relapsing polychondritis
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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
- inflammation & damage to cartilage
- 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%)*
- both ears generally involved
- sudden onset of pain, tenderness & swelling of cartilagenous portion of ear
- earlobes are spared because they do not contain cartilage
- overlying skin is red or violaceous
- recurrent episodes result in droopy ears
- cochlear or vestibular dysfunction
- hearing loss (10%/30%)*
- tinnitus & or vertigo[3]
- nasal chondritis (25%/55%)*
- saddle nose deformity (20%/30%)*
- ocular deformity/disorder (20%/50%)*
- respiratory disease (25%/50%)*
- mucosal edema & strictures
- laryngeal chondritis or tracheal chondritis (50%)
- 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%)*
- aortic regurgitation ( /5%)*
- vasculitis (3%/10%)*
- other manifestations
- polyarthritis
- cardiac abnormalities
- aortic regurgitation (5%)
- pericarditis
- myocarditis
- conduction abnormalities
- skin lesions
- glomerulonephritis may occur in the absence of systemic vasculitis
- relapsing course with exacerbations & remissions
* (presenting/cumlative frequencies)
# images[9]
Diagnostic criteria
- 3 of the following 6
- recurrent bilateral auricular chondritis (90%)
- non-erosive inflammatory polyarthritis (65%)
- nasal chondritis (60%)
- ocular inflammation: conjuctivitis, keratitis, scleritis, episcleritis, uveitis (55%)
- laryngeal chondritis or tracheal chondritis (50%)
- cochlear &/or vestibular dysfunction: tinnitus &/or vertigo, neurosensory hearing loss (10%)[3]
Laboratory
- complete blood count (CBC)
- elevated erythrocyte sedimentation rate (ESR)
- serology
- 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 gammopathy may be present
- biopsy of cartilagenous portion of ear
Diagnostic procedures
- bronchoscopy if indicated
- pulmonary function testing with flow volume loops[3]
Radiology
- chest X-ray
- computed tomography (CT) of thorax Differential diagnisis:
Complications
- myelodysplastic syndrome has been reported in several patients with relapsing polychondritis
- ulceration & perforation of the cornea
- pericarditis & myocarditis
- 50% of deaths due to respiratory complications
Management
- prednisone
- 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
- ↑ 3.0 3.1 3.2 3.3 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022.
- ↑ Leroux G et al Treatment of relapsing polychondritis with rituximab: A retrospective study of nine patients. Arthritis Rheum 2009 May 15; 61:577 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19405005
- ↑ Rafeq S, Trentham D, Ernst A. Pulmonary manifestations of relapsing polychondritis. Clin Chest Med. 2010 Sep;31(3):513-8 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20692543
- ↑ Kent PD, Michet CJ Jr, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol. 2004 Jan;16(1):56-61. PMID: https://www.ncbi.nlm.nih.gov/pubmed/14673390
- ↑ Chopra R, Chaudhary N, Kay J. Relapsing polychondritis. Rheum Dis Clin North Am. 2013 May;39(2):263-76. PMID: https://www.ncbi.nlm.nih.gov/pubmed/23597963
- ↑ McAdam LP, O'Hanlan MA, Bluestone R, Pearson CM. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine (Baltimore). 1976 May;55(3):193-215. PMID: https://www.ncbi.nlm.nih.gov/pubmed/775252
- ↑ 9.0 9.1 Haslag-Minoff J, Regunath H. Relapsing Polychondritis. Images in Clinical Medicine. N Engl J Med 2018; 378:1715. May 3, 2018 PMID: https://www.ncbi.nlm.nih.gov/pubmed/29719184 https://www.nejm.org/doi/full/10.1056/NEJMicm1713302
- ↑ Vitale A, Sota J, Rigante D et al Relapsing Polychondritis: an Update on Pathogenesis, Clinical Features, Diagnostic Tools, and Therapeutic Perspectives. Curr Rheumatol Rep. 2016 Jan;18(1):3. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26711694
- ↑ 11.0 11.1 NEJM Knowledge+
Kingdon J, Roscamp J, Sangle S, D'Cruz D. Relapsing polychondritis: a clinical review for rheumatologists. Rheumatology (Oxford). 2018 Sep 1;57(9):1525-1532. PMID: https://www.ncbi.nlm.nih.gov/pubmed/29126262 Review.
Longo L, Greco A, Rea A, Lo Vasco VR, De Virgilio A, De Vincentiis M. Relapsing polychondritis: A clinical update. Autoimmun Rev. 2016 Jun;15(6):539-43. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26876384 Review.