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Relapsing Polychondritis

By

Alana M. Nevares

, MD, The University of Vermont Medical Center

Last full review/revision Feb 2020| Content last modified Feb 2020
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Relapsing polychondritis is a rare, episodic, inflammatory, and destructive disorder involving primarily cartilage of the ear and nose but also potentially affecting the eyes, tracheobronchial tree, heart valves, kidneys, joints, skin, and blood vessels. Diagnosis is by a combination of clinical, laboratory, imaging, and rarely biopsy findings. Treatment usually requires prednisone and other immunosuppressants.

Relapsing polychondritis affects men and women equally; onset typically is in middle age. An association with rheumatoid arthritis, systemic vasculitis, systemic lupus erythematosus (SLE), and other connective tissue disorders suggests an autoimmune etiology.

Symptoms and Signs

Acute pain, erythema, and swelling most commonly affect the pinna cartilage. Nasal cartilage inflammation is the next most common manifestation, followed by arthritis that varies from arthralgias to symmetric or asymmetric nondeforming arthritis involving large and small joints, with a predilection for the costochondral joints and knees.

The next most common manifestations, in decreasing order of frequency, are inflammation of the

  • Eye (eg, conjunctivitis, scleritis, iritis, keratitis, chorioretinitis)

  • Cartilaginous tissue of the larynx, trachea, or bronchi (causing hoarseness, cough, and tenderness over the laryngeal cartilage)

  • Internal ear

  • Cardiovascular system (eg, aortic regurgitation, mitral regurgitation, pericarditis, myocarditis, aortic aneurysms, aortitis)

  • Kidney

  • Skin

Bouts of acute inflammation heal over weeks to months, with recurrences over several years.

Advanced disease can lead to destruction of supporting cartilage, causing floppy ears, saddle nose, pectus excavatum, and visual, auditory, and vestibular abnormalities. Tracheal narrowing can lead to dyspnea, pneumonia, or even tracheal collapse. Coexisting systemic vasculitis (leukocytoclastic vasculitis or polyarteritis nodosa), myelodysplastic syndrome, or cancer is possible.

Diagnosis

  • Clinical criteria

  • Sometimes biopsy

Diagnosis of relapsing polychondritis is established if the patient develops at least 3 of the following:

  • Bilateral chondritis of the external ears

  • Inflammatory polyarthritis

  • Nasal chondritis

  • Ocular inflammation

  • Respiratory tract chondritis

  • Auditory or vestibular dysfunction

Biopsy of involved cartilage, most often the pinna, is helpful if clinical diagnosis is not clear-cut but is rarely required.

Laboratory tests are not specific but are done when necessary to help exclude other disorders. Synovial fluid analysis reveals mild inflammatory changes that are nonspecific but help rule out an infectious process. Blood tests may show normocytic-normochromic anemia, leukocytosis, elevated erythrocyte sedimentation rate (ESR) or gamma-globulin levels, and occasionally positive rheumatoid factor, antinuclear antibodies (ANA), or, in up to 25% of patients, antineutrophil cytoplasmic antibodies (ANCA). Abnormal renal function may indicate an associated glomerulonephritis. A positive c-ANCA test (ANCA that are reactive mainly to proteinase-3) may suggest granulomatosis with polyangiitis, which can rarely cause chondritis.

The upper and lower airways should be evaluated, including complete spirometric testing and chest CT, when the diagnosis is made.

Prognosis

Mortality rates have decreased with newer therapies. Survival is now 94% after 8 years, with death typically resulting from collapse of laryngeal and tracheal structures or from cardiovascular complications such as large-vessel aneurysm, cardiac valvular insufficiency, or systemic vasculitis.

Treatment

  • Nonsteroidal anti-inflammatory drugs or dapsone for mild ear disease

  • Corticosteroids

  • Sometimes methotrexate or other immunosuppressants (eg, cyclosporine, cyclophosphamide, azathioprine, anti-tumor necrosis factor drugs)

Mild recurrent ear disease may respond to nonsteroidal anti-inflammatory drugs in anti-inflammatory doses, or oral dapsone (50 to 100 mg once a day). However, most patients are treated with oral prednisone 30 to 60 mg once a day, with tapering of the dose as soon as there is a clinical response. Some patients require chronic use. In such patients, oral methotrexate 7.5 to 20 mg once a week can reduce the requirement for corticosteroids. Very severe cases may require other immunosuppressants, such as cyclosporine, cyclophosphamide, anti-tumor necrosis factor drugs (eg, infliximab, etanercept), or azathioprine (see Rheumatoid Arthritis (RA) : Immunomodulatory, cytotoxic, and immunosuppressive drugs). None of these therapies has been tested in controlled trials or has been shown to decrease mortality. If tracheal narrowing causes stridor, a tracheostomy or stent may be needed.

More extensive tracheobronchial collapse may require tracheal reconstruction. Eye disease may sometimes be recalcitrant to treatment, especially when involving the sclera, and has a poor prognosis. Patients on long-term corticosteroid therapy should receive osteoporosis prophylaxis. Prophylaxis for opportunistic infections, such as Pneumocystis jirovecii (see prevention of Pneumocystis jirovecii pneumonia), should be added if combination immunosuppressive therapy is used.

Endotracheal intubation can be technically difficult because of tracheal involvement and narrowing; also, intratracheal manipulation can lead to life-threatening postanesthetic deterioration by causing further glottal or subglottal inflammation. Thus, endotracheal intubation should be avoided whenever possible (eg, instead using local and regional anesthesia). When endotracheal intubation is unavoidable, preparations should be made for emergency cricothyrotomy.

Key Points

  • Consider relapsing polychondritis if patients develop inflammation of the pinna or nasal cartilage, particularly with symptoms and signs compatible with respiratory tract chondritis or unexplained arthritis, ocular inflammation, or auditory or vestibular dysfunction.

  • Biopsy the affected cartilage only if necessary to confirm the diagnosis.

  • Treat mild ear disease with nonsteroidal anti-inflammatory drugs or dapsone.

  • Treat more severe disease with corticosteroids and sometimes methotrexate or other immunosuppressants.

  • Avoid endotracheal intubation or, if it is unavoidable, prepare for emergency cricothyrotomy.

Drugs Mentioned In This Article

Drug Name Select Trade
CYTOXAN (LYOPHILIZED)
OTREXUP
IMURAN
NEORAL, SANDIMMUNE
ENBREL
REMICADE
RAYOS
ACZONE
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