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Relapsing Polychondritis Medication

  • Author: Nicholas Compton, MD; Chief Editor: Herbert S Diamond, MD  more...
Updated: Jun 21, 2016

Medication Summary

Prednisone is the drug of choice for relapsing polychondritis (RP) and is used in acute flares and for long-term suppression of inflammation. Continuous treatment with prednisone decreases severity, duration, and frequency of relapses.

In patients who require higher maintenance doses of prednisone, methotrexate (MTX) is often administered as an adjuvant treatment. MTX is used with prednisone to reduce the overall steroid requirement for disease control; however, some patients may eventually be maintained with MTX alone. Dapsone has been beneficial in some patients with mild relapsing polychondritis, although more current clinical experience has found dapsone to be less useful.



Class Summary

These agents are the mainstay of therapy. They have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.

Prednisone (Deltasone, Orasone, Meticorten)


McAdam et al found that continuous use of prednisone decreased severity, frequency, and duration of relapses. Some patients may use reduced prednisone doses or remain steroid free with use of MTX.

For the acute phase, administer 20-60 mg/d and taper to 5-25 mg/d for maintenance. Severe flares may require 80-100 mg/d. Most patients require low daily dose for maintenance; however, rarely, some patients can be treated successfully by intermittent administration of high doses during flares of the condition. In acute airway obstruction, IV pulse steroids are necessary.


Disease-modifying antirheumatic agents

Class Summary

These agents inhibit cell growth and proliferation.

Methotrexate (Folex, Rheumatrex)


Unknown mechanism of action in treatment of inflammatory reactions; may affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness).

Effective steroid-sparing treatment for relapsing polychondritis. Adjust dose gradually to attain satisfactory response.

Anakinra (Kineret)


Recombinant interleukin 1 receptor antagonist expressed from Escherichia coli. Natural interleukin 1 receptor antagonist produced by macrophages/activated monocytes blocking effects of interleukin 1.


Anti-inflammatory agents

Class Summary

These agents possibly inhibit lysosomal enzyme activity, which in turn may reduce inflammation.

Dapsone (Avlosulfon)


Bactericidal and bacteriostatic against mycobacteria; mechanism of action is similar to that of sulfonamides in which competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth. Used in some patients in whom prednisone did not control symptoms. Successes and failures have been reported; therefore, prednisone remains the DOC.


Monoclonal antibodies - Antitumor necrosis factor-alpha inhibitors

Class Summary

These agents inhibit action of TNF-alpha, an inflammatory cytokine implicated for its contribution to rheumatic disease and cancer cachexia. Use described only in case reports.

Infliximab (Remicade)


Chimeric human-murine IgG1-kappa monoclonal antibody that binds to TNF-alpha. Binds both soluble and transmembrane forms and inhibits its binding to its receptors. Cells with transmembrane TNF-alpha bound to infliximab appear to be lysed with complement.

Etanercept (Enbrel)


Soluble, dimeric recombinant TNF receptor fused to the Fc fragment of human IgG1. This binds to TNF and inhibits its activities.

Adalimumab (HUMIRA)


Recombinant fully-human IgG1 anti-tumor necrosis factor monoclonal antibody. It binds to TNF-alpha and reduces it ability to effect its biological activities.


Anti-CD20 antigen on B lymphocytes

Class Summary

CD20 is a B-lymphocyte antigen that regulates cell cycle initiation. Use described in one case report.

Rituximab (Rituxan)


Murine/Human chimeric anti-CD20 monoclonal antibody. CD20 is expressed early in pre-B cell development. Binding induces complement-dependent B-cell cytotoxicity along with antibody-dependent cellular toxicity.


Interleukin-1 receptor antagonists

Class Summary

These agents have anti-inflammatory characteristics.

Leflunomide (Arava)


Isoxazole immunomodulatory agent with anti-inflammatory characteristics. Mechanism of action is through the inhibition of dihydroorotate dehydrogenase, which leads to a decrease in proliferative activity.

Although not entirely elucidated, it is thought to inhibit de novo pyrimidine synthesis. It inhibits proliferation of immune cells.

Contributor Information and Disclosures

Nicholas Compton, MD Staff Physician, Department of Medicine, Division of Dermatology, University of Washington Medical Center

Nicholas Compton, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, Medical Dermatology Society

Disclosure: Nothing to disclose.


Karin I Harp, MD Consulting Staff, Department of Dermatology, Everett Clinic

Karin I Harp, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Gregory J Raugi, MD, PhD Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle School of Medicine; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle

Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Jane H Buckner, MD Member, Director of Translation Research, Benaroya Research Institute; Clinical Associate Professor, Division of Rheumatology, University of Washington School of Medicine

Jane H Buckner, MD is a member of the following medical societies: American College of Physicians, Phi Beta Kappa, Sigma Xi, American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Bryan L Martin, DO Associate Dean for Graduate Medical Education, Designated Institutional Official, Associate Medical Director, Director, Allergy Immunology Program, Professor of Medicine and Pediatrics, Ohio State University College of Medicine

Bryan L Martin, DO is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Osteopathic Internists, American College of Physicians, American Medical Association, American Osteopathic Association

Disclosure: Nothing to disclose.

