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Relapsing Polychondritis Follow-up

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

Prognosis

In earlier studies, the 5-year survival rate associated with relapsing polychondritis was reported to be 66%-74% (45% if relapsing polychondritis occurs with systemic vasculitis), with a 10-year survival rate of 55%. More recently, Trentham and Le found a survival rate of 94% at 8 years.[19] However, those data may represent relapsing polychondritis in patients with less severe disease than patients studied in earlier reports.

The most common causes of relapsing polychondritis–related death include infection secondary to corticosteroid treatment or respiratory compromise (10-50% of deaths result from airway complications), systemic vasculitis, and malignancy unrelated to relapsing polychondritis.

Complications of relapsing polychondritis such as saddle-nose deformity, systemic vasculitis, laryngotracheobronchial stricture, arthritis, and anemia in patients younger than 51 years portend a poorer prognosis than in age-matched patients with relapsing polychondritis without complications. Among patients older than 51 years, only anemia is associated with a poorer prognosis. Renal involvement is a poor prognostic factor at all ages.

 
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, Medical Dermatology Society

Disclosure: Nothing to disclose.

Coauthor(s)

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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