Relapsing Polychondritis
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Contents |
Introduction
- Episodic & often progressive inflammatory disorder affecting the cartilage of the ear, nose & tracheobronchial tree.
Etiology
- idiopathic
- antibodies to type II collagen
- 30% have another connective tissue disease
Epidemiology
- most common between age 40-60, but may affect children or elderly
- relatively uncommon
- all races
Pathology
- depletion of proteoglycan from cartilage matrix
- inflammatory infiltrate of mononuclear cells & occasional plasma cells adjacent to involved cartilage
- neutrophils may be present in acute disease
- destruction of cartilage begins at outer edges & continues centrally
- lacunar breakdown & loss of chondrocytes
- degenerating cartilage is replaced by granulation tissue, & later by fibrosis & focal areas of calcification
- small focal areas of cartilage regeneration may be present
- immunoglobulin & complement detected by immunofluorescence at sites of involved cartilage
- loss of cartilage matrix hypothesized secondary to action of proteolytic enzymes released by chondrocytes & inflammatory cells activated by cytokines
Clinical-manifestations
- involvement of ears, nose, larynx, trachea, & joints
- auricular chondritis (40%/85%)
- hearing loss (10%/30%)
- nasal chondritis (25%/55%)
- saddle nose deformity (20%/30%)
- ocular deformity/disorder (20%/50%)
- conjunctivitis, episcleritis, scleritis, iritis, keratitis
- ulceration & perforation of the cornea
- eyelid & periorbital edema
- proptosis
- cataracts
- optic neuritis
- extraocular muscle palsies
- retinal vasculitis & retinal vein occlusion
- respiratory disease (25%/50%)
- mucosal edema & strictures
- expiratory collapse of major airways
- collapse of laryngeal, tracheal or bronchial cartilage (late manifestation)
- airway obstruction requiring emergency tracheostomy
- hoarseness, non-productive cough
- pneumonia
- 50% of deaths due to respiratory complications
- arthritis (35%/50%)
- asymmetric
- involves both large & small peripheral joints
- generally lasts a few days to several weeks & resolves spontaneously without deformity
- aortic regurgitation ( / 5%)
- vasculitis ( 3%/10%)
- other manifestations
- polyarthritis
- cardiac abnormalities
- aortic regurgitation (5%)
- pericarditis
- myocarditis
- conduction abnormalities
- glomerulonephritis may occur in the absence of systemic vasculitis
- relapsing course with exacerbations & remissions
- (presenting/cumlative frequencies)
Laboratory
-
- rheumatoid factor ( RF) & anti-nuclear antibody ( ANA) are often positive in low titers
- antibodies to type II collagen are positive in most patients, but non-specific
- circulating immune complexes may be detected
- ANCA ( cANCA or pANCA) are positive in some
- abnormal liver function tests ( LFTs)
- polyclonal gammapathy may be present
- biopsy of cartilaginous portion of ear
Diagnostic-procedures
- bronchoscopy if indicated
Radiology
Complications
- myelodysplastic syndrome has been reported in several patients with relapsing polychondritis
- ulceration & perforation of the cornea
- pericarditis & myocarditis
Management
-
- 40-60 mg PO QD for active chondritis or vasculitis
- taper after disease is controlled
- in some patients prednisone may be stopped
- 10-15 mg PO QD may be required to maintain remission
- NSAIDs for chronic disease management
- immunosuppressive agents
- indicated for patients who fail prednisone therapy
- azathiaprine
- cyclophosphamide
- cyclosporine or dapsone may benefit some patients
- rituximab of little benefit
- intraocular steroids plus systemic glucocorticoids for significant ocular inflammation
More General Terms
Additional Terms
References
- Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 1951-53
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 173, 789
- Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15 American College of Physicians, Philadelphia 1998, 2006, 2009
- Leroux G et al Treatment of relapsing polychondritis with rituximab: A retrospective study of nine patients. Arthritis Rheum 2009 May 15; 61:577 PMID: [1]
