Which autoimmune disorders are associated with relapsing polychondritis (RP)?

Answer

The hypothesis of an autoimmune etiology for relapsing polychondritis is also supported by the high prevalence of other autoimmune disorders found in patients with relapsing polychondritis. McAdam et al reported that 25%-35% of patients with relapsing polychondritis had a concurrent autoimmune disease.^[16]

Table. Autoimmune Conditions Reported in Patients With Relapsing Polychondritis (Open Table in a new window)

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

In addition, several reports have linked relapsing polychondritis with internal malignancy. It is thought to be paraneoplastic in these cases. The underlying malignancy is most often hematological in nature, but solid tumors have also been described.^[23]

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Media Gallery

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.

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Table. Autoimmune Conditions Reported in Patients With Relapsing Polychondritis

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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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.

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, Lewis Katz School of Medicine at Temple University

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.

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.

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