eMedicine Specialties > Rheumatology > Vasculitis

Polychondritis: Differential Diagnoses & Workup

Author: Nicholas Compton, MD, Staff Physician, Department of Medicine, Division of Dermatology, University of Washington Medical Center
Coauthor(s): Jane H Buckner, MD, Clinical Assistant Professor of Immunology, University of Washington; Director of Translation Research Program, Associate Member, Department of Immunology/Rheumatology, Benaroya Research Institute at Virginia Mason Research Center; Karin I Harp, MD, Consulting Staff, Department of Dermatology, Everett Clinic; Gregory J Raugi, MD, PhD, Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle
Contributor Information and Disclosures

Updated: Jun 11, 2009

Differential Diagnoses

Addison Disease
Rheumatoid Arthritis
Behcet Disease
Syphilis
Cellulitis
Systemic Lupus Erythematosus
Hyperthyroidism
Wegener Granulomatosis
Polyarteritis Nodosa

Other Problems to Be Considered

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)

Auricular chondritis

Infectious perichondritis (commonly due to Pseudomonas aeruginosa infection); also, fungal infection, tuberculosis, syphilis, and leprosy
Chronic external otitis
Trauma
Frostbite
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

Rheumatoid arthritis (adult or juvenile)
Reactive arthritis
Acute gonococcal arthropathy
Rheumatic fever
Wegener granulomatosis
Polyarteritis nodosa
Systemic lupus erythematosus and other collagen-vascular disorders

Nasal chondritis/saddle-nose deformity

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

Ocular inflammation

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 Wegener granulomatosis (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)

Tracheal obstruction

Trauma (eg, strangulation)
Prolonged intubation
Sarcoidosis
Wegener granulomatosis
Endoluminal malignancy
Tuberculosis/sarcoidosis webs

Respiratory tree chondritis

Perichondritis of the larynx resulting from herpes, syphilis, erysipelas, tonsillitis, peritonsillar abscess, tuberculosis, measles, diphtheria, scarlet fever, avitaminosis, blastomycosis, actinomycosis, Wegener granulomatosis, xanthoma, typhus, Vincent infection, anthrax, or smallpox

CNS alterations

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

Aortitis

Erdheim cystic medial necrosis
Marfan syndrome
Syphilitic aortitis
Giant cell arteritis

Workup

Laboratory Studies

  • No laboratory findings are specific for relapsing polychondritis (RP).
  • Anemia, if present, is typically normochromic and normocytic and is associated with a poor prognosis.
  • Nonspecific indicators of inflammation (eg, elevated erythrocyte sedimentation rate, elevated levels of C-reactive protein) are often present.
  • Mild leukocytosis may be detected.
  • Because relapsing polychondritis is associated with many multisystemic diseases, a laboratory evaluation commensurate with the spectrum of reported symptoms is indicated to ascertain the presence of complicating conditions.
  • Use antinuclear antibody reflexive panel, rheumatoid factor, and antiphospholipid antibodies (if history of thrombosis is found) to evaluate for other autoimmune connective-tissue diseases.
  • For a vasculitis workup, perform a CBC count with differential; metabolic panel; creatinine, liver transaminase, and serum alkaline phosphatase studies; urinalysis dipstick and microscopic evaluation of sediment; cryoglobulins; viral hepatitis panel; antinuclear antibody (ANA); and antineutrophil cytoplasmic antibody (ANCA) tests.
  • Use the purified protein derivative test to evaluate for exposure to tuberculosis. (Tuberculosis is often overlooked as an infectious cause of perichondritis.)
  • Use serologic tests for syphilis if it is suspected, including rapid plasma reagent or VDRL testing. Saddle-nose deformity is a clinical manifestation of congenital syphilis and can go undiagnosed into adulthood; however, it can also be a consequence of gumma formation in adulthood.
  • Cultures may be indicated, depending on the clinical presentation.
    • Sputum cultures for bacteria and acid-fast bacilli may be needed in patients with respiratory symptoms.
    • Bacterial, acid-fast bacilli, and fungal cultures may be appropriate for cartilage biopsy samples, especially from the respiratory tree.
    • Blood cultures may be useful in the assessment of febrile episodes that are combined with nausea, vertigo, and/or muscle weakness.
    • Bacterial and viral cultures of the cerebrospinal fluid may be indicated to exclude meningitis or to help exclude aseptic meningitis or CNS vasculitis.

Imaging Studies

  • Chest radiography (posteroanterior [PA] and lateral views)
    • Tracheal stenosis may be observed on plain radiographs.

      Tracheal stenosis on chest x-ray film. Courtesy o...

      Tracheal stenosis on chest x-ray film. Courtesy of Julie E. Takasugi, MD.

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      Tracheal stenosis on chest x-ray film. Courtesy o...

      Tracheal stenosis on chest x-ray film. Courtesy of Julie E. Takasugi, MD.

