Common variable immunodeficiency is the second most common primary immunodeficiency with a prevalence of approx. 1/10.000-50.000. The clinical challenge is early diagnosis and efficient supportive treatment. The purpose of the present article is to focus on the complexity of the disease, including the risk of a long pre-diagnostic period and to focus on the sarcoidosis-like variant and the possible impact of immunoglobulin subclass deficiency.

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