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 #Relapsing #Polychondritis #rheumatology #diagnosis #management