    • Calcification of cartilaginous structures supports the diagnosis of relapsing polychondritis.
    • Coexisting systemic vasculitis may be suggested by the presence of pulmonary parenchymal infiltrates.
  • Spiral CT scanning (without contrast)
    • Spiral CT scanning (without contrast), from the superior trachea to the lower lobe bronchi, is advised in patients with relapsing polychondritis and respiratory symptoms.
    • Spiral CT scanning is a noninvasive test that readily identifies tracheal and bronchial thickening, stenosis, and calcification. Smooth anterior and lateral wall thickening with sparing of the posterior wall of the trachea and mainstem bronchi is virtually pathognomonic for relapsing polychondritis.
    • High-resolution CT scanning can reveal air trapping and diffuse or focal thickening of the airways. Expiratory CT scanning can be used to evaluate for air trapping and malacia of the airways. A series of 18 patients with relapsing polychondritis and pulmonary symptoms revealed that 94% had airway malacia and air trapping on dynamic expiratory CT scans.34 The authors suggest that this modality should be used in all patients with relapsing polychondritis to allow for early detection of airway compromise. However, they did not provide the duration of disease in the study population, nor did they correlate the findings with those of pulmonary function tests. The benefit of dynamic expiratory CT scanning is unproven but may provide more information in difficult cases.
    • CT scanning results correlate well with pulmonary function tests, identifying obstructive patterns. CT scanning is not only safer but is also more sensitive and specific than bronchoscopy.
  • MRI
    • MRI has been a useful adjunct in the clinical diagnosis of relapsing polychondritis
    • MRI is better able to distinguish between edema, fibrosis, and inflammation than is CT scanning.
    • T1-weighted images, T2-weighted images, and T1-weighted images with gadolinium contrast provide characterization of relapsing polychondritis-related changes in cartilaginous tissues.
    • MRI also reveals thickening of the thoracic aorta before dilatation occurs.
    • MRI may be useful for monitoring the effects of treatment.
  • Posteroanterior and lateral dye contrast pharyngotracheogram
    • PA and lateral dye contrast pharyngotracheogram may be helpful if tracheal narrowing or edema is suggested.
    • Both views are required to avoid underestimating the severity of stenosis or swelling.
  • Scintigraphy
    • Scintigraphy may prove helpful for identifying potential sites for biopsy to aid the histologic diagnosis when the clinical diagnosis is in doubt (ie, because of unfulfilled diagnostic criteria).
    • Technetium Tc 99m methylene diphosphonate bone scintigraphy has been used in the evaluation of chest pain, allowing identification of possible sites for biopsy in costochondral tissues.
    • Gallium Ga 67 citrate scintigraphy has also been found to show increased uptake in affected areas.

Other Tests

  • Pulmonary function testing with flow-volume loop studies
    • Pulmonary function testing (PFT) with flow-volume loops is strongly recommended in patients who present with respiratory symptoms, since PFT may assist in the differential diagnoses and provide information about severity of the disease. This may also be used to monitor patients' disease activity.
    • PFT in patients with relapsing polychondritis who have respiratory involvement demonstrates a nonreversible obstructive pattern with collapse and stenosis of the airways. The decrease in forced expiratory volume in 1 second correlates with the degree of dyspnea.
  • Electrocardiography
    • Perform ECG to assess patients with relapsing polychondritis who demonstrate signs of vasculitis.
    • Also, perform ECG to monitor these patients, since they may incur silent ischemia if vasculitis has developed.
  • Echocardiography: Echocardiogram may be needed to assess aortic root dilatation and degree of aortic regurgitation.

Procedures

  • Intubation may be dangerous and futile.
  • Tracheostomy is usually the best method for providing an airway in patients with relapsing polychondritis in acute respiratory distress (because of the high likelihood of tracheal or bronchial stenosis or edema).
  • Biopsy of the cartilage is a potential source of infection and cosmetic damage. Perform biopsy on cartilage only if histopathological data are required to meet the diagnostic criteria for relapsing polychondritis.
  • Biopsy of skin lesions (nonadjacent to cartilage) may provide useful adjunctive information.

Histologic Findings

Biopsy of cartilage in patients with relapsing polychondritis demonstrates chondrolysis, chondritis, and perichondritis. The cartilage loses its basophilia, probably by release of sulfated proteoglycans from the matrix, and the chondrocytes are decreased in number and may appear pyknotic. Early relapsing polychondritis is characterized by a mixed inflammatory infiltrate of lymphocytes, neutrophils, and plasma cells in the perichondrium. As the cartilage degenerates, mononuclear cells and macrophages infiltrate the matrix. The cartilage matrix is eventually destroyed and replaced by fibrous connective tissue. Despite the presence of clinical erythema, overlying skin is normal.

Distant lesions with the clinical appearance of vasculitis have histologic features consistent with the clinical syndrome, including leukocytoclastic or granulomatous vascular injury.

More on Polychondritis

Overview: Polychondritis
Differential Diagnoses & Workup: Polychondritis
Treatment & Medication: Polychondritis
Follow-up: Polychondritis
Multimedia: Polychondritis
References
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Further Reading

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Keywords

relapsing polychondritis, RP, cartilaginous inflammation, cartilage inflammation, inflamed cartilage, inflamed ear, ear inflammation, inflamed nose, nose inflammation, inflamed laryngotracheobronchial tree, laryngotracheobronchial tree inflammation, airway chondritis, infection secondary to corticosteroid treatment, respiratory compromise, systemic vasculitis, auricular chondritis, seronegative arthritis, non-nodular arthritis, nonnodular arthritis, respiratory tract chondritis, audiovestibular damage, audio-vestibular damage, aortic arch syndrome, abdominal aortic aneurysm, aortic regurgitation, chondrolysis, chondritis, perichondritis

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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, Clinical Assistant Professor of Immunology, University of Washington; Director of Translation Research Program, Associate Member, Department of Immunology/Rheumatology, Benaroya Research Institute at Virginia Mason Research Center
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; 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.

Medical Editor

Bryan L Martin, DO, Chief, Allergy Immunology Department, Walter Reed Army Medical Center; Associate Professor of Medicine and Pediatrics, Uniformed Services University of the Health Sciences; United States Army Consultant in Allergy Immunology and Immunizations
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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

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.

CME Editor

Alex J Mechaber, MD, FACP, 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, Professor of Medicine, Temple University 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: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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