Fact-sheet: Relapsing polychondritis


Updated on 04/19/2018 at 7:58 AM

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Definition

Rare autoimmune disease (3.5 per million) causing destruction of the cartilage of the Ear, nose, and laryngotracheal tree

Diagnosis often delayed (mean delay: 3 years)

Most often in adults between 40 and 50 years of age

Death from respiratory complications in 30% of cases

Unknown etiology

Clinical features

Chondritis:

  • of the auricle (85% of cases), the most specific manifestation. In the acute stage, it presents as a unilateral or bilateral, warm, red or violaceous swelling, painful both spontaneously and on the slightest contact. The entire cartilaginous portion of the Ear may be affected. In contrast, the lobule, which is non-cartilaginous, is always spared, which distinguishes chondritis from an infectious process.
  • nasal chondritis (65% of cases) presents in the acute stage as inflammatory nasal swelling, rarely accompanied by mucus or blood discharge.
  • of the respiratory tract, less frequent (55% of cases) but potentially severe, occurring more often in women. Involvement of the respiratory tract is often diffuse. It may be asymptomatic and detected only by pulmonary function tests. Chondritis of the larynx and trachea is almost always accompanied by clinical signs. Tracheal collapse (chondromalacia: softening of the walls that narrows the airway, particularly during expiration) can be sudden and lead to fatal respiratory arrest.
  • of the laryngeal cartilages: spontaneous or palpation-induced pain, and hoarseness or voice loss, which should not be dismissed as trivial.

Extra-chondritic manifestations:

  • during severe flares: fever, anorexia, weight loss
  • rheumatologic (70-85%): arthralgia, intermittent, asymmetric, migratory polyarthritis
  • ENT (40%): sensorineural hearing loss, peripheral vestibular syndromes
  • ocular (60%): episcleritis, scleritis, and conjunctivitis (red, painful eye)
  • cardiovascular (20%): valvular disease (Aortic regurgitation), rhythm and conduction disturbances, aneurysms (thoracic aorta), stenosis of the great arterial trunks
  • dermatologic (20 to 40% of cases): vasculitis
  • renal (rare)
  • neurologic (rare)

Laboratory findings

Marked elevation of C-reactive protein (CRP), hyperfibrinemia, inflammatory anemia, and elevated white blood cell count (neutrophilic leukocytosis), myelodysplastic syndrome

A normal erythrocyte sedimentation rate should not rule out the diagnosis.

Autoimmune serology (limited): anti-type II collagen antibodies (very low specificity), anticartilage antibodies by indirect immunofluorescence (low sensitivity)

CT

  • diffuse thickening of the tracheal wall sparing the posterior wall +++
  • bronchiectasis (25% of cases): consequence of superinfections or cartilaginous destruction
  • bronchomalacia with expiratory air trapping (50% of cases)
  • ascending aortic dilation
  • chondritis: 2 phases (one or more inflammatory flares followed, in some cases, by permanent atrophy of the cartilaginous structures)

Management

Severe forms (laryngeal and/or tracheobronchial chondritis, systemic vasculitis): corticosteroid therapy

Minor forms: nonsteroidal anti-inflammatory drugs, dapsone, occasionally colchicine.

+/- low-dose corticosteroid therapy

Classification

McAdam diagnostic criteria = Presence of at least three of six criteria:

  • Bilateral auricular chondritis
  • Seronegative non-erosive polyarthritis
  • Nasal chondritis
  • Ocular inflammation
  • Respiratory tract chondritis
  • Vestibulocochlear involvement

Damiani and Levine diagnostic criteria = Presence of at least one of the following:

  • Presence of three McAdam criteria
  • Presence of one McAdam criterion and a typical histologic finding
  • Presence of two McAdam criteria and response to corticosteroids or dapsone

Differential diagnosis

Auricular chondritis:

  • Acute infection (Cellulitis),
  • Chondrodermatitis nodularis helicis (auricular lesion,
  • Chronic infection (tuberculosis, Leprosy, syphilis), circumscribed and less painful,
  • Various physical trauma (boxing, rugby, piercing),
  • Cystic chondromalacia of the Ear (painless swelling, chilblains, burns) of the auricle,
  • Auricular neoplasms (squamous cell carcinoma, Wegener's disease and basal cell carcinoma)

Nose:

  • Systemic lupus erythematosus
  • Nasal chondritis,
  • Wegener's disease,
  • Lymphomas and other neoplasms, saddle nose,
  • Chronic intranasal cocaine abuse,
  • Chronic infection (Leprosy, syphilis),
  • Post-traumatic (nasal fracture, septoplasty), scleritis,

Other sites:

  • Rheumatoid arthritis (rheumatoid vasculitis),
  • Systemic lupus erythematosus,
  • Wegener's disease,
  • Sarcoidosis,
  • Other vasculitides (Microscopic polyangitis, Churg-Strauss syndrome, inflammatory bowel disease, Polyarteritis nodosa),
  • Infection (shingles, Herpes simplex, Lyme disease, tuberculosis, syphilis),
  • Behçet's disease,
  • Idiopathic (50% of cases)
  • Airway stenosis: Post-traumatic (intubation), respiratory Sarcoidosis, Neoplastic (papillomas, cancers, lymphomas), Rhinoscleroma (infection by Klebsiella rhinoscleromatis), Wegener's disease, Pemphigus vulgaris, saber-sheath trachea, Cicatricial pemphigoid, chronic obstructive pulmonary disease), Laryngeal amyloidosis, Chronic granulomatous infection (tuberculosis, Tracheopathia osteoplastica, Histoplasmosis)