Relapsing Polychondritis Medication

Updated: Sep 25, 2017
  • Author: Nicholas Compton, MD; Chief Editor: Herbert S Diamond, MD  more...
  • Print
Medication

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.

Next:

Corticosteroids

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.

Previous
Next:

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.

Previous
Next:

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.

Previous
Next:

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.

Previous
Next:

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.

Previous
Next:

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.

Previous