Vnitr Lek 2015, 61(2):119-124

Interstitial lung diseases and granulomatoses associated common variable immunodeficiency.

Martina Doubková^1,*, Mojmír Moulis², Jana Skřičková¹

¹ Klinika nemocí plicních a tuberkulózy LF MU a FN Brno, pracoviště Bohunice, přednostka prof. MUDr. Jana Skřičková, CSc.

² Ústav patologie LF MU a FN Brno, pracoviště Bohunice, přednosta doc. MUDr. Leoš Křen, Ph.D.

Common variable immunodeficiency disorder belongs to the most common primary human immunodeficiencies and it is characterized by primary defective immunoglobulin production. Hypogammaglobulinemia manifests in every age, usually in adult people. There is no gender predisposition. The prevalence is 1 : 25 000-1 : 50 000. The ethiopathogenesis of the majority of CVIDs is unknown. The main clinical respiratory symptoms include recurrent respiratory infects, especially bacterial etiology, sinusitis, bronchitis, pneumonia, leading to bronchiectasis and lung fibrosis. Interstitial lung fibrosis and granulomatosis often manifest at diagnosis of CVID and they are negative prognostic factors of the disease.

Keywords: common variable immunodeficiency; granulomatosis; interstitial lung fibrosis

Received: September 23, 2014; Accepted: November 25, 2014; Published: February 1, 2015  Show citation