Diagnostic evaluation of relapsing polychondritis
- Clement J Michet, MD
Clement J Michet, MD
- Associate Professor of Medicine
- Mayo Clinic
Relapsing polychondritis (RPC) is a systemic inflammatory/degenerative disease process that may potentially compromise the structural and functional integrity of cartilage, organs of special sense, and the cardiovascular, renal, and nervous systems (table 1). (See "Clinical manifestations of relapsing polychondritis".)
The diagnostic evaluation of RPC is reviewed here. The pathogenesis, pathology, clinical manifestations, and treatment of RPC are discussed separately. (See "Etiology and pathogenesis of relapsing polychondritis" and "Pathology of relapsing polychondritis" and "Clinical manifestations of relapsing polychondritis" and "Treatment of relapsing polychondritis".)
The diagnosis of relapsing polychondritis (RPC) is established by the combination of clinical findings, supportive laboratory data, imaging procedures, and biopsy of an involved cartilaginous site (see 'Diagnostic criteria' below). There is no blood test that is specific for RPC.
A spectrum of histologic findings may be present in involved organs. The specific findings depend, in part, on the timing of the biopsy. Histopathologic findings upon biopsy of the auricular cartilage during active disease are pathognomonic, while an auricle with advanced disease and deformity reveals fibrosis. The pathologic features of auricular chondritis and lesions of the aorta, tracheobronchial tree, synovium, kidneys, and eye are presented separately. (See "Pathology of relapsing polychondritis".)
Diagnostic criteria — Either of two sets of empirically derived and related diagnostic criteria may be used to diagnose RPC:To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- McAdam LP, O'Hanlan MA, Bluestone R, Pearson CM. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine (Baltimore) 1976; 55:193.
- Damiani JM, Levine HL. Relapsing polychondritis--report of ten cases. Laryngoscope 1979; 89:929.
- Thompson LD. Chondrodermatitis nodularis helicis. Ear Nose Throat J 2007; 86:734.
- Moncrieff M, Sassoon EM. Effective treatment of chondrodermatitis nodularis chronica helicis using a conservative approach. Br J Dermatol 2004; 150:892.
- Zuber TJ, Jackson E. Chondrodermatitis nodularis chronica helicis. Arch Fam Med 1999; 8:445.
- Lambru G, Miller S, Matharu MS. The red ear syndrome. J Headache Pain 2013; 14:83.
- Verma G, Kanawaty D, Hyland R. Rhinoscleroma causing upper airway obstruction. Can Respir J 2005; 12:43.
- Krell WS, Staats BA, Hyatt RE. Pulmonary function in relapsing polychondritis. Am Rev Respir Dis 1986; 133:1120.
- Behar JV, Choi YW, Hartman TA, et al. Relapsing polychondritis affecting the lower respiratory tract. AJR Am J Roentgenol 2002; 178:173.
- Lin ZQ, Xu JR, Chen JJ, et al. Pulmonary CT findings in relapsing polychondritis. Acta Radiol 2010; 51:522.
- Lee KS, Ernst A, Trentham DE, et al. Relapsing polychondritis: prevalence of expiratory CT airway abnormalities. Radiology 2006; 240:565.
- Fornadley JA, Seibert DJ, Ostrov BE, Warren WS. The role of MRI when relapsing polychondritis is suspected but not proven. Int J Pediatr Otorhinolaryngol 1995; 31:101.
- Heman-Ackah YD, Remley KB, Goding GS Jr. A new role for magnetic resonance imaging in the diagnosis of laryngeal relapsing polychondritis. Head Neck 1999; 21:484.
- Dupont A, Bossuyt A, Somers G. Relapsing polychondritis: gallium-67 uptake in recurrent lung lesions. J Nucl Med Allied Sci 1983; 27:57.
- Chang MC, Tsai SC, Lin WY. Value of gallium-67 scanning in monitoring therapeutic effectiveness in a patient with relapsing polychondritis. Kaohsiung J Med Sci 2008; 24:328.
- Kao CH, Wang SJ, Yeh SH. Radionuclide imaging of relapsing polychondritis. Clin Nucl Med 1992; 17:397.
- Shi XH, Zhang FC, Chen LB, Ouyang M. The value of 99mTc methylene diphosphonate bone scintigraphy in diagnosing relapsing polychondritis. Chin Med J (Engl) 2006; 119:1129.
- Murgu S, Kurimoto N, Colt H. Endobronchial ultrasound morphology of expiratory central airway collapse. Respirology 2008; 13:315.
- Nishiyama Y, Yamamoto Y, Dobashi H, et al. [18F]fluorodeoxyglucose positron emission tomography imaging in a case of relapsing polychondritis. J Comput Assist Tomogr 2007; 31:381.
- Yamashita H, Takahashi H, Kubota K, et al. Utility of fluorodeoxyglucose positron emission tomography/computed tomography for early diagnosis and evaluation of disease activity of relapsing polychondritis: a case series and literature review. Rheumatology (Oxford) 2014; 53:1482.
- Deng H, Chen P, Wang L, et al. Relapsing polychondritis on PET/CT. Clin Nucl Med 2012; 37:712.
- Diagnostic criteria
- Diagnostic evaluation
- - History and physical examination
- - Laboratory testing
- - Tissue biopsy
- - Other testing/specialized examinations
- DIFFERENTIAL DIAGNOSIS
- Auricular inflammation
- Saddle nose deformity
- Narrowing of the airway
- Ocular inflammation
- Granulomatosis with polyangiitis (Wegener’s)
- Systemic inflammatory polyarthritis
- Aortitis and aortic aneurysms
- Chest wall pain
- POST-DIAGNOSTIC EVALUATION
- Overview of post-diagnostic testing
- - All patients
- - Selected patients
- Evaluation for pulmonary involvement
- - Pulmonary function testing
- - Imaging
- Chest radiography
- Computed tomography
- Magnetic resonance imaging
- Other imaging techniques
- - Bronchoscopy
- Evaluation for cardiac involvement
- SUMMARY AND RECOMMENDATIONS