title: Severe, chronic cough caused by pulmonary arteriovenous malformations in a patient with hereditary haemorrhagic telangiectasia: case report.
abstract: BACKGROUND
  Patients with pulmonary arteriovenous malformations usually complain of dyspnoea upon exertion, fatigue or migraine, or may be asymptomatic. We describe a patient with an unreported manifestation of a pulmonary arteriovenous malformation: a severe chronic cough.
  
  
  METHODS
  A 51-year old Caucasian non-smoking female police officer presented with a chronic cough. She had been diagnosed with hereditary haemorrhagic telangiectasia in 1992. She complained of a severe, dry cough at the time of the diagnosis and a pulmonary arteriovenous malformation in the upper left lobe as demonstrated by CT of the chest. The fistula was occluded and the cough disappeared rapidly but resumed in 1994. Recanalisation of the fistula led to a new embolisation procedure, and the cough disappeared. Similar episodes occurred in 1998 and 2004, leading to embolisation of a fistula in the right lower lobe and reperfused fistula in the upper left lobe, respectively. The patient was referred to our research team in 2010 because of reappearance of her dry cough that was more pronounced during exercise and exposure to volatile irritants, and absent during the night. Despite extensive investigations, no cause was found other than reperfusion of the fistula in the left upper lobe. The malformation was not accessible to embolisation, leading us to recommend surgical excision of the malformation. A surgeon undertook atypical resection of the left upper lobe in 2012. The cough disappeared immediately after surgery and has not recurred.
  
  
  CONCLUSIONS
  Physicians caring for patients with pulmonary arteriovenous malformations should know that a severe, chronic cough can be caused by the malformation. A cough associated with a pulmonary arteriovenous malformation can be treated effectively by embolisation but may resume in cases of reperfusion of the malformation. In our case, the severity of the cough led to surgical excision because embolisation was not possible. The mechanism of action of this cough remains to be determined.
authors.affiliation: APHP, Department of Pulmonology and Thoracic Oncology, Ambroise Paré Hospital, Boulogne, France. emjutant@gmail.com.
authors.forename: Etienne-Marie
authors.initials: EM
authors.lastname: Jutant
authors.affiliation: Foch Hospital, Department of Thoracic Surgery, Suresnes, France. p.puyo@hopital-foch.org.
authors.forename: Philippe
authors.initials: P
authors.lastname: Puyo
authors.affiliation: APHP, Department of Radiology, Ambroise Paré Hospital, Boulogne, France. mostafa.elhajjam@apr.aphp.fr.
authors.forename: Mostafa
authors.initials: M
authors.lastname: El Hajjam
authors.affiliation: APHP, Department of Pulmonology and Thoracic Oncology, Ambroise Paré Hospital, Boulogne, France. sandra.blivet@apr.aphp.fr.
authors.forename: Sandra
authors.initials: S
authors.lastname: Blivet
authors.affiliation: APHP, Department of Neuroradiology, Lariboisière Hospital, Paris, France. emmanuel.houdart@lrb.aphp.fr.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Houdart
authors.affiliation: APHP, Department of Pulmonology, Bichat Hospital, Paris, France. michel.aubier@bch.aphp.fr.
authors.forename: Michel
authors.initials: M
authors.lastname: Aubier
authors.affiliation: APHP, Department of Radiology, Ambroise Paré Hospital, Boulogne, France. pascal.lacombe@apr.aphp.fr.
authors.forename: Pascal
authors.initials: P
authors.lastname: Lacombe
authors.affiliation: APHP, Department of Pulmonology and Thoracic Oncology, Ambroise Paré Hospital, Boulogne, France. thierry.chinet@apr.aphp.fr.
authors.forename: Thierry
authors.initials: T
authors.lastname: Chinet
mesh_terms: D000792:Angiography; D001164:Arteriovenous Fistula / Q000000981:diagnostic imaging / Q000209:etiology* / Q000628:therapy; D021721:Balloon Occlusion; D002908:Chronic Disease; D003371:Cough / Q000209:etiology* / Q000628:therapy; D004621:Embolization, Therapeutic; D005260:Female; D006801:Humans; D008875:Middle Aged; D011013:Pneumonectomy; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging; D012008:Recurrence; D012720:Severity of Illness Index; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*