Polychondritis Treatment & Management

  • Author: Nicholas Compton, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Jan 19, 2012
 

Medical Care

No controlled trials of therapy for relapsing polychondritis (RP) have been published. The goal of treatment is to abate current symptoms and to preserve the integrity of cartilaginous structures.

  • The mainstay of treatment is systemic corticosteroid therapy. Prednisone (20-60 mg/d) is administered in the acute phase and is tapered to 5-25 mg/d for maintenance. Severe flares may require 80-100 mg/d. Most patients require a low daily dose of prednisone for maintenance; however, intermittent administration of high doses during only flares of the condition is successful in rare cases. McAdam et al found that continuous prednisone decreased the severity, frequency, and duration of relapses. See the images below. Same patient as in Image 5 after 4-6 weeks of sterSame 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. ForwaClose-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.
  • Other medications reported to control symptoms and, perhaps, progression of the disease, include dapsone (25-200 mg/d), azathioprine, methotrexate (MTX; 7.5-22.5 mg/wk), cyclophosphamide, and cyclosporin A. MTX has been administered beginning at 7.5 mg/wk, increasing up to 22.5 mg/wk in conjunction with steroid administration and has been found to significantly decrease corticosteroid requirements while controlling symptoms.
  • Biologics: Case reports have described successful treatment with anti–tumor necrosis factor-alpha inhibitors infliximab, etanercept, and adalimumab. Anakinra, an interleukin 1 receptor antagonist; leflunomide, which inhibits pyrimidine synthesis; and rituximab, an anti-CD20 chimeric antibody, have also shown benefit.[36, 37, 38, 39, 40]
  • Oral administration of nonsteroidal anti-inflammatory drugs (NSAIDs) has not been effective.
  • Medical care must include assessment for and treatment of other confounding or concurrent autoimmune disorders.
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Surgical Care

Surgeries encountered in the care of patients with relapsing polychondritis may include tracheostomy, permanent tracheotomy placement, tracheal stent placement, aortic aneurysm repair, cardiac valve replacement, and saddle-nose deformity repair. The benefits of any proposed surgery must be weighed adequately against the patient's risk for infection, especially in the event of acute relapse, since patients are at an increased risk of infection whether or not they are using corticosteroids.

Additionally, patients with relapsing polychondritis and tracheal disease may be at particular risk regarding complications resulting from tracheal intubation and extubation.

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Consultations

Relapsing polychondritis is a complex condition that requires a team approach for patient care.

  • Dermatologists or specialists in infectious diseases are often involved early in the course of the disease to evaluate the patient for infectious causes of cellulitis or perichondritis.
  • Rheumatologists usually become the primary care provider and should be involved early in patient care.
  • Ophthalmologists should also be involved early to diagnose, monitor, and treat the potentially devastating ocular complications.
  • Cardiologists, neurologists, nephrologists, and otolaryngologists may be asked to manage other aspects of relapsing polychondritis.
  • Plastic surgeons can aid in nasal reconstruction if saddle-nose deformity is present.
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Diet

No special recommendations have been noted.

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Activity

No special recommendations have been noted.

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Contributor Information and Disclosures
Author

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, and Medical Dermatology Society

Disclosure: Nothing to disclose.

Coauthor(s)

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, American College of Rheumatology, Phi Beta Kappa, and Sigma Xi

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.

Specialty Editor Board

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, and American Osteopathic Association

Disclosure: Nothing to disclose.

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

Disclosure: Medscape Salary Employment

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, and American College of Rheumatology

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; 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, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

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  109. Zion VM, Brackup AH, Weingeist S. Relapsing polychondritis, erythema nodosum and sclerouveitis. A case report with anterior segment angiography. Surv Ophthalmol. Sep-Oct 1974;19(2):107-14. [Medline].

 
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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
DiseasePatients With Condition/Total PatientsReferences
Systemic vasculitis3 (5%) of 62Zeuner et al[15]
11 (10%) of 112Michet et al[16]
8 (12%) of 66Trentham and Le[17]
28 (18%) of 159McAdam et al[14]
50 (13%) of 399Total
Cutaneous leukocytoclastic vasculitis2 (33%) of 6Priori et al[18]
6 (5%) of 112Michet et al[16]
8 (7%) of 118Total
Thyroid disease8 (5%) of 159McAdam et al[14]
10 (15%) of 66Trentham and Le[17]
2 (33%) of 6Priori et al[18]
4 (4%) of 112Michet et al[16]
2 (3%) of 62Zeuner et al[15]
26 (6%) of 405Total
Rheumatoid arthritis*8 (5%) of 159McAdam et al[14]
3 (2%) of 180Piette et al[19]
8 (7%) of 112Michet et al[16]
7 (11%) of 62Zeuner et al[15]
26 (5%) of 513Total
Systemic lupus erythematosus†2 (1%) of 159McAdam et al[14]
9 (5%) of 180Piette et al[19]
1 (17%) of 6Priori et al[18]
6 (5%) of 112Michet et al[16]
3 (5%) of 62Zeuner et al[15]
21 (4%) of 519Total
Sjögren syndrome (possible)5 (3%) of 159McAdam et al[14]
5 (5%) of 111Piette et al[19]
10 (4%) of 270Total
Ulcerative colitis3 (2%) of 159McAdam et al[14]
2 (3%) of 62Zeuner et al[15]
5 (2%) of 221Total
Crohn disease2 (1%) of 180Piette et al[19]
1 (2%) 62Zeuner et al[15]
1 (100%) of 1Haigh et al[20]
4 (2%) of 243Total
Mixed connective-tissue disease5 (3%) of 180Piette et al[19]
2 (2%) of 112Michet et al[16]
7 (2%) of 292Total
Takayasu arteritis3 (2%) of 180Piette et al[19]
Mesenteric panniculitis3 (2%) of 180Piette et al[19]
Spondyloarthropathy2 (1%) of 180Piette et al[19]
3 (3%) of 112Michet et al[16]
2 (3%) of 62Zeuner et al[15]
7 (2%) of 354Total
Diabetes mellitus1 (2%) of 62Zeuner et al[15]
3 (2%) of 159McAdam et al[14]
4 (2%) of 221Total
Reactive arthritis/psoriatic arthritis2 (1%) of 159McAdam et al[14]
1 (< 1%) of 112Michet et al[16]
3 (1%) of 271Total
Systemic sclerosis2 (1%) of 159McAdam et al[14]
Raynaud syndrome2 (1%) of 159McAdam et al[14]
Glomerulonephritis2 (1%) of 159McAdam et al[14]
Dysgammaglobulinemia2 (1%)of 159McAdam et al[14]
Pernicious anemia1 (1%) of 159McAdam et al[14]
Behçet disease*1 (< 1%) of 112Michet et al[16]
Psoriasis2 (1%) of 180Piette et al[19]
Lichen planus2 (1%) of 180Piette et al[19]
Primary biliary cirrhosis1 (< 1%) of 112Michet et al[16]
*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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