title: Inflammatory disease of the aorta: patterns and classification of giant cell aortitis, Takayasu arteritis, and nonsyndromic aortitis.
abstract: Inflammatory diseases of the aorta, other than those of known infective etiology, are poorly understood. We analyzed a large series of affected patients who had histologic diagnoses with a view to improving the classification of the extent of aortitis to enable a more targeted approach of treatment.
  
  
  
  Between 1996 and 2012, we operated on 7551 patients with ascending or aortic arch disease, of whom 279 had clinically diagnosed inflammatory disease. Of these, 156 (2%) were found to have aortitis on histologic examination.
  
  
  
  Patients were divided into 4 histologically based groups: giant cell aortitis, 31% (49/156); Takayasu arteritis, 5.1% (8/156); isolated aortitis, 59% (92/156); and other, 4.5% (7/156). Patterns of anatomic extent were also analyzed, and in particular it was noted that giant cell aortitis and isolated aortitis had more extensive disease. In addition, specimen analysis suggested early indications of unrecognized preexistent infections. Death after surgery occurred in 3.2% (5/156), and stroke in 1.9% (3/156). Kaplan-Meier survival at 8 years was 55%. We present a classification for disease extent and management.
  
  
  
  Aortitis continues to be a conundrum; however, good results are achievable with surgery. Intervention should be based on a clearer understanding of the histologic pattern and extent of disease, which helps in subsequent targeted disease management.
authors.affiliation: Department of Cardiothoracic Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio. Electronic address: svenssl@ccf.org.
authors.forename: Lars G
authors.initials: LG
authors.lastname: Svensson
authors.affiliation: Department of Cardiothoracic Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Amr
authors.initials: A
authors.lastname: Arafat
authors.affiliation: Department of Cardiothoracic Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Eric E
authors.initials: EE
authors.lastname: Roselli
authors.affiliation: Department of Cardiothoracic Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Jay
authors.initials: J
authors.lastname: Idrees
authors.affiliation: Department of Rheumatology, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Allison
authors.initials: A
authors.lastname: Clifford
authors.affiliation: Department of Anatomic Pathology, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Carmela
authors.initials: C
authors.lastname: Tan
authors.affiliation: Department of Rheumatology, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Gary
authors.initials: G
authors.lastname: Hoffman
authors.affiliation: Genomic Medicine Institute, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Charis
authors.initials: C
authors.lastname: Eng
authors.affiliation: Department of Rheumatic and Immunologic Disease, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Carol
authors.initials: C
authors.lastname: Langford
authors.affiliation: Department of Cardiothoracic Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: E Rene
authors.initials: ER
authors.lastname: Rodriguez
authors.affiliation: Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Heather L
authors.initials: HL
authors.lastname: Gornik
authors.affiliation: Department of Cardiothoracic Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Eugene
authors.initials: E
authors.lastname: Blackstone
authors.affiliation: Department of Cardiothoracic Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Joseph F
authors.initials: JF
authors.lastname: Sabik
authors.affiliation: Department of Cardiothoracic Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Bruce W
authors.initials: BW
authors.lastname: Lytle
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001011:Aorta / Q000473:pathology* / Q000601:surgery; D018572:Disease-Free Survival; D005260:Female; D013700:Giant Cell Arteritis / Q000145:classification / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009820:Ohio; D011183:Postoperative Complications / Q000401:mortality; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012307:Risk Factors; D013625:Takayasu Arteritis / Q000145:classification / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D013997:Time Factors; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000009:adverse effects; D055815:Young Adult