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Relapsing Polychondritis
What is Relapsing Polychondritis
Relapsing polychondritis is a rare disease of unknown etiology characterized by episodic attacks of inflammation of hyaline and elastic cartilaginous structures, including the ears, nose, laryngotracheal, and articular cartilages as well as the organs of special sense. It is thought to be an autoimmune disease based on these cardinal features, the observed association with other conditions such as vasculitis, the presence of cartilage inflammation on biopsy, anticollagen antibodies, and a therapeutic response to corticosteroid therapy.
HISTORY AND DIAGNOSTIC CRITERIA of Relapsing Polychondritis
Relapsing polychondritis was first described in 1923 by Jaksch-Wartenhorst as polychondropathia in a young man with fever, inflammatory arthritis, and nasal and auricular chondritis with hearing loss. Subsequent cases were reported and termed systemic chondromalacia or chronic chondromalacia . Carl Pearson suggested the diagnostic term relapsing polychondritis in 1960, highlighting the episodic nature of the syndrome. He emphasized the associated inflammatory manifestations occurring in noncartilaginous structures such as the middle and inner ear, sclera, and uveal tract as well as the association of relapsing polychondritis with other rheumatic diseases.
EPIDEMIOLOGY, DISEASE ASSOCIATIONS, AND PROGNOSIS of Relapsing Polychondritis
Polychondritis is rare, and the annual incidence is uncertain. An estimate from the predominately Caucasian population of Rochester, Minnesota revealed an incidence of 3.5 cases per million. Cases are observed equally between the sexes and in all racial groups. Relapsing polychondritis occurs at all ages but is most frequently diagnosed in persons between 40 and 50 years of age.
Relapsing Polychondritis Disease manifestations may vary based on the age and sex of the patient. Nasal and subglottic or laryngotracheal chondritis are more common among younger patients, especially females. About 40% of cases are associated with another rheumatic, inflammatory, or hematologic disorder.
Systemic vasculitis is the most commonly associated inflammatory syndrome, followed by rheumatoid arthritis (RA), the spondyloarthropathies, and connective tissue disease disorders. Nonrheumatologic disease associations have included myelodysplastic syndromes and inflammatory bowel diseases.
Conditions associated with polychondritis
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| Systemic vasculitis syndromes |
| Rheumatoid arthritis |
| Systemic lupus erythematosus |
| Sjögren's syndrome |
| Ankylosing spondylitis |
| Psoriasis and psoriatic arthritis |
| Reiter's syndrome |
| Inflammatory bowel disease |
| Behçet's syndrome |
| Myelodysplastic syndrome |
| Dermatitis herpetiformis |
| Hodgkin's disease |
| Pannicullitis |
| Primary biliary cirrhosis |
| Retroperitoneal fibrosis |
| Thymoma |
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Clinical manifestations of relapsing polychondritis
Relapsing polychondritis presents in varied patterns but with usually one or two areas of cartilaginous inflammation.
The herald sign of relapsing polychondritis is auricular chondritis, a manifestation of the illness in more than 85% of patients. Unilateral, or bilateral in a simultaneous or sequential pattern, inflammation occurs in remitting and exacerbating attacks lasting for days to weeks. Characteristic findings include violaceous or erythematous tender swelling of the cartilaginous pinna with sparing of the soft lobule. Rarely, only the tragal and conchal cartilage is effected. When the external auditory canal is involved, permanent narrowing occurs. In patients who have suffered severe episodes, the ear may be left in a softened cauliflower deformity that may harden over time because of ossification of the damaged tissues.
LABORATORY INVESTIGATIONS for relapsing polychondritis
There are no pathognomonic laboratory abnormalities in relapsing polychondritis. During the acute phase of the illness, the erythrocyte sedimentation rate and C-reactive protein are usually elevated. Thrombocytosis, mild leukocytosis, anemia, and hypergammaglobulinemia related to inflammation are also observed. A macrocytic anemia may be the first sign that there is an associated evolving myelodysplastic syndrome. Urinalysis should be obtained in all cases to screen for an accompanying glomerulonephritis.
Autoantibodies, including antinuclear antibodies and rheumatoid factor, are found in cases of relapsing polychondritis associated with connective tissue disease syndromes or RA.
Treatment of relapsing polychondritis
The treatment of relapsing polychondritis is based entirely on empiric clinical observations. Because of the rarity of the disease, no clinical trials have been performed. The mainstay of therapy is oral corticosteroids, which have a generally reliable effect on the acute manifestations of chondritis. Initial doses of prednisone range from 0.5 to 1.0 mg/kg depending on the specific problems at presentation. Mild to moderate auricular and nasal chondritis may respond to lower doses, whereas scleritis, audiovestibular nerve involvement, laryngotracheal chondritis, and aortitis merit initial high-dose treatment. Nonsteroidal antiinflammatory drugs (NSAIDs) can be used to manage mild auricular, nasal, and costochondral chondritis, or synovitis. Case reports have suggested the utility of dapsone (100 mg twice daily) or colchicine (0.6 mg twice daily) as alternatives to corticosteroids for managing auricular or nasal chondritions
). If tolerated, an effective drug response should be seen within 1 to 2 weeks; hence, longer trials of these agents are probably not warranted.
When relapsing polychondritis is associated with a systemic vasculitis, treatment should be tailored to the underlying arteritis.
Chronic care of the patient with relapsing polychondritis requires periodic reassessment of the cardiovascular, pulmonary, and renal systems.
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