Relapsing polychondritis with aortic involvement

Abstract

A 35-year-old female with relapsing polychondritis and aortic insufficiency is presented. Cases previously reported are reviewed. Aortic insufficiency appears to be fairly common in cases of relapsing polychondritis.Very little attention had been directed to relapsing polychondritis until 1958, when Dr. William Bean reviewed 8 cases from the world's literature and reported 1 of his own (1). I n this disorder, cartilage inflammation is the predominating feature, but there are an increasing number of reports with aortic involvement (2-4). The case reported below is that of a young female with a classical case of relapsing polychondritis who subsequently developed aortic insufficiency 20 months after the onset of her symptoms. REPORT OF CASEJT, a 35-year-old Caucasian female, was originally seen on Apr 17, 1968, for an evaluation of relapsing polychondritis which had been diagnosed previously. The patient had been in good health until Jan 1967, when severe episcleritis and anterior a s t ochondral pain developed requiring systemic corticosteroids for relief. Pain in the regioii of the thyroid cartilage developed in May 1967, and later, in Oct, nose and left ear pain and swelling ensued. These symptoms were associated with fever and pain in the cervical and thoracic spine. Prednisotie therapy was instituted with benefit, but 20 mg daily was required continuously for relief of symptoms. A brother, age 37, is reported to have ankylosing spondyli tis.Initial examination revealed a slightly obese female with a mild cushingoid appearance who was afebrile. The blood pressure was 110/70. Pertinent and significant findings revealed a left ear which was deformed and floppy, a saddle deformity of the nose (Fig. 1), and a very mild episcleritis. The remainder of the examination, including careful cardiac examination, was negative.Laboratory studies revealed an erythrocyte sedimentation rate of 25 mm/hr (Wintrobe-corrected) .The white blood count was 18,2OO/cu mm with a normal differential. Normal or negative values weIe noted for the following: hemoglobin, urinalysis, VDRL, LE cell preparation, serum uric acid, and rheumatoid factor (latex flocculation and sensitized human cell tests).One week after the initial consultation, severe left episcleritis developed. Prednisone was increased to 60 mg/day, and a trial of indomethacin. was instituted. T h e episcleritis, which was very difficult to control initially, was much improved 6 weeks later. The dose of prednisone was subsequently reduced to 15 mg/day, and indomethacin 150 mg/day was continued. The patient complained of mild dyspnea on exertion on Sept 4, 1968. At that time, it was noted that her pulse was 96/min and regular, and her blood pressure 115/55-0. A grade II/VI highpitched basilar decrescendo diastolic murmur was noted which radiated along the left sternal border, and a grade 1I/VI harsh ejection systolic murmur which transmitted faintly to the neck vessels was also heard. Neither of these murmurs had been *Indocin@, Merck Sharp Rc Dohme, West Point, Pa.

Report of Two Cases With an Immunopathological Review

“…Ocular involvement presenting as episcleritis, keratitis and iritis may be severe and destructive (Hughes et al, 1972). Involvement of heart valves may cause serious complications (Owen, Irbyr & Toone, 1970), such as aortic regurgitation, which may be associated with aortic aneurysm (Hughes et al,There are no specific laboratory findings but there is an increased incidence of rheumatoid factor, positive treponemal serology, raised ASO titre and of LE cells. Liver function tests have been abnormal in some cases (Dolan et al, 1966) and at autopsy, fatty changes with congestion were noted in the liver.…”

Relapsing Polychondritis with Glomerulonephritis and Severe Aortic Insufficiency Surgically Treated with Success

“…Th e po st op erat ive course was complicated by low-output syndrome which respond ed weil to inotrop ic support , digita lis and diuretic s. 18,19,20) . However, a review of the literature discloses only 12 cases of RP with AR, from data given by Eisdale and co-workers (6) and an additional case of Soh!…”

Drugs and Uric Acid

“…Tophaceous deposits tend to occur in various tissues in patients with long maintained hyperuricemia, and joint destruction or nephropathy, or both, develop insidiously over the course of years (78,172). In unusual instances the disease may mimic rheumatoid arthritis (45,69,165) ; it may be associated with diabetes mellitus (17,223), cystinuria (149), arterial hy pertension (24,28) ; or it may produce necrosis of the head of the femurs (93,140), sacroiliac arthritis (146), or carpal tunnel nerve compression (164). Aminoaciduria (101) and impaired excretion of bromsulphthalein dye (188) are interesting additional anomalies in patients with gout.…”