Relapsing Polychondritis

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Synonyms: None

Etiology: Unknown, antibodies against type II collagen are common;

HLA-DR4 association Associations: Myelodysplastic syndromes, systemic vasculitis, connective tissue/autoimmune diseases Clinical: Erythematous, painful ear(s), nose, arthropathy, respiratory, ocular symptoms

Histology: Frequently nonspecific, degenerative changes of cartilage with fibrosis and lymphocytic infiltrate at chondrofibrous junction IF: Immunoglobulin and C3 at chondrofibrous junction

Evaluation: CBC, creatinine, urinalysis, ESR, RF, PFTs, ophthalmological evaluation, EKG, possible biopsy of involved ear Treatment: Systemic corticosteroids, nonsteroidal anti-inflammatory drugs, colchicine, methotrexate Prognosis: >55% 10-year survival

In 1923, Jaksch-Wartenhorst described a 32-year-old man with polyarthritis and fever who subsequently developed bilateral auricular and nasal chondritis. He applied the term polychondropathia to this entity (1). In 1960, Pearson and colleagues added four more cases, and coined the term relapsing polychondritis, which has become the accepted terminology for this disease (2).

Relapsing polychondritis (RP) is a systemic disease of unknown etiology, associated with the formation of antibodies to type II collagen, the type of collagen predominating in cartilage. This results in inflammation of the cartilage of the ears, nose, tracheobronchial tree, and joints (3). Involvement of inner ear and heart may be explained by evidence suggesting that type II collagen exists in those tissues (4,5). Murine models suggest that tissue-specific antibody and complement activation are both necessary for expression of the disease (6). The presence of circulating antibody to type II collagen is not specific to RP. In one investigation, these antibodies were found in 50% of RP patients, 15% of rheumatoid arthritis patients, and 4% of normal controls (7). Reactivity against different epitopes probably results in different disease manifestations. Antibodies in the sera of normal controls are specific to bovine or chick type II collagen, suggesting that they are induced by dietary exposure to antigen. These antibodies do not cross-react with human type II

collagen (7). Genetic susceptibility to RP is associated with expression of HLA-DR4, but no predominant subtype is present in patients with the disease (8). Further evidence of a genetic component to RP is seen in its association with multiple autoimmune diseases.

The most common presentation of RP is auricular chondritis or arthritis. Other relatively frequent presentations include nasal chondritis, laryngotracheal symptoms, ocular inflammation, auditory or vestibular dysfunction, or cutaneous eruption. The onset of disease is usually abrupt, and most commonly is manifested as a tender, erythematous, indurated ear (Figure 34.1). Bilateral involvement is common. Acute episodes of chondritis last from days to several weeks, and resolve spontaneously. Repeated episodes result in a distorted auricular contour, with a "cauliflower ear" deformity, and a similar nasal deformity, resulting in a "saddle nose." Arthropathy is also a common presenting manifestation of RP, and is usually migratory, asymmetric, non-nodular, nonerosive, and seronegative. Joint sizes involved are variable and include parasternal articulations (9). Anemia and an elevated erythrocyte sedimentation rate may be present. Noncutaneous manifestations of RP are catalogued in Table 34.1 (10-12).

Cutaneous involvement in RP is divided into two categories: that consisting of changes overlying areas of auric-

Figure 34.1. Erythema and induration of the ear, with sparing of the lobe.

ular or nasal chondritis, and those cutaneous manifestations not directly associated with chondritis. Typical ear and nasal involvement with cutaneous erythema and induration occurs in the majority of patients with RP at some time during the course of their disease, frequently at the outset. Other cutaneous findings attributable to RP occur in approximately 35% of patients. However, the actual percentage of patients with skin disease in RP is greater than that because of the cutaneous manifestations of co-morbidities. Cutaneous, nonchondritic presentations of RP represent approximately 10% of the total (13). The more common mucocutaneous lesions seen in a series of 200 patients with RP include oral and genital aphtho-sis, acral subcutaneous nodules resembling erythema nodosum, and purpura, most of which is due to small vessel neutrophilic vasculitis. A summary of cutaneous findings excluding skin changes superficial to chondritis reported in this case series is given in Table 34.2 (13).

