[CIS PIDD] some help from Chile

[Christina Price]

Patricia Roessler



I have been collecting patients just like yours here at Yale- patients with
autoimmune conditions who received Rituximab, were hypogammaglobulinemic,
normal peripheral B cell number but low switched/unswitched memory B cells,
presenting with frequent infections, sometimes refractory to antibiotics
and required IVIG.  About half of the patients have been given a diagnosis
of CVID 1a. The other half, we are waiting to see how they turn out. I give
the patients at least 2 years to fully recover their memory compartment.


What was her IgG level prior to Rituximab? In these patients that receive
RTX, the pre Rituximab treatment immune work up is critical.



Regarding your questions

1. I don't think the answer is clear. Yes I think these finding CAN be
secondary to Rituximab. The long term consequences of RTX are unknown. Your
patient has normal IgM and IgA levels, so does not fit the traditional
criteria for CVID diagnosis. However, her last dose of RTX was more than 2
years ago. I agree with you that autoimmunity and immune deficiency share
immune dysregulation. I am doing further work on my patients like this. If
you would like to send me her samples, I would be happy to include your
patient.


2. The literature tells us that patients with low IgG at onset of RTX have
more infection risk. Before your patients receives more RTX, I would wait
for the pneumococcal results. If they are low, vaccinate with
polysaccharide and conjugate vaccines. I would also check her vaccine
titers to Hib, Hepatitis B, tetanus, and others and vaccinate accordingly
BEFORE rituximab is given.



3. Unless she is seriously ill, it sounds like you have time to wait for
the pneumococcals. I would give IVIG. Your patient clinically has frequent
infections. While the bladder infections are common in MS, I have also seen
CVID patients with frequent UTIs. We reported on a young female with
myasthenia gravis with bilateral mastoiditis that responded very well to
IVIG.

Am J Otolaryngol. <http://www.ncbi.nlm.nih.gov/pubmed/22361345#> 2012 Feb
21.
Use of intravenous immunoglobulin to treat chronic bilateral otomastoiditis
in the setting of rituximab induced hypogammaglobulinemia.
Otremba MD<http://www.ncbi.nlm.nih.gov/pubmed?term=Otremba%20MD%5BAuthor%5D&cauthor=true&cauthor_uid=22361345>
, Adam SI<http://www.ncbi.nlm.nih.gov/pubmed?term=Adam%20SI%5BAuthor%5D&cauthor=true&cauthor_uid=22361345>
, Price CC<http://www.ncbi.nlm.nih.gov/pubmed?term=Price%20CC%5BAuthor%5D&cauthor=true&cauthor_uid=22361345>
, Hohuan D<http://www.ncbi.nlm.nih.gov/pubmed?term=Hohuan%20D%5BAuthor%5D&cauthor=true&cauthor_uid=22361345>
, Kveton JF<http://www.ncbi.nlm.nih.gov/pubmed?term=Kveton%20JF%5BAuthor%5D&cauthor=true&cauthor_uid=22361345>
.
Source

Departments of Surgery, Section of Otolaryngology, Head, and Neck
Surgery, Yale University School of Medicine, New Haven, CT.


http://www.ncbi.nlm.nih.gov/pubmed/22361345


Hope this is helpful. I have been looking at this issue for the last 2
years as your clinical problem has come up several times. It has become a
research interest, so I hope to be able to provide more answers in the
future.


Best of luck,


Christina



Christina C. Price, MD

Yale University School of Medicine

Instructor

Allergy and Clinical Immunology
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