title: Acute aortitis and aortic incompetence due to systemic rheumatological disorders.
abstract: We report the clinical, laboratory and echocardiographic features of five cases of aortic incompetence associated with ankylosing spondylitis, rheumatoid arthritis and undefined connective tissue diseases. Immunosuppression with steroids and cytotoxic agents was used to suppress aortic root inflammation in four cases; in three the aortic root size stabilized and the patients remain well with no evidence of increasing aortic incompetence. In one case, control of the inflammatory process was never fully achieved for any length of time and the patient died shortly after aortic valve replacement. A fifth case required urgent valve replacement and remains well. A systemic rheumatological disorder should be considered in cases of apparent "lone" aortic incompetence and conversely aortic incompetence should not be overlooked in established systemic rheumatological disease. Immunosuppressive therapy may prevent or delay the need for aortic valve replacement in such cases.
authors.affiliation: University of Birmingham, Department of Cardiovascular Medicine, U.K.
authors.forename: J N
authors.initials: JN
authors.lastname: Townend
authors.forename: P
authors.initials: P
authors.lastname: Emery
authors.forename: M K
authors.initials: MK
authors.lastname: Davies
authors.forename: W A
authors.initials: WA
authors.lastname: Littler
mesh_terms: D000328:Adult; D001022:Aortic Valve Insufficiency / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D001025:Aortitis / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D001706:Biopsy; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004452:Echocardiography; D005260:Female; D006350:Heart Valve Prosthesis; D006801:Humans; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D008875:Middle Aged; D012216:Rheumatic Diseases / Q000150:complications*
chemical_list: D003520:Cyclophosphamide; D008775:Methylprednisolone