eMedicine Specialties > Rheumatology > Vasculitis

Polychondritis: Follow-up

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

Follow-up

Complications

  • Complications of relapsing polychondritis (RP) include vertigo, tinnitus, voice hoarseness, joint deformity, epiglottitis, scleritis, conjunctivitis, iritis, need for permanent tracheotomy (severe cases), severe pulmonary infection, blindness, frail chest wall, respiratory failure, aortic regurgitation, mitral regurgitation, aortic dissection, and glomerulonephritis-associated renal failure.

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.16 However, these 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.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • Delay in diagnosis of relapsing polychondritis (RP) is common. Intubation can be difficult in patients with relapsing polychondritis, and an ear, nose, and throat evaluation may be indicated prior to elective intubation in any patient with a history of tracheal symptoms.

Special Concerns

  • Pregnant patients with relapsing polychondritis or patients who develop relapsing polychondritis during pregnancy fare well overall. Of relapsing polychondritis cases diagnosed during pregnancy, 26% were diagnosed at a mean gestational age of 20 weeks. Disease flares occur in 30% of pregnancies in patients with relapsing polychondritis, and only 30% of the patients require changes in medication. Of 24 pregnancies observed in patients with relapsing polychondritis, fetal loss was uncommon (3 of 21 nonectopic pregnancies). Of the 18 live births, 15 mature and 3 premature babies were delivered without evidence of neonatal inflammatory disease.
    • The presence of accompanying systemic diseases in pregnancy and in relapsing polychondritis is often disconcerting to practitioners and to patients. One study reported a concurrent prevalence of other inflammatory disorders to occur in 45% of patients with relapsing polychondritis, including RA, systemic lupus erythematosus with antiphospholipid syndrome, Takayasu arteritis, and ankylosing spondylitis. Patients who are severely affected by relapsing polychondritis are advised against pregnancy; however, analysis of 25 pregnancies did not find a difference in pregnancy outcome in patients with concurrent systemic disease and relapsing polychondritis.
    • Systemic administration of prednisone is the treatment of choice in pregnant patients with relapsing polychondritis. NSAIDs have been used but are not effective and must be withdrawn 8 weeks prior to delivery because of the risk of problems in the neonate and/or mother, such as "delayed onset or increased duration of labor, constriction of the ductus arteriosus in utero, and persistent pulmonary hypertension in the neonate".39,40
    • Dapsone has not been reported to be embryotoxic and has been found to be safe in clinical experience with pregnant patients treated for leprosy and Hansen disease. Cyclophosphamide is toxic in the first trimester of pregnancy, and MTX is also toxic in early pregnancy.
 


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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