Miscellaneous
Relapsing Polychondritis in the Department of Defense Population and Review of the Literature

Presented in part at the American College of Rheumatology (ACR)/Association of Rheumatology Health Professionals (ARHP) 2010 Annual Scientific Meeting, November 10, 2010, Atlanta, GA, USA.
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Objective

The objective of this study was to characterize the clinical features of relapsing polychondritis (RPC) within the Department of Defense beneficiary population and determine the utility of echocardiography, imaging studies, and pulmonary function testing for diagnosis and monitoring disease.

Methods

We performed a retrospective Electronic Medical Record chart review of all patients diagnosed with RPC within the Department of Defense between January 2004 and December 2009.

Results

Thirty patients met McAdam's diagnostic criteria and an additional 13 met our criteria for partial RPC. Auricular chondritis (88%), inflammatory eye disease (57%), and arthritis (60%) were the most common clinical manifestations. Pulmonary involvement was seen in 16 (37%) patients. Methotrexate (42%) and corticosteroids (21%) were the most conventional therapies. Thirty (70%) patients had pulmonary function tests with flow volume loop abnormalities observed in 33%. Chest computed tomography was performed in 63%, with abnormalities in 48%. Abnormalities on echocardiography were observed in 12 of 25 (48%) patients.

Conclusions

The incidence, demographic data, and organ involvement in our RPC patients were similar to previous studies. The diagnosis of RPC was determined primarily on physical examination and symptom-driven diagnostic testing. There was no notable pattern by rheumatologists for monitoring the progression of tracheobronchial tree or large vessel involvement. Interpreting flow volume loops is recommended with pulmonary function testing to detect early laryngotracheal involvement. Computed tomography of the chest is also recommended to monitor for vascular and tracheobronchial tree involvement.

Section snippets

Materials and Methods

We performed a retrospective electronic medical record (EMR) review, with approval by the Brooke Army Medical Center Institutional Review Board, for all patients diagnosed with RPC, within the DoD beneficiary population between January 2004 and December 2009. The International Classification of Diseases, ninth Revision (ICD-9) diagnostic code, 733.9 polychondritis (atrophic, chronic, relapsing), was used to search the centralized DoD database to identify these patients. During the specified

Results

There were 50 patients diagnosed with RPC in the DoD between January 2004 and December 2009 based on the EMR. These 50 charts were reviewed and 43 patients met the study inclusion criteria. Thirty patients met McAdam's diagnostic criteria and an additional 13 met criteria for partial RPC. Seven patients were excluded, as the patients were erroneously identified on a single visit as having RPC. Of the 7 excluded patients, 1 had biopsy-proven Wegener's granulomatosis, 1 had trauma to the

Discussion

RPC is a rare autoimmune disease characterized by recurrent inflammation of cartilaginous structures. The disease frequently presents with a sudden onset, although the presenting features are variable. The clinical manifestations and biopsy findings of this disease were first classified by McAdam and colleagues (5); the criteria for diagnosis have been modified to accommodate different presentations of disease and limit the need for biopsy (Table 1). McAdam's clinical features include bilateral

References (83)

  • A. Del Rosso et al.

    Cardiovascular involvement in relapsing polychondritis

    Semin Arthritis Rheum

    (1997)
  • L. Sampaio et al.

    Central nervous system involvement in relapsing polychondritis

    Joint Bone Spine

    (2010)
  • K.A. Mark et al.

    Colchicine and indomethacin for the treatment of relapsing polychondritis

    J Am Acad Dermatol

    (2002)
  • A. Belot et al.

    Pediatric-onset relapsing polychondritis: Case series and systematic review

    J Pediatr

    (2010)
  • R.P. Rapini et al.

    Relapsing polychondritis

    Clin Dermatol

    (2006)
  • T. Nakajima et al.

