorption of protein, calories, lipid soluble vitamins continues.... 
> 
> Ciprofloxacin a.s.f. attempted ex juvantibus to no avail, bacterial overgrowth seems unlikely. Further causes for diarrhea tested and not found (We are actually preparing a manuscript on GI manifestations in CVIDs in our cohort, this lady has been tested for all known and unknown alternative causes...).
> 
> We are left with - in biopsies after celiac diet very moderate - enteropathy resembling coeliac disease with only subtotal villus atrophy (HLA-DQ8+), mild chronic PBC-like cholangiopathy: sinus dilation with mild sinusoidal fibrosis in biopsy, in ERC mild cholangipathy (Adursal), chronic norovirus, severe diarrhea and severe malabsorption. She also has gastroparesis of completely unknown origin (no SFN, no diabetes...).  
> 
> She further has asthma, lymphatic hyperplasia (spleen 13 cm). She receives s.c. Gammanorm 16.5% 120ml /week (SIC!), P-IgG before norovirus therapy attempts 12.9-13.1, now 10.9 g/l...(more diarrhea)
> 
> She has no switched-memory B cells, during follow up loses all B cells from blood (no thymoma), CD4 and CD8 counts normal, NKs 0.04 (low). Her Treg counts are normal, though function seemed somewhat but mildly impaired. Rapamycin not attempted.
> 
> Any further suggestions?
> 
> I appreciate Your time spent, thanks in advance,
> 
> Mikko Seppänen, MD, PhD
> Helsinki, Finland
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