phenylephrine-hydrochