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relapsing polychondritis

Episodic & often progressive inflammatory disorder affecting the cartilage of the ear, nose & tracheobronchial tree. Etiology: 1) idiopathic 2) antibodies to type II collagen 3) 30% have another connective tissue disease a) systemic vasculitis (most common) 1] leukocytoclastic vasculitis 2] polyarteritis nodosa 3] temporal arteritis 4] Takayasu's arteritis b) rheumatoid arthritis c) systemic lupus erythematosus d) Sjogren's syndrome Epidemiology: 1) most common between age 40-60, but may affect children or elderly 2) relatively uncommon 3) all races Pathology: 1) inflammation & damage to cartilage 2) depletion of proteoglycan from cartilage matrix 3) inflammatory infiltrate of mononuclear cells & occasional plasma cells adjacent to involved cartilage 4) neutrophils may be present in acute disease 5) destruction of cartilage begins at outer edges & continues centrally 6) lacunar breakdown & loss of chondrocytes 7) degenerating cartilage is replaced by granulation tissue, & later by fibrosis & focal areas of calcification 8) small focal areas of cartilage regeneration may be present 9) immunoglobulin & complement detected by immunofluorescence at sites of involved cartilage 10) loss of cartilage matrix hypothesized secondary to action of proteolytic enzymes released by chondrocytes & inflammatory cells activated by cytokines Clinical manifestations: 1) involvement of ears, nose, larynx, trachea, & joints 2) auricular chondritis (40%/85%)* a) both ears generally involved b) sudden onset of pain, tenderness & swelling of cartilagenous portion of ear c) earlobes are spared because they do not contain cartilage d) overlying skin is red or violaceous e) recurrent episodes result in droopy ears 3) cochlear or vestibular dysfunction a) hearing loss (10%/30%)* b) tinnitus & or vertigo [3] 4) nasal chondritis (25%/55%)* a) nasal stuffiness b) rhinorrhea c) epistaxis 5) saddle nose deformity (20%/30%)* 6) ocular deformity/disorder (20%/50%)* a) conjunctivitis, episcleritis, scleritis, iritis, keratitis b) ulceration & perforation of the cornea c) eyelid & periorbital edema d) proptosis e) cataracts f) optic neuritis g) extraocular muscle palsies h) retinal vasculitis & retinal vein occlusion 7) respiratory disease (25%/50%)* a) mucosal edema & strictures b) laryngeal chondritis or tracheal chondritis (50%) c) expiratory collapse of major airways 1] collapse of laryngeal, tracheal or bronchial cartilage (late manifestation) 2] airway obstruction requiring emergency tracheostomy d) hoarseness, non-productive cough e) pneumonia f) 50% of deaths due to respiratory complications 8) arthritis (35%/50%)* a) asymmetric b) involves both large & small peripheral joints c) generally lasts a few days to several weeks & resolves spontaneously without deformity 9) aortic regurgitation ( /5%)* 10) vasculitis (3%/10%)* a) seizures b) stroke c) peripheral neuropathies 11) other manifestations a) polyarthritis b) cardiac abnormalities 1] aortic regurgitation (5%) 2] pericarditis 3] myocarditis 4] conduction abnormalities c) skin lesions 1] erythema nodosum 2] erythema multiforme 3] urticaria & angioedema 4] purpura 5] livedo reticularis 6] panniculitis d) glomerulonephritis may occur in the absence of systemic vasculitis 12) 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: 1) complete blood count (CBC) a) mild leukocytosis b) normocytic, normochromic anemia 2) elevated erythrocyte sedimentation rate (ESR) 3) serology a) rheumatoid factor (RF) & anti-nuclear antibody (ANA) are often positive in low titers b) antibodies to type II collagen are positive in most patients, but non-specific c) circulating immune complexes may be detected d) ANCA (cANCA or pANCA) are positive in some 4) abnormal liver function tests (LFTs) 5) polyclonal gammopathy may be present 6) biopsy of cartilagenous portion of ear Special laboratory: - bronchoscopy if indicated - pulmonary function testing with flow volume loops [3] Radiology: 1) chest X-ray 2) computed tomography (CT) of thorax Differential diagnisis: - granulomatosis with polyangiitis (Wegener's granulomatosis) - pauci-immune glomerulonephritis - sarcoidosis - hilar lymphadenopathy - pinna inflammation uncommon - Behcet disease - recurrent oral ulcers & genital ulcers [11] Complications: 1) myelodysplastic syndrome has been reported in several patients with relapsing polychondritis 2) ulceration & perforation of the cornea 3) pericarditis & myocarditis 4) 50% of deaths due to respiratory complications Management: 1) prednisone a) 40-60 mg PO QD for active chondritis or vasculitis b) taper after disease is controlled c) in some patients prednisone may be stopped d) 10-15 mg PO QD may be required to maintain remission 2) NSAIDs for chronic disease management 3) immunosuppressive agents a) indicated for patients who fail prednisone therapy b) azathiaprine c) cyclophosphamide 4) cyclosporine or dapsone may benefit some patients 5) rituximab of little benefit 6) intraocular steroids plus systemic glucocorticoids for significant ocular inflammation

Related

collagen type-2 saddle nose deformity

General

musculoskeletal disease/disorder connective tissue disease; soft tissue disease

References

  1. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 1951-53
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 173, 789
  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.
  4. Leroux G et al Treatment of relapsing polychondritis with rituximab: A retrospective study of nine patients. Arthritis Rheum 2009 May 15; 61:577 PMID: 19405005
  5. Rafeq S, Trentham D, Ernst A. Pulmonary manifestations of relapsing polychondritis. Clin Chest Med. 2010 Sep;31(3):513-8 PMID: 20692543
  6. Kent PD, Michet CJ Jr, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol. 2004 Jan;16(1):56-61. PMID: 14673390
  7. Chopra R, Chaudhary N, Kay J. Relapsing polychondritis. Rheum Dis Clin North Am. 2013 May;39(2):263-76. PMID: 23597963
  8. 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: 775252
  9. Haslag-Minoff J, Regunath H. Relapsing Polychondritis. Images in Clinical Medicine. N Engl J Med 2018; 378:1715. May 3, 2018 PMID: 29719184 https://www.nejm.org/doi/full/10.1056/NEJMicm1713302
  10. 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: 26711694
  11. 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: 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: 26876384 Review.