Diagnostic Considerations
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
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Auricular calcification (secondary to other conditions, eg, trauma, Addison disease, diabetes, hyperthyroidism)
Auricular chondritis
Causes include the following:
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Infectious perichondritis (commonly due to Pseudomonas aeruginosa infection); also, fungal infection, tuberculosis, syphilis, and leprosy
-
Chronic external otitis
-
Trauma
-
Calcification of the pinna resulting from Addison disease, ochronosis, acromegaly, essential hypertension, diabetes mellitus, and familial cold hypersensitivity
-
Complication of mastoid surgery
-
Benign nodular deformity (ie, chondrodermatitis nodularis chronica helicis)
Inflammatory arthritis
Causes include the following:
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Rheumatoid arthritis (adult or juvenile)
-
Reactive arthritis
-
Acute gonococcal arthropathy
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Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)
-
Polyarteritis nodosa
-
Systemic lupus erythematosus and other collagen-vascular disorders
Nasal chondritis/saddle-nose deformity
Causes include the following:
-
Infectious perichondritis
-
Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)
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Congenital syphilis
-
Nasal NK/peripheral T-cell lymphoma (formerly known as angiocentric lymphoma)
Ocular inflammation
Causes include the following:
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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)
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Sjögren syndrome (ie, keratoconjunctivitis sicca plus arthritis)
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Cogan syndrome (ie, intersitial keratitis plus cochlear and vestibular damage)
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Arteriosclerosis, syphilis, collagen vascular disease, herpes zoster, sickle cell disease, migraine, coagulation disorders (ie, ischemic optic neuropathy)
Tracheal obstruction
Causes include the following:
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Trauma (eg, strangulation)
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Prolonged intubation
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Sarcoidosis
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Granulomatosis with polyangiitis
-
Endoluminal malignancy
-
Tuberculosis/sarcoidosis webs
Respiratory tree chondritis
Perichondritis of the larynx resulting from any of the following:
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Herpes
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Syphilis
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Erysipelas
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Tonsillitis
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Peritonsillar abscess
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Tuberculosis
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Measles
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Diphtheria
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Scarlet fever
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Avitaminosis
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Blastomycosis
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Actinomycosis
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Granulomatosis with polyangiitis
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Xanthoma
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Typhus
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Vincent infection
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Anthrax
CNS alterations
Causes include the following:
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Septic meningitis (fungal, bacterial, mycobacterial)
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Aseptic meningitis unrelated to relapsing polychondritis, ie, viral
-
Ménière disease
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Temporal arteritis
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Malignancy
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Drug toxicity
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Encephalitis or meningoencephalitis
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Other causes of cerebral vasculitis
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Other causes of seizure disorder
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Leprosy
Aortitis
Causes include the following:
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Erdheim cystic medial necrosis
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Syphilitic aortitis
Differential Diagnoses
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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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