Rare lung diseases: Williams – Campbell syndrome

Abstract

Williams – Campbell syndrome (WCS) is a rare developmental defect characterized by deficiency or complete absence of cartilage mainly in the subsegmental bronchi. Inferiority of the cartilaginous apparatus creates favorable conditions for the development of recurrent lower respiratory tract infection and, as a consequence, the formation of bronchiectasis, which ultimately leads to the appearance and progression of respiratory failure, as well as to a decrease in the quality of life of patients. Insufficient awareness of this disease among doctors leads to later diagnosis, and complex conservative therapy in the absence of specific treatment for WCS entails a positive effect only in the short term.

The aim. Demonstration of a clinical case of a rare respiratory disease – WCS in a 35-year-old man.

Conclusion. This case illustrates the complexities of diagnosing and treating WCS and highlights the importance of raising awareness among healthcare workers regarding this disease.