Published on 18/03/2015 by admin
Filed under Dermatology
Last modified 18/03/2015
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David P. D’Cruz
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Relapsing polychondritis is an autoimmune rheumatic disorder characterized by cartilage inflammation. Characteristic features include nasal bridge, auricular and ocular inflammation, and major airway disease. It is rare with around 800 patients described in the literature and there is often delay in establishing a diagnosis.
The diagnosis is established by the presence of chondritis in two of three characteristic anatomical sites: auricular, nasal, laryngotracheal, or one of these sites and two other features, including ocular inflammation, audiovestibular damage or seronegative inflammatory arthritis. In most patients, histological confirmation is not necessary for diagnosis.
Auricular chondritis manifests as ear pain, redness, and swelling with sparing of the non-cartilaginous lobule. After repeated relapses, the pinnae may be floppy and distorted or have a cauliflower like appearance. The ear may become rigid following extensive calcification. Recurrent nasal chondritis results in a saddle-nose deformity.
Skin manifestations include oral ulceration, sometimes with genital ulceration, nodules, purpura, papules, sterile pustules, superficial phlebitis, livedo reticularis, skin ulcers, and distal necrosis.
Laryngeal chondritis presents with hoarseness, tracheal ring tenderness, cough, breathlessness, and stridor. All patients with suspected pulmonary disease should undergo computed tomography (CT) imaging including end-inspiratory and dynamic expiratory volumetric imaging. End-inspiratory scanning may reveal tracheal and bronchial stenosis, wall thickening, and calcification. Expiratory scans may demonstrate tracheobronchial malacia with airway collapse and air trapping.
Cardiovascular complications eventually occur in half of all patients and are the second most frequent cause of mortality. Aortic valve inflammation, the most common cardiac manifestation (10% of patients), may occur in asymptomatic patients and can silently progress during seemingly effective systemic corticosteroid therapy. Atrioventricular block, mitral regurgitation, and acute pericarditis may also occur.
Central and peripheral nerve involvement is rare. Cranial nerve lesions are the most common, but other complications include seizures, cerebral dysfunction, confusion, headaches, cerebral aneurysm, and rhomboencephalitis.
All patients should be monitored for the development of renal disease with routine urine dip testing for blood and protein.
Disease activity is assessed clinically by standard methods. There is now an objective scoring system, yet to be validated in clinical practice, that may provide objective disease assessment, for use in clinical studies.
Pearson CM, Kline HM, Newcomer VD. N Engl J Med 1960; 263: 51–8.
In this seminal paper, Pearson and colleagues coined the term ‘relapsing polychondritis’. They recognized the episodic inflammatory nature and the significant morbidity and mortality of relapsing polychondritis. Excellent clinical responses to prednisone were reported with relapses occurring on tapering the corticosteroid dosage.
Michet CJ Jr, McKenna CH, Luthra HS, O’Fallon WM. Ann Intern Med 1986; 104: 74–8.
Any major airway involvement in relapsing polychondritis is associated with a poorer prognosis than isolated head and neck disease affecting the ears, eyes or nose. This report describes the poor long-term prognostic factors: 5- and 10-year survival rates were 74% and 55%, respectively. The most frequent causes of death were infection, systemic vasculitis, and malignancy, and only 10% of the deaths were attributed to airway involvement by chondritis. Anemia at diagnosis was a marker for decreased survival. Corticosteroid therapy did not influence survival and is not a disease-modifying therapy.
Bachor E, Blevins NH, Karmody C, Kühnel T. Auris Nasus Larynx 2006; 33: 135–41.
A good description of the broad spectrum of ear manifestations. No patients died, suggesting patients with limited disease have a good prognosis.
Up to 46% of patients have impaired hearing, and inadequately treated patients can suffer permanent hearing loss. Screening audiometry is highlighted.
Yoo JH, Chodosh J, Dana R. Semin Ophthalmol 2011; 26: 261–9.
A thorough description of the ocular manifestations of relapsing polychondritis and an up-to-date review of the current therapeutic approaches to systemic disease manifestations.
Lee KS, Ernst A, Trentham DE, Lunn W, Feller-Kopman DJ, Boiselle PM. Radiology 2006; 240: 565–73.
Dynamic expiratory CT scans demonstrated abnormalities such as tracheomalacia and air trapping in 94% of relapsing polychondritis patients who had pulmonary symptoms, yet only half the patients demonstrated abnormalities on routine inspiratory CT scans. The most common findings were air trapping (94%), malacia (72%), and calcification (39%).
Expiratory CT scans show clinically relevant bronchopulmonary abnormalities earlier than standard inspiratory CT scans, allowing earlier institution of aggressive therapy to prevent disease progression.
Airway manifestations are ultimately present in over 50% of patients, and are the leading cause of death. Airway obstruction may be asymptomatic in the earlier stages, detected only on pulmonary function testing. Other sources stress the importance of plain radiography, CT, and MRI for early detection of tracheal narrowing and upper airways disease.
Francès C, el Rassi R, Laporte JL, Rybojad M, Papo T, Piette JC. Medicine (Baltimore) 2001; 80: 173–9.
The largest series in the literature of 200 patients. Oral ulceration was the most common finding in patients with relapsing polychondritis who did not have any other disease. Nodules on the limbs were the most common skin lesions and were described as erythema nodosum-like lesions with septal panniculitis. Histologic findings included vasculitis in 19 patients (leukocytoclastic in 17 and lymphocytic in two), neutrophilic infiltrates in six, thrombosis of skin vessels in four, septal panniculitis in three, and minor non-specific changes in two patients. Myelodysplastic syndrome was described in 22 patients who were more likely to have dermatological manifestations, including limb nodules, purpura, papules, and livedo reticularis. The authors recommend that elderly patients with relapsing polychondritis and skin lesions should be investigated for an underlying myelodysplastic syndrome. This confirmed previous data from Hebbar et al. 1995 (see below).
Hebbar M, Brouillard M, Wattel E, Decoulx M, Hatron PY, Devulder B, et al. Leukemia 1995; 9: 731–3.
Twenty-eight percent (five of 18) of relapsing polychondritis patients over a period of 13 years were found to have myelodysplastic syndromes.
Anemia is a poor prognostic sign in patients with relapsing polychondritis; those with concurrent myelodysplasia often develop refractory anemia requiring transfusions. Several patients have progressed to leukemia.
Del Rosso A, Rosa Petrix N, Pratesi M, Bini A. Semin Arthritis Rheum 1997; 26: 840–4.
The most frequent cardiovascular abnormalities are aortic regurgitation and aortic aneurysm and several cases of atrioventricular block, mitral regurgitation, and acute pericarditis have been reported.
For early diagnosis and treatment of these severe complications, periodic cardiovascular examination is mandatory.
Nadeau SE. Neurol Clin 2002; 20: 151–78.
This review covers the main neurological complications of relapsing polychondritis, amongst other conditions.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
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