Cutaneous lesions have not been found to correlate with disease severity, or specific organ system involvement, but

Table 34.1. Clinical Features of Relapsing Polychondritis Auricular and nasal chondritis

Laryngotracheal inflammation (throat pain, cough, hoarseness, dyspnea, stridor, wheezing, choking) Arthritis

Ocular (episcleritis, scleritis, iritis, keratoconjunctivitis sicca, keratitis, optic neuritis, retinopathy, cataracts, proptosis, eyelid edema)

Neurologic (headaches, cranial nerve palsies, cerebellar ataxia, hemiplegia, encephalopathy, seizures) Audiovestibular (hearing loss, tinnitus, vertigo, dizziness, nausea, vomiting)

Cardiovascular (aortic or mitral regurgitation, aortic aneurysm, myocarditis, pericarditis, silent myocardial infarction, conduction system abnormalities) Renal (mesangial proliferation, focal and segmental necrotizing glomerulonephritis, glomerulosclerosis, IgA nephropathy, tubulointerstitial nephritis) Constitutional (fever, malaise, weight loss)

occur with higher frequency in patients with coexistent disease, particularly those with myelodysplastic syndromes. Cutaneous disease activity correlates with systemic disease activity in approximately 50% of cases.

RP is a multisystem disease with a wide array of clinical manifestations, potentially delaying diagnosis. This is further complicated by its occurrence with other diseases in 25%-35% of cases (11). The most common disease associations are myelodysplastic syndromes, in approximately 10% of cases, other connective tissue and autoimmune disease, and systemic vasculitis. Disease associations with RP are summarized in Table 34.3 (10,11,13).

There is no specific diagnostic test for RP. Criteria for diagnosis as suggested by McAdam include presence of three of the following clinical features and histologic confirmation: (1) bilateral auricular chondritis, (2) non-erosive seronegative inflammatory polyarthritis, (3) nasal chondritis, (4) ocular inflammation, (5) respiratory tract chondritis, (6) audiovestibular damage (11). In an attempt to diagnose RP, biopsy specimens of areas suspected to represent chondritis are frequently taken, the ear being the most common site. Sampling of posterior auricular

Table 34.2. Mucocutaneous Findings in RP (in Approximate Order of Frequency) Aphthosis, oral and genital

Acral subcutaneous nodules (septal panniculitis, neutrophilic vasculitis, neutrophilic panniculitis, vascular thrombosis) Purpura (neutrophilic predominating over lymphocytic vasculitis) Papular eruption (urticarial, or blue-gray) Sterile pustules Superficial phlebitis Livedo reticularis Digital necrosis

Table 34.3. Relapsing Polychondritis: Associated Diseases (in Approximate Order of Frequency)

Hematologic (myelodysplastic syndrome, IgA myeloma, lymphoma, acute leukemia)

Connective tissue disease (rheumatoid arthritis, juvenile rheumatoid arthritis, systemic lupus erythematosus, scleroderma, dermatomyositis, Sjogren's syndrome, Reiter's syndrome, psoriatic arthritis)

Autoimmune diseases (Grave's disease, hypothyroidism, Hashimoto's thyroiditis, ulcerative colitis, Crohn's disease, myasthenia gravis, primary biliary cirrhosis, pernicious anemia, diabetes mellitus) Systemic vasculitis (Wegener's granulomatosis, polyarteritis nodosa, Behcet's disease, Churg-Strauss syndrome, temporal arteritis, Takayasu's arteritis) Dermatologic disease (psoriasis, lichen planus, vitiligo, atopic dermatitis, Sweet's syndrome (14), erythema nodosum, erythema elevatum diutinum (15)) Infection (hepatitis C infection (16), HIV infection (17))