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Auricular edema and erythema sparing the lobule. Courtesy of Gregory J. Raugi, MD, PhD.
Severe auricular edema and inflammation. Courtesy of the University of Washington, Division of Dermatology.
Forward listing ear. Courtesy of the University of Washington, Division of Dermatology.
Floppy ear. Courtesy of the University of Washington, Division of Dermatology.
Bilateral inflammation and structural collapse of the auricles in a patient found to have aortic dissection. Courtesy of the University of Washington, Division of Dermatology.
Same patient as in Image 5 after 4-6 weeks of steroid treatment. Note resolution of auricular inflammation with nodularity and forward listing of the ears. Courtesy of the University of Washington, Division of Dermatology.
Close-up view of same patient as in Image 6. Forward flopping of ear with nodularity after steroid treatment. Courtesy of the University of Washington, Division of Dermatology.
Unilateral episcleritis. Courtesy of Gregory J. Raugi, MD, PhD.
Saddle-nose deformity. Courtesy of the University of Washington, Division of Dermatology.
Tracheal stenosis on chest x-ray film. Courtesy of Julie E. Takasugi, MD.
Table. Autoimmune Conditions Reported in Patients With Relapsing Polychondritis
Disease Patients With Condition/Total Patients References
Systemic vasculitis 3 (5%) of 62 Zeuner et al[17]
11 (10%) of 112 Michet et al[18]
8 (12%) of 66 Trentham and Le[19]
28 (18%) of 159 McAdam et al[16]
50 (13%) of 399 Total
Cutaneous leukocytoclastic vasculitis 2 (33%) of 6 Priori et al[20]
6 (5%) of 112 Michet et al[18]
8 (7%) of 118 Total
Thyroid disease 8 (5%) of 159 McAdam et al[16]
10 (15%) of 66 Trentham and Le[19]
2 (33%) of 6 Priori et al[20]
4 (4%) of 112 Michet et al[18]
2 (3%) of 62 Zeuner et al[17]
26 (6%) of 405 Total
Rheumatoid arthritis* 8 (5%) of 159 McAdam et al[16]
3 (2%) of 180 Piette et al[21]
8 (7%) of 112 Michet et al[18]
7 (11%) of 62 Zeuner et al[17]
26 (5%) of 513 Total
Systemic lupus erythematosus† 2 (1%) of 159 McAdam et al[16]
9 (5%) of 180 Piette et al[21]
1 (17%) of 6 Priori et al[20]
6 (5%) of 112 Michet et al[18]
3 (5%) of 62 Zeuner et al[17]
21 (4%) of 519 Total
Sjögren syndrome (possible) 5 (3%) of 159 McAdam et al[16]
5 (5%) of 111 Piette et al[21]
10 (4%) of 270 Total
Ulcerative colitis 3 (2%) of 159 McAdam et al[16]
2 (3%) of 62 Zeuner et al[17]
5 (2%) of 221 Total
Crohn disease 2 (1%) of 180 Piette et al[21]
1 (2%) 62 Zeuner et al[17]
1 (100%) of 1 Haigh et al[22]
4 (2%) of 243 Total
Mixed connective-tissue disease 5 (3%) of 180 Piette et al[21]
2 (2%) of 112 Michet et al[18]
7 (2%) of 292 Total
Takayasu arteritis 3 (2%) of 180 Piette et al[21]
Mesenteric panniculitis 3 (2%) of 180 Piette et al[21]
Spondyloarthropathy 2 (1%) of 180 Piette et al[21]
3 (3%) of 112 Michet et al[18]
2 (3%) of 62 Zeuner et al[17]
7 (2%) of 354 Total
Diabetes mellitus 1 (2%) of 62 Zeuner et al[17]
3 (2%) of 159 McAdam et al[16]
4 (2%) of 221 Total
Reactive arthritis/psoriatic arthritis 2 (1%) of 159 McAdam et al[16]
1 (< 1%) of 112 Michet et al[18]
3 (1%) of 271 Total
Systemic sclerosis 2 (1%) of 159 McAdam et al[16]
Raynaud syndrome 2 (1%) of 159 McAdam et al[16]
Glomerulonephritis 2 (1%) of 159 McAdam et al[16]
Dysgammaglobulinemia 2 (1%)of 159 McAdam et al[16]
Pernicious anemia 1 (1%) of 159 McAdam et al[16]
Behçet disease* 1 (< 1%) of 112 Michet et al[18]
Psoriasis 2 (1%) of 180 Piette et al[21]
Lichen planus 2 (1%) of 180 Piette et al[21]
Primary biliary cirrhosis 1 (< 1%) of 112 Michet et al[18]
*Individual patients may carry more than one autoimmune diagnosis.

†Reported as 13 (20%) of 66 prevalence by Trentham and Le without division by disease

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