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Relapsing Polychondritis Differential Diagnoses

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

Diagnostic ConsiderationsAuricular chondritisInflammatory arthritisNasal chondritis/saddle-nose deformityOcular inflammationTracheal obstructionRespiratory tree chondritisCNS alterationsAortitis

Other conditions to consider in the differential diagnosis of polychondritis include the following:

  • Rheumatoid arthritis
  • Polyarteritis nodosa
  • Cogan syndrome
  • Infectious perichondritis
  • MAGIC syndrome (relapsing polychondritis [RP] plus Behçet disease)
  • Trauma (especially in boxers and wrestlers)
  • Congenital syphilis
  • Chronic external otitis
  • Auricular calcification (secondary to other conditions, eg, trauma, Addison disease, diabetes, hyperthyroidism)

Causes include the following:

Causes include the following:

  • Rheumatoid arthritis (adult or juvenile)
  • Reactive arthritis
  • Acute gonococcal arthropathy
  • Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)
  • Polyarteritis nodosa
  • Systemic lupus erythematosus and other collagen-vascular disorders

Causes include the following:

  • Infectious perichondritis
  • Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)
  • Congenital syphilis
  • Nasal NK/peripheral T-cell lymphoma (formerly known as angiocentric lymphoma)

Causes include the following:

  • Reactive arthritis (ie, conjunctivitis plus arthritis plus urethritis)
  • Rheumatoid arthritis, Behçet disease, enteropathic arthritis, or Still disease (ie, iritis or chorioretinitis plus arthritis)
  • Polyarteritis nodosa or granulomatosis with polyangiitis (ie, scleritis or episcleritis plus arthritis)
  • Sjögren syndrome (ie, keratoconjunctivitis sicca plus arthritis)
  • Cogan syndrome (ie, intersitial keratitis plus cochlear and vestibular damage)
  • Arteriosclerosis, syphilis, collagen vascular disease, herpes zoster, sickle cell disease, migraine, coagulation disorders (ie, ischemic optic neuropathy)

Causes include the following:

  • Trauma (eg, strangulation)
  • Prolonged intubation
  • Sarcoidosis
  • Granulomatosis with polyangiitis
  • Endoluminal malignancy
  • Tuberculosis/sarcoidosis webs

Perichondritis of the larynx resulting from any of the following:

  • Herpes
  • Syphilis
  • Erysipelas
  • Tonsillitis
  • Peritonsillar abscess
  • Tuberculosis
  • Measles
  • Diphtheria
  • Scarlet fever
  • Avitaminosis
  • Blastomycosis
  • Actinomycosis
  • Granulomatosis with polyangiitis
  • Xanthoma
  • Typhus
  • Vincent infection
  • Anthrax

Causes include the following:

  • Septic meningitis (fungal, bacterial, mycobacterial)
  • Aseptic meningitis unrelated to relapsing polychondritis, ie, viral
  • Temporal arteritis
  • Malignancy
  • Drug toxicity
  • Encephalitis or meningoencephalitis
  • Other causes of cerebral vasculitis
  • Other causes of seizure disorder
  • Leprosy

Causes include the following:

Differential Diagnoses

Contributor Information and Disclosures

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.


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
DiseasePatients With Condition/Total PatientsReferences
Systemic vasculitis3 (5%) of 62Zeuner et al[17]
11 (10%) of 112Michet et al[18]
8 (12%) of 66Trentham and Le[19]
28 (18%) of 159McAdam et al[16]
50 (13%) of 399Total
Cutaneous leukocytoclastic vasculitis2 (33%) of 6Priori et al[20]
6 (5%) of 112Michet et al[18]
8 (7%) of 118Total
Thyroid disease8 (5%) of 159McAdam et al[16]
10 (15%) of 66Trentham and Le[19]
2 (33%) of 6Priori et al[20]
4 (4%) of 112Michet et al[18]
2 (3%) of 62Zeuner et al[17]
26 (6%) of 405Total
Rheumatoid arthritis*8 (5%) of 159McAdam et al[16]
3 (2%) of 180Piette et al[21]
8 (7%) of 112Michet et al[18]
7 (11%) of 62Zeuner et al[17]
26 (5%) of 513Total
Systemic lupus erythematosus†2 (1%) of 159McAdam et al[16]
9 (5%) of 180Piette et al[21]
1 (17%) of 6Priori et al[20]
6 (5%) of 112Michet et al[18]
3 (5%) of 62Zeuner et al[17]
21 (4%) of 519Total
Sjögren syndrome (possible)5 (3%) of 159McAdam et al[16]
5 (5%) of 111Piette et al[21]
10 (4%) of 270Total
Ulcerative colitis3 (2%) of 159McAdam et al[16]
2 (3%) of 62Zeuner et al[17]
5 (2%) of 221Total
Crohn disease2 (1%) of 180Piette et al[21]
1 (2%) 62Zeuner et al[17]
1 (100%) of 1Haigh et al[22]
4 (2%) of 243Total
Mixed connective-tissue disease5 (3%) of 180Piette et al[21]
2 (2%) of 112Michet et al[18]
7 (2%) of 292Total
Takayasu arteritis3 (2%) of 180Piette et al[21]
Mesenteric panniculitis3 (2%) of 180Piette et al[21]
Spondyloarthropathy2 (1%) of 180Piette et al[21]
3 (3%) of 112Michet et al[18]
2 (3%) of 62Zeuner et al[17]
7 (2%) of 354Total
Diabetes mellitus1 (2%) of 62Zeuner et al[17]
3 (2%) of 159McAdam et al[16]
4 (2%) of 221Total
Reactive arthritis/psoriatic arthritis2 (1%) of 159McAdam et al[16]
1 (< 1%) of 112Michet et al[18]
3 (1%) of 271Total
Systemic sclerosis2 (1%) of 159McAdam et al[16]
Raynaud syndrome2 (1%) of 159McAdam et al[16]
Glomerulonephritis2 (1%) of 159McAdam et al[16]
Dysgammaglobulinemia2 (1%)of 159McAdam et al[16]
Pernicious anemia1 (1%) of 159McAdam et al[16]
Behçet disease*1 (< 1%) of 112Michet et al[18]
Psoriasis2 (1%) of 180Piette et al[21]
Lichen planus2 (1%) of 180Piette et al[21]
Primary biliary cirrhosis1 (< 1%) of 112Michet 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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