skin and underlying cartilage is preferred to prevent accentuation of any contour deformities. Histopathologic changes of affected ear are not specific, and may include perichondrial fibroplasia, collagen hyalinization, angio-plasia, hemosiderin deposits, vacuolated or pyknotic chondrocytes, loss of cartilaginous matrix basophilia, and lymphohistiocytic and plasmacytic inflammation (Figure 34.2A and 34.2B) (18). Given the distinctive clinical pattern of RP in most cases and nonspecific histologic findings, it has been suggested that performing a biopsy is usually unnecessary to make the diagnosis (19). Direct immunofluorescence of affected areas may show granular deposits of immunoglobulins and C3 at the chondrofi-brous junction (20).

The differential diagnosis of relapsing polychondritis is extensive because of the wide variety of disease manifesta-

Table 34.4. Evaluation of the Patient with Relapsing Polychondritis

1. Complete blood count

2. Erythrocyte sedimentation rate

3. Urinalysis

4. Serum creatinine

5. Rheumatoid factor

6. Pulmonary function tests, with CT of chest if abnormal

7. Ophthalmological examination

8. Electrocardiogram

9. Echocardiography if abnormal cardiac physical examination tions. In the classic presentation with auricular chondri-tis, the differential diagnosis includes auricular cellulitis associated with otitis externa, systemic vasculitis, trauma, frostbite, phototoxic reaction, and arthropod bite reaction. Chondritis is usually distinguished by its frequent bilateral occurrence and typical sparing of the lobe because of its lack of cartilage. With repeated episodes, distortion of the auricular contour may be noted, and there may be radiographic evidence of dystrophic calcification. Joint presentations may mimic other forms of arthritis, including rheumatoid arthritis, septic arthritis, and crystal-induced arthritis. Other organ system involvement may result in clinical presentations resembling Beh├žet's disease, Wegener's granulomatosis, sarcoidosis, asthma, posterior circulation stroke, or Meniere's disease.

Patients with RP should undergo diagnostic evaluation because of the possibility of multisystem disease and coexistent diseases. While evaluation should be directed by clinical symptoms and signs, general guidelines are suggested in Table 34.4. Additional diagnostic evaluation may be necessary if preliminary evaluation suggests the presence of coexistent disease.

Prominent Cartilage Lower Ear
Figure 34.2. (A and B) Prominent degenerative changes of ear cartilage with perichondral scarring and lymphoplasmacytic inflammation.

Mortality in RP is most frequently due to complications of respiratory tract involvement, either respiratory collapse or infection. Other causes of death include cardiovascular involvement (vasculitis, aneurysm, or valve rupture), and malignancy (11). Ten-year survival in a group of 112 patients followed at one institution was 55%, but a more recent series of 66 patients had a survival rate of 94% with a mean follow-up of eight years (10,12). Anemia at the time of diagnosis predicts decreased survival. In patients less than 51 years old, saddle-nose deformity and systemic vasculitis are the worst prognostic signs, and in older patients, only anemia correlates with worse clinical outcomes (12).

The mainstay of treatment for RP is systemic cortico-steroids. Doses of 10 to 20 mg of prednisone per day may be sufficient. Higher doses are usually required for respiratory, audiovestibular, and ocular involvement (19). Low-dose colchicine and methotrexate may be useful as steroid-sparing agents. Nonsteroidal anti-inflammatory agents may help with mild flares of inflammation. Other treatments have included azathioprine, dapsone, cyclo-sporine, penicillamine, cyclophosphamide, minocycline, and plasma exchange (10). Recently, infliximab has been reported to be of benefit in two patients with refractory RP (21). A novel treatment using oral type II collagen as a tolerogen has been described in a child with RP (22). Adjunctive treatments may include airway stent placement, cardiac valve replacement, or surgical repair of aneurysms. Long-term follow-up is indicated because of a propensity for disease persistence, recurrences, and evolution of coexistent diseases.

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