Bronchiectasis in rare pulmonary diseases: A case series: Bronchiectasis in rare pulmonary diseases

Ines Maria Grazia Piroddi; Edmundo Rosales; Catia Cilloniz; Adrian Ceccato; Antonello Nicolini

Authors

Ines Maria Grazia Piroddi Respiratory Diseases Unit, Hospital of Sestri Levante, Sestri Levante, Italy
Edmundo Rosales Department of Pneumology, Hospital Clínic, CIBERES, IDIBAPS, Barcelona, Spain
Catia Cilloniz Department of Pneumology, Hospital Clínic, CIBERES, IDIBAPS, Barcelona, Spain
Adrian Ceccato Department of Pneumology, Hospital Clínic, CIBERES, IDIBAPS, Barcelona, Spain
Antonello Nicolini Respiratory Diseases Unit, Hospital of Sestri Levante, Sestri Levante, Italy

Keywords:

Autoimmune diseases, bronchiectasis, bronchiectasis outpatient clinic, rare diseases

Abstract

Bronchiectasis, defined as permanent dilatation of the airways, usually causes chronic cough and sputum production with intermittent bacterial exacerbations. Bronchiectasis can have a severe impact on quality of life for many patients due to these symptoms. Establishing the cause of bronchiectasis may be difficult. Even with exhaustive clinical, laboratory, and pathologic testing, up to 50-80% of cases of bronchiectasis may still be idiopathic. Congenital bronchiectasis is much rarer than previously considered. A variety of respiratory and systemic diseases such as autoimmune or rheumatologic diseases may be complicated by pathological bronchial dilatation, and therefore various medical specialists will be dealing with the condition in one-way or another. Some bowel diseases are associated with a variety of systemic manifestations including large and small airway involvement: One of the most commonly associated airway diseases is bronchiectasis. On this regard, we report five patients with bronchiectasis and rare diseases admitted to our hospitals since 2012. Patients were recruited over a period of 36 months after opening a bronchiectasis outpatient clinic.

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Bronchiectasis in rare pulmonary diseases: A case series