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A. Characteristics [4] navigator

  1. Systemic inflammatory disorder, mainly affecting cartilagenous tissues
  2. Immunological Origen
    1. P-ANCA+ in 30-40% cases
    2. HLA-DR4 increased proportion
  3. Associated with other autoimmune disease in ~30% of patients
    1. Systemic Lupus Erythematosus
    2. Sjogren Syndrome
    3. Rheumatoid Arthritis
    4. Systemic Vasculitis
    5. Spondyloarthropathies
    6. Myelodyspastic Syndromes (including post chemotherapy)
    7. Diabetes mellitus
    8. Other P-ANCA Associated Disease
  4. Very rare disorder
    1. Occurs at any age with peak in fifth decade
    2. Male = Female
    3. 5 year survival ~75%

B. Symptoms [4]navigator

  1. Delay from symptom initiation to diagnosis median ~3 years
  2. Inflammation of cartilage in nose, ears, trachea and joints
  3. External Ear
    1. ~40% of patients present with acute painful swelling and erythema of external ear
    2. >85% will eventually develop ear symptoms
    3. Eventually external ear cartilage is destroyed
    4. Results is soft and floppy, or hard and fibrotic, knobby ear
    5. Stenosis of external auditory canal may occur
    6. Hearing and/or vestibular problems occur in ~30% of patients
  4. Nasal Bridge
    1. Inflammation may lead to bridge collapse
    2. About 20% present with nasal symptoms
    3. Overall, ~60% of patients with RP will develop nasal bridge involvement
    4. Wegener's granulmatosis should be ruled out (clinically and serum ANCA test)
  5. Respiratory Symptoms
    1. Eventually develop in ~50% of patients
    2. Symptoms of tracheal stenosis
    3. Hoarseness, coughing, wheezing, dyspnea
    4. Stridor may occur
    5. Airway narrowing may become critical leading to full obstruction
    6. Increased incidence of lower respiratory tract infections
  6. Cardiovascular Disease
    1. Small, medium and large vessel vasculitis may occur
    2. Aortic root inflammation may leads to insufficiency
    3. Arrhythmias and heart block are uncommon
    4. Valve disease and pericarditis reported
    5. Cardiovascular function should be monitored in all patients
  7. Arthritis
    1. Seronegative arthritis occurs in ~50% of patients
    2. Oligo- or polyarthritis
    3. Usually non-erosive disease
    4. Affects small and large joints
  8. Ocular Disease
    1. Any portion of the eye may be affected
    2. Intraocular: iridocyclitis and retinal vasculitis
    3. Extraocular: conjunctivitis, keratitis, scleritis, episcleritis, soft tissue involvement
    4. About 50% of patients with relapsing polychondritis develop ocular symptoms
  9. Dermatologic Symptoms
    1. Leukocytoclastic vasculitis
    2. Erythema nodosum
    3. Panniculitis
    4. Urticaria
    5. Recurrent thrombophlebitis
  10. Neurologic Symptoms
    1. Headaches
    2. Cranial nerve palsies: CN III, IV, VI, VII, and/or VIII
    3. Optic neuritis
    4. Cerebellar signs
    5. Hemiplegia
    6. Seizures
    7. Encephalopathy
  11. Pregnancy does not appear to affect disease activity [6]

C. Pathology navigator

  1. Invasion of fibrocartilage with mononuclear cells leads to destruction
  2. Predominantly CD4+ T cell infiltrates
  3. May have high levels of anti-collagen Abs
  4. Collagen types IX and XI may be targets; Type II may also play a role
  5. Granular deposits of IgG, IgA, IgM and C3 have been found (probably immune complexes)

D. Diagnosis [1,5,7]navigator

  1. Diagnosis Requires One of the Following:
    1. Three clinical features
    2. One clinical feature and histologic evidence of chondritis
    3. Chondritis at >1 separate anatomic locations, with response to glucocorticoids or dapsone
  2. Clinical Features for Diagnosis:
    1. Bilateral auricular chondritis
    2. Inflammatory polyarthritis, nonerosive, seronegative
    3. Nasal chondritis
    4. Ocular Inflammation
    5. Respiratory tract chondritis
    6. Cochlear and/or vestibular dysfunction
  3. ESR often highly elevated

E. Differential Diagnosis of Inflammatory Processes of Ear (Table 2 in Ref [1])navigator

  1. Infection - otitis externa
  2. Trauma, especially with superinfection of ear (often Pseuomonas)
  3. "Cauliflower Ear"
    1. Also called perichondrial hematoma
    2. Blood clot or other fluid collection under the perichondrium
    3. Common in wrestlers, rugby players, boxers, martial arts
    4. External portion of ear suffers trauma leading to the hematoma
    5. Fluid separates cartilage from perichondrium, which supplies nutrients to cartilage
    6. Cartilage will die if conditions remains, and ear can resemble a cauliflower
    7. May become infected
  4. Insect bite
  5. Sunburn
  6. Frostbite
  7. Relapsing Polychondritis

F. Treatment [1] navigator

  1. No specific therapy
  2. Mild disease may respond to NSAIDs
  3. Glucocorticoids for moderate to severe disease
    1. Low doses of 7.5-12.5mg/d po prednisone may be effective
    2. Respiratory, renal disease, or vasculitis, require high doses
    3. Any serious disease should receive IV methylprednisolone 30-40mg IV bid
    4. Slow taper generally required in serious disease
    5. Conversion to oral prednisone 30-40mg qd with slow taper
  4. Glucocorticoid-Sparing Agents
    1. Mycophenolate mofetil (Celcept®) 500mg bid to 750mg bid is generally first line
    2. Methotrexate (MTX) has shown good glucocorticoid-sparing effects
    3. MTX is highly recommended in patients with systemic disease
    4. MTX dose is 15-20mg per week, usually given subcutaneously
    5. Cyclophosphamide or azathioprine may be used if other agents not effective
  5. Other Agents
    1. Some patients respond to dapsone or cyclosporine
    2. Minocycline may be considered
    3. Cochicine and indomethacin reported effective in maintenance phase [5]

References navigator

  1. Butterton JR, Collier DS, Romero JM, Zembowicz A. 2007. NEJM. 356(19):1980 (Case Record) abstract
  2. Letko E, Zafirakis P, Baltatzis S, et al. 2002. Semin Arthritis Rheum. 31(6):384 abstract
  3. Trentham DE and Le CH. 1998. Ann Intern Med. 129(2):114 abstract
  4. Zeuner M, Straub RH, Rauh G, et al. 1997. J Rheumatol. 24(1):96 abstract
  5. Mark KA and Franks AG Jr. 2002. J Am Acad Dermatol. 46:S22 abstract
  6. Papo T, Wechsler B, Bletry O, et al. 1997. Arthritis Rheum. 40(7):1245 abstract
  7. Braman SS, Grillo HC, Mark EJ. 1999. NEJM. 341(17):1292 (Case Record)