Article

The discovery of hypogamma-globulinemia, which is defined as a plasmatic level of
immunoglobulin (Ig) under 5g/L is rare in clinical practice. However, the
management of immunodepressed patients in rheumatology, sometimes due
to the use
of immunosuppressive treatments such as anti-CD20 in chronic inflammatory
rheumatisms, increases the risk of being confronted to this situation. The discovery of hypogammaglobulinemia in clinical practice, sometimes by chance,
must never be neglected and requires a rigorous diagnosis approach. First of all,
in adults, secondary causes, in particular lymphoid hemopathies or drug-related
causes (immunosuppressors, antiepileptics) must be eliminated. A renal (nephrotic
syndrome) or digestive (protein-losing enteropathy) leakage of Ig is also
possible. More rarely, it is due to an authentic primary immunodeficiency (PID)
discovered in adulthood: common variable immunodeficiency (CVID) which is the
most frequent form of PID, affects young adults between 20 and 30 years and can
sometimes trigger joint symptoms similar to those in rheumatoid arthritis; or
Good syndrome, which associates hypogammaglobulinemia, thymoma and recurrent
infections around the age of 40 years. In most cases, after confirming
hypogammaglobulinemia on a second test, biological examinations and
thoracic-abdominal-pelvic CT scan will guide the diagnosis, after which the
opinion of a specialist can be sought depending on the findings of the above
examinations. At the end of this review, we provide a decision tree to guide the
clinician confronted to an adult-onset hypogammaglobulinemia.
CI - Copyright (c) 2010 Societe francaise de rhumatologie. Published by Elsevier SAS.
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Langue : ANGLAIS