RESUMO
Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly includes clinical and radiographic findings of tracheobronchial dilatation and recurrent respiratory infections. MKS is a very rare pathology, especially in the paediatric age group which makes it a diagnostic challenge. A 4-year-old girl suffered from dyspnea, recurrent respiratory infections and joint pain. Chest radiography detected peribronchial reinforcement and CT-scan revealed extended tracheal dilatation and bronchiectasis. In addition to MKS our patient was diagnosed with juvenile idiopathic arthritis (JIA) and scleroderma. MKS can be caused by congenital disorder or acquired aetiology. Several connective tissue diseases have been associated with MKS but no cases of JIA or scleroderma are described previously. Our case illustrates that patients who suffer from recurrent respiratory infections with unsatisfactory evolution and unspecific chest X-ray alteration, MKS always has to be considered in the differential diagnosis particularly in patients who suffer from connective tissue diseases.