    Long-term management of polychondritis with serial tracheobronchial stents

    Ann Thorac Surg

    (2006)
  • R. Jaksch-Wartenhorst

    Polychondropathia

    Wien Arch Intern Med

    (1923)
  • C.M. Pearson et al.

    Relapsing polychondritis

    N Engl J Med

    (1960)
  • P. Kent et al.

    Relapsing polychondritis

    Curr Opin Rheumatol

    (2003)
  • L.P. McAdam et al.

    Relapsing polychondritis: Prospective study of 23 patients and a review of the literature

    Medicine (Baltimore)

    (1976)
  • C.J. Michet et al.

    Relapsing polychondritisSurvival and predictive role of early disease manifestations

    Ann Intern Med

    (1986)
  • J.M. Damiani et al.

    Relapsing polychondritis–report of ten cases

    Laryngoscope

    (1979)
  • M.T. Chow et al.

    Relapsing polychondritis

    Optom Vis Sci

    (2000)
  • A. Mohammad et al.

    Relapsing polychondritis: Reversible airway obstruction or asthma

    Exp Rheum

    (2008)
  • Z.Q. Lin et al.

    Pulmonary CT findings in relapsing polychondritis

    Acta Radiol

    (2010)
  • K.S. Lee et al.

    Relapsing polychondritis: Prevalence of expiratory CT airway abnormalities

    Radiology

    (2006)
  • Y.D. Heman-Ackah et al.

    A new role for magnetic resonance imaging in the diagnosis of laryngeal relapsing polychondritis

    Head Neck

    (1999)
  • W.S. Krell et al.

    Pulmonary function in relapsing polychondritis

    Am Rev Respir Dis

    (1986)
  • B. Hojaili et al.

    Relapsing polychondritis presenting with complete heart block

    J Clin Rheum

    (2008)
  • A. Chang-Miller et al.

    Renal involvement in relapsing polychondritis

    Medicine (Baltimore)

    (1987)
  • C. Francès et al.

    Dermatologic manifestations of relapsing polychondritisA study of 200 cases at a single center

    Medicine (Baltimore)

    (2001)
  • K.C. Hsu et al.

    Aseptic meningitis and ischemic stroke in relapsing polychondritis

    Clin Rheum

    (2006)
  • S.M. Yang et al.

    Relapsing polychondritis with encephalitis

    J Clin Rheum

    (2004)
  • T. Papo et al.

    Pregnancy in relapsing polychondritis

    Arthritis Rheum

    (1997)
  • R. Ananthakrishna et al.

    Relapsing polychondritis-case series from South India

    Clin Rheum

    (2009)
  • T. Stabler et al.

    Serum cytokine profiles in relapsing polychondritis suggest monocyte/macrophage activation

    Arthritis Rheum

    (2004)
  • E. Keller et al.

    A novel variant of DR4 (DRB1*0421) identified in a patient with polychondritis

    Immunogenetics

    (1995)
  • S. Hue-Lemoine et al.

    HLA-DQA1 and DQB1 alleles are associated with susceptibility to relapsing polychondritis: From transgenic mice to humans

    Arthritis Rheum

    (1999)
  • A. Anstey et al.

    Relapsing polychondritis: Autoimmunity to type II collagen and treatment with cyclosporin A

    Br J Dermatol

    (1991)
  • A.D. Ormerod et al.

    Relapsing polychondritis-treatment with cyclosporin A

    Br J Dermatol

    (1992)
  • Y. Matsumoto et al.

    Measurement of anti-type II collagen antibody diagnosis and follow-up useful in a case of relapsing polychondritis

    Nihon Kokyuki Gakkai Zasshi

    (2002)
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    Michael J. Morras is on the speakers bureau for Pfizer/Boehringer-Ingelheim. The authors have no conflicts of interest to disclose.

    “The opinions in this manuscript do not constitute endorsement by Brooke Army Medical Center, the U.S. Army Medical Department, the U.S. Army Office of the Surgeon General, the Department of the Army, Department of Defense or the U.S. Government of the information contained therein.”

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