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Large vessel arteritis in relapsing polychondritis.

A healthy 58-year-old woman presented with recurrent swelling and pain of the nose and both auricules. Bruits were heard over both carotid arteries. Magnetic resonance angiography revealed stenosis of both internal carotid arteries. Relapsing polychondritis was diagnosed. These symptoms improved after treatment with prednisolone and azathioprine. Although relapsing polychondritis is sometimes associated with systemic vasculitis, large vessel arteritis is rare and can negatively affect prognosis. We conclude that the detection of systemic vascular lesions, including those involving the central nervous system, can play an important role in the diagnosis of relapsing polychondritis and that early treatment is essential for a good outcome.

Arteritis↗

Colchicine and indomethacin for the treatment of relapsing polychondritis.

This report describes the effectiveness of oral colchicine and indomethacin in the maintenance phase of treatment of relapsing polychondritis. Systemic corticosteroids are commonly used in the induction phase of managing cases of relapsing polychondritis. Clinicians are then faced with the challenge of controlling the patient's symptoms while trying to decrease the dose of steroid.

Anti-Inflammatory Agents↗

Obliterative microangiopathy presenting as chronic conjunctivitis in a patient with relapsing polychondritis.

PURPOSE: To report histopathologic findings and treatment of obliterative microangiopathy in a patient with chronic conjunctivitis due to relapsing polychondritis (RP). METHODS: This interventional case report describes a 50-year-old woman with relapsing polychondritis was referred to the Ocular Immunology and Uveitis Service for bilateral tearing due to refractory chronic conjunctivitis. Ocular examination revealed the presence of bilateral follicular conjunctivitis. Conjunctival biopsy of the inferior palpebral conjunctiva was performed, and the histopathologic findings guided the therapeutic intervention. RESULTS: The pathology of the conjunctival biopsy included granulomatous obliterative microangiopathy with numerous eosinophils, plasma cells, lymphocytes, and epithelioid cells in the substantia propia. The chronic conjunctivitis resolved with systemic methotrexate therapy. CONCLUSIONS: Microangiopathy may cause chronic conjunctivitis in patients with RP and may be a harbinger of evolving nonocular problems as a consequence of incomplete control of this autoimmune disorder. Immunomodulatory therapy should be considered in such cases to prevent possible cardiovascular, renal, respiratory, and neurologic complications of vasculitis. Although methotrexate has been used in treatment of RP-related necrotizing scleritis with poor results, it can be sufficient for the conjunctivitis with microangiopathy associated with RP.

Chronic Disease↗

Relapsing polychondritis: an ultrastructural study.

Ear cartilage from a typical case of relapsing polychondritis was examined with the electron microscope. A large number of dense granules and vesicles, which were compatible with matrix vesicles or lysosomes, surrounded the affected chondrocytes. In less severely damaged chondrocytes, these granules and vesicles appeared to be formed by pinching off of the cytoplasmic processes or by budding from the processes. Calcification of the granules was minimal. In severely damaged chondrocytes, an admixture of these granules and cytoplasmic organelles occurred. It is speculated that many of these dense granules are lysosomal in nature and that they may produce inflammation and reduce the proteoglycan content of cartilage.

Cartilage↗

Relapsing polychondritis in childhood: a rare observation studied by CT and MRI.

Relapsing polychondritis is very rare in children. The diagnosis must be based on a combination of clinical and pathologic features. CT is very useful for an accurate and rapid assessment of laryngo-tracheo-bronchial involvement and the typical finding is lumen narrowing by wall thickening and collapse of the supporting cartilaginous structures. The role of MR imaging should be complementary to CT.

Child↗

Articular involvement in patients with relapsing polychondritis.

Our objective was to study joint symptoms in patients with relapsing polychondritis (RP) and their relationship to other clinical manifestations and laboratory findings. Fourteen patients who met the diagnostic criteria proposed by Michet et al. for RP were studied. Clinical symptoms were recorded and a detailed laboratory analysis with HLA-DR typing was carried out. In 2 patients arthritis was the first manifestation. During the follow-up, 10 patients developed arthritis. It was polyarticular in 6, and oligoarticular in 4 cases. The development of arthritis was unrelated to the appearance of chondritis at other sites. HLA class II typing was determined in 7 patients. Six of them were positive for HLA-DR4. Arthritis in RP is a frequent manifestation occurring in approximately 70% of patients with an asymmetric articular involvement. There is no correlation between articular involvement and any particular clinical or laboratory feature. Susceptibility to RP is significantly related to the presence of HLA-DR4.

Adult↗

Mouth and genital ulcers with inflamed cartilage: MAGIC syndrome. Five patients with features of relapsing polychondritis and Behçet's disease.

Five patients with features of coexistent relapsing polychondritis and Behçet's disease are described. Review of the literature supports the overlap of the clinical manifestations of these two conditions. A common immunologic abnormality is likely, and elastin is cited as a possible target antigen. The "mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome" is the proposed name for this entity.

Adult↗

Relapsing polychondritis.

Inflammatory destruction of cartilages, presumably on an autoimmune basis, characterizes relapsing polychondritis, a disease of varying severity and outcome. The otolaryngologist-head and neck surgeon is often the first physician contact, because the preponderance of signs and symptoms are in the head and neck, ie, auricular and nasal chondritis, ocular and auditory manifestations, and laryngotracheobronchial involvement.

Humans↗

Head and neck manifestations of relapsing polychondritis: review of 29 cases.

This review of 29 patients with relapsing polychondritis seen at the Mayo Clinic between 1962 and 1976 emphasizes the head and neck manifestations of the disease and the role of the otolaryngologist in its diagnosis and treatment. The major clinical features included inflammation of the pinna, eye involvement, nasal cartilage involvement, laryngotracheal involvement, arthropathy, hearing loss, costal chondritis, and fever. The erythrocyte sedimentation rate was often elevated, and mild anemia was not uncommon.

Adolescent↗

Relapsing polychondritis.

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 550 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.

Adult↗

[The treatment of relapsing polychondritis using sulfones. One case (author's transl)].

A case of relapsing polychondritis was successfully treated with a daily dose of 120 mg of diaminodiphenylsulfone. Clinical signs (fever, fatigue, conjunctivitis, chondritis and hoarseness of the voice) rapidly regressed and remain so 8 months later, despite a decrease in the dose. Biological parameters returned to normal (sedimentation rate, plasma perchlorosoluble seromucoids, urinary acid mucopolysaccharides), and histological and ultrastructural lesions of the auricular cartilage disappeared. The pathogenic role of a herpes infection at the beginning of the disease is discussed.

Aged↗

Involvement of an enchondroma in relapsing polychondritis.

We describe a 49-year-old Caucasian woman with relapsing polychondritis involving ears, eyes, nose, and airways. An enchondroma in the left humerus was also involved. This unusual manifestation of the disease has not been previously reported.

Biopsy↗