CLINICAL BRIEF
Figure 2. Organ Systems Affected in CVID
For instance, dosing for autoimmune
conditions can range from 1 gram to 2
grams per kilogram of body weight,
whereas dosing for immune deficiencies
is typically 0.4 grams per kilogram.

In some cases, Rituxan (rituximab)
and other immunosuppressants such
as azathioprine (Imuran, Azasan) and
6-mercaptopurine (6-MP) (Purixan,
Purinethol) or mycophenolate (Cellcept)
and splenectomy have been used in
refractory (treatment-resistant) cases.

Joint manifestations. Joint conditions
resembling RA and SLE are other autoim-
mune conditions associated with CVID.

With these, damage and inflammation
occurs in the joints, specifically the syn-
ovium (a membrane that provides protec-
tion and lubrication of the joints), espe-
cially during movement. A wide range of
oral, subcutaneous and intravenous
treatments are available for RA and SLE.

However, for many who have RA and
SLE prior to developing CVID, it may
be unclear whether immunosuppressant
treatment for the primary disease pro-
duces low IgG levels or if the autoimmune
disease and the hypogammaglobulinemia
arise from the same immune defect.

Source: tommythedoc.weebly.com/immunology.html
Awareness Is Key
common, occurring in 6 percent to 10
percent of CVID patients. 3 Intestinal
inflammation can be difficult to treat,
and prolonged oral steroid treatment is
not ideal given the risk of immunosup-
pression. 4 Other treatments for IBD
such as oral budesonide (a second‐
generation steroid that allows local,
Autoimmune disorders occur in
approximately one-quarter of CVID
patients. Therefore, awareness of those
associated with CVID is helpful for
monitoring patients, particularly if
there are new signs and symptoms
consistent with autoimmunity.

MICHELLE GREER, RN, is senior vice
president of sales for Nufactor, a Specialty
Infusion Company. MARC GOLDSTEIN,
MD, is chief of allergy and immunology at
Pennsylvania Hospital at the University of
Pennsylvania and associate professor of clinical
medicine at Drexel University College of Medicine.

Immune dysregulation caused by CVID can
lead to a variety of autoimmune disorders
not necessarily influenced by age or gender.

References Other autoimmune conditions. Other
conditions associated with CVID
include IBD, juvenile idiopathic
arthritis, Sjogren’s syndrome, vitiligo,
reactive arthritis and autoimmune
hepatitis. IBD (chronic inflammation
of the digestive tract) is the most
1. Genetics Home Reference. Common Variable Immune Deficiency.

Accessed at ghr.nlm.nih.gov/condition/common-variable-immune-
deficiency#genes. 2. Michel, M, Chanet, V, Dechartres, A, et al. The Spectrum of Evans
Syndrome in Adults: New Insight into the Disease Based on the
Analysis of 68 Cases. Blood, (2009) 114 (15): 3167–3172. Accessed
at www.ncbi.nlm.nih.gov/pubmed/19638626.

3. Cunningham-Rundles, C, and Bodian, C. Common Variable
Immunodeficiency: Clinical and Immunological Features of 248
Patients. Clinical Immunology, 1999 Jul; 92(1):34-48. Accessed at
www.ncbi.nlm.nih.gov/pubmed/10413651. 4. Agarwal, S, and Cunningham-Rundles, C. Autoimmunity in
Common Variable Immunodeficiency. Current Allergy and Asthma
Reports, 2009 Sep; 9(5): 347–352. Accessed at www.ncbi.nlm.

nih.gov/pubmed/19671377. selective treatment of the gastrointestinal
tract and the liver, minimizing systemic
exposure), immunosuppressants azathio-
prine and 6-MP, and immunomodu-
latory monoclonal antibodies such as
infliximab, vedolizumad and ustek-
inumab can be used with various results.

IG Living
| August-September 2020
| IGLiving.com
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