Relapsing polychondritis

Abstract

PURPOSE: This article describes a clinically-diagnosed case of relapsing polychondritis (RP), attended at the Hospital São Paulo, and presents a literature review of the subject. SOURCE OF RESEARCH: The literature review was made via Medline (1990-96), Lilacs (1980-96), textbooks of rheumatology, and some articles about the history of the disease. In Medline, 113 articles from 1990 to 1996 were found, and there were 23 articles from 1980 to 1996 in Lilacs. RESEARCH PROCEDURE: We reviewed the articles available at BIREME (Biblioteca Regional de Medicina) with the primary focus being on the disease in question. SUMMARY: RP is a rare disease of unknown etiology described initially by Jackson-Wartenhorst in 1923 and characterized by a recurrent and acute inflammatory process that causes the collapse of the cartilaginous structures and their subsequent replacement by fibrous connective tissue. The cartilage most commonly attacked is that of the auricle of the ear and nasal septum, while the cartilage of the trachea, larynx, epiglottis, ribs, and articulations may also be involved. Ocular inflammations and systemic reactions with fever are also described. In 1976, McAdam presented a complete prospective study of 23 patients, reviewed the 136 cases described up until that time, and then proposed diagnostic criteria which were later expanded by Damiani and Levine. Currently, more than 500 cases have been described. CONCLUSION: Although a rare disease, better knowledge of it is needed, as RP may be lethal with tracheal collapse and obstruction of respiratory pathways, making precise diagnosis and adequate therapeutic intervention necessary.

Feline Relapsing Polychondritis: Two Cases and a Review of the Literature

“…Over 30% of human patients with RPC suffer from an existing autoimmune or hematologic disease (Luthra, 1998). Systemic lupus erythematosus (SLE) is associated with 1.25% of RPC cases in humans (Miyaska 1998). This association in human medicine may have been the reason why several cats were tested for antinuclear antibodies (ANA).…”

A case of relapsing polychondritis mimicking Ludwig’s angina

“…9 There are no specific diagnostic tests but markers for inflammation; for example, increased leukocyte count, raised CRP or ESR, an ECG and echocardiogram may show large vessel vasculitis, while MRI and CT scans may show cartilaginous involvement. 10 The histology of involved cartilage demonstrates chondrolysis, chondritis, and perichondritis. The cartilage loses its basophilia, probably by the release of sulfated proteoglycans from the matrix, and the chondrocytes are decreased in number and may appear pyknotic.…”

Relapsing Polychondritis: Systemic and Ocular Manifestations, Differential Diagnosis, Management, and Prognosis

“…A clinical response should be seen within a few months, and treatment duration can last a few months to more than several years. 5,23,42 There have been limited reports documenting the efficacy of mycophenolate mofetil in RP. In a study by Thorne, mycophenolate mofetil is documented to be an effective corticosteroid-sparing agent in treating inflammatory eye disease such as uveitis, scleritis, and orbital inflammatory disease.…”