Bronchiectasis in a patient with CREST syndrome.
Bronchiectasis is an uncommon pulmonary manifestation of systemic sclerosis (SSc). We report the case of a 70-year-old woman with CREST syndrome and vasculitis who developed multifocal symptomatic bronchiectasis. The bronchiectasis and immunosuppressive therapy precipitated severe lower respiratory tract infection, which was fatal within a few months. The concomitant occurrence of bronchiectasis and SSc raises the possibility of a pathophysiological relationship. Several hypotheses can be put forward to explain the occurrence of bronchial wall damage leading to bronchiectasis. Whatever the mechanism, cases of bronchiectasis in patients with SSc should be reported to make physicians aware of the substantial risk associated with this combination.
Lavie
Frédéric
F
Department of Rheumatology School of Medicine Pitié-Salpêtrière and University Pierre & Marie Curie, Paris VI, Pitié-Salpêtrière Teaching Hospital, 83, boulevard de l'Hĵpital, 75651 Paris cedex 13, France.
Rozenberg
Sylvie
S
Coutaux
Anne
A
Koeger
Anne-Claude
AC
Bourgeois
Pierre
P
Fautrel
Bruno
B
D000368:Aged; D001987:Bronchiectasis / Q000188:drug therapy / Q000209:etiology / Q000473:pathology*; D017675:CREST Syndrome / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D003520:Cyclophosphamide / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone