Relapsing Polychondritis



What is it?

Recurrent inflammation of the cartilage in the body (Autoimmune disorder)



Who?

• Most frequently: 40 and 60 years

• Even as young as 20 years

• Females slightly > Males

• Affects all ethnicities



Symptoms:

• Sudden onset of pain, tenderness, and swelling of the cartilage of one or both ears (Auricular chondritis is the most common clinical manifestation)

• + Nonspecific symptoms: Fever, weight loss, malaise, night sweats, and fatigue



Criteria:

• Recurrent bilateral auricular chondritis

• Inflammatory polyarthritis (Non-erosive)

• Nasal chondritis

• Ocular inflammation (conjunctivitis, keratitis, uveitis, scleritis)

• Tracheal chondritis

• Laryngeal chondritis (Change in voice)

• Cochlear damage - hearing loss

• Vestibular damage - dizziness, hearing loss, nausea



Inflammatory process may damage connective tissue components of:

• Heart

• Large vessels (aorta)

• Eyes

• Inner ear

• Skin

• Joints - Inflammation of joint cartilage (seronegative)

• Kidneys

• Other organs

Also manifest with:

• Costochondritis

• Hoarseness of voice

• Inflammation - glottis - dysphagia



Relapsing polychondritis usually spares the earlobe (Not always though!)

• Earlobe is typically not involved because it has no cartilage

• Can appear like "cauliflower ear"

• Violaceous discoloration

• Warm and swollen

• Episodic/Self-limiting



Associated with other immunologically mediated diseases 30% of cases such as:

• SLE

• RA - Most common

• Sjogren’s syndrome

• MAGIC syndrome

• Vexus syndrome - Associated with Polyarteritis Nodosa, GCA, Sweet syndrome, MDS & Multiple myeloma



Complications:

• Airway collapse or obstruction

• Deafness

• Loss of vision

• Aortic and other large vessel aneurysms

• Cardiac arrhythmia

• Heart failure

• Renal insufficiency

• Cognitive dysfunction



DDX:

• Sarcoidosis

• Behcet disease

• Infections

	○ Tuberculosis

	○ Leprosy

	○ Syphilis

	○ Fungal

	○ Bacterial

	○ Viral infection

• Lymphoma

• GPA/EGPA

• RA



Diagnosis:

• Clinical tests for (not sensitive or specific):

	○ Anti-Matrilin-1 Ab

	○ Antibodies to type II collagen

• Nonspecific ↑ ESR, CRP, ANA +

• Biopsy of affected tissue



Treatment:

• Corticosteroids: Prednisone



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