title: Chronic periaortitis with thoracic aorta and epiaortic artery involvement: a systemic large vessel vasculitis?
abstract: OBJECTIVE
  Chronic periaortitis (CP) is a rare disease characterized by fibro-inflammatory tissue surrounding the abdominal aorta and the iliac arteries. Anecdotal reports have shown that CP may also involve other vascular districts, particularly the thoracic aorta. The aim of this study was to investigate the thoracic aorta and epiaortic artery involvement in CP.
  
  
  METHODS
  Patients were eligible if they had undergone imaging studies assessing inflammatory involvement of the thoracic aorta and its major branches (e.g. contrast CT, MRI or PET-CT). We explored the patterns of thoracic vessel involvement and compared the clinical characteristics of patients with and without thoracic disease. Where available, we also reviewed the thoracic vascular/perivascular tissue biopsies.
  
  
  RESULTS
  Of 153 CP patients seen between 1999 and 2012, 77 were eligible. Of these, 28 (36%) had thoracic involvement: 15 (54%) had thoracic periaortitis, with 7 also showing epiaortic artery involvement; 6 (21%) had periaortitis surrounding a thoracic aortic aneurysm, 2 of them with epiaortic artery involvement; 7 (25%) had a thoracic aortic aneurysm without periaortitis. Patients with thoracic disease were more frequently female (P = 0.01), were older (P = 0.001) and had a higher frequency of pain and constitutional symptoms (P = 0.02). Thoracic (peri)vascular biopsies revealed adventitial and peri-adventitial fibro-inflammatory patterns similar to those observed in abdominal CP.
  
  
  CONCLUSIONS
  In about one-third of patients, CP also involves the thoracic aorta and the epiaortic arteries, which supports the hypothesis of a systemic inflammatory disease of the large arteries.
authors.affiliation: Nephrology Unit, University Hospital of Parma.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Palmisano
authors.affiliation: Nephrology Unit, University Hospital of Parma.
authors.forename: Maria L
authors.initials: ML
authors.lastname: Urban
authors.affiliation: Department of Biomedical, Biotechnological and Translational Sciences, Section of Pathology, University of Parma.
authors.forename: Domenico
authors.initials: D
authors.lastname: Corradi
authors.affiliation: Radiology Unit, Parma University Hospital, Parma.
authors.forename: Rocco
authors.initials: R
authors.lastname: Cobelli
authors.affiliation: Nephrology Unit, University Hospital of Parma.
authors.forename: Federico
authors.initials: F
authors.lastname: Alberici
authors.affiliation: Nephrology Unit, University Hospital of Parma.
authors.forename: Federica
authors.initials: F
authors.lastname: Maritati
authors.affiliation: Nuclear Medicine and.
authors.forename: Annibale
authors.initials: A
authors.lastname: Versari
authors.affiliation: Rheumatology Unit, Santa Maria Nuova Hospital, IRCCS, Reggio Emilia, Italy.
authors.forename: Nicolò
authors.initials: N
authors.lastname: Pipitone
authors.affiliation: Rheumatology Unit, Santa Maria Nuova Hospital, IRCCS, Reggio Emilia, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Salvarani
authors.affiliation: Nephrology Unit, University Hospital of Parma.
authors.forename: Carlo
authors.initials: C
authors.lastname: Buzio
authors.affiliation: Nephrology Unit, University Hospital of Parma, augusto.vaglio@virgilio.it.
authors.forename: Augusto
authors.initials: A
authors.lastname: Vaglio
mesh_terms: D000367:Age Factors; D000368:Aged; D001011:Aorta / Q000473:pathology*; D001013:Aorta, Thoracic / Q000000981:diagnostic imaging* / Q000473:pathology*; D001027:Aortography; D001706:Biopsy; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012185:Retroperitoneal Fibrosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D012189:Retrospective Studies; D012737:Sex Factors; D056647:Systemic Vasculitis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D013896:Thoracic Diseases / Q000150:complications; D014057:Tomography, X-Ray Computed
keywords: aneurysms; aorta; giant cell arteritis; periaortitis; retroperitoneal fibrosis; vasculitis