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. A variety of other conventional immunosuppressants and biologic therapies are used as steroid-sparing agents or for combination therapy in severe cases.
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.
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.
Abatacept (Orencia, Orencia ClickJect)
Chimeric protein that inhibits T-lymphocyte activation.
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.
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Auricular edema and erythema sparing the lobule. Courtesy of Gregory J. Raugi, MD, PhD.
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Severe auricular edema and inflammation. Courtesy of the University of Washington, Division of Dermatology.
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Forward listing ear. Courtesy of the University of Washington, Division of Dermatology.
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Floppy ear. Courtesy of the University of Washington, Division of Dermatology.
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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.
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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.
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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.
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Unilateral episcleritis. Courtesy of Gregory J. Raugi, MD, PhD.
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Saddle-nose deformity. Courtesy of the University of Washington, Division of Dermatology.
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Tracheal stenosis on chest x-ray film. Courtesy of Julie E. Takasugi, MD.
Tables
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 |
|
3 (2%) of 159 |
McAdam et al [16] |
|
2 (3%) of 62 |
Zeuner et al [17] |
|
5 (2%) of 221 |
Total |
|
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 |
|
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 |
|
2 (1%) of 159 |
McAdam et al [16] |
|
1 (< 1%) of 112 |
Michet et al [18] |
|
3 (1%) of 271 |
Total |
|
2 (1%) of 159 |
McAdam et al [16] |
|
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] |
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] |
2 (1%) of 180 |
Piette et al [21] |
|
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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