Interstitial lung diseases and granulomatoses associated common variable immunodeficiency.

Česká verzia

Authors: Martina Doubková ¹; Mojmír Moulis ²; Jana Skřičková ¹
Authors place of work: Klinika nemocí plicních a tuberkulózy LF MU a FN Brno, pracoviště Bohunice, přednostka prof. MUDr. Jana Skřičková, CSc. ¹; Ústav patologie LF MU a FN Brno, pracoviště Bohunice, přednosta doc. MUDr. Leoš Křen, Ph. D. ²
Published in the journal: Vnitř Lék 2015; 61(2): 119-124
Category: Review

Summary

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.

Key words:
common variable immunodeficiency – granulomatosis – interstitial lung fibrosis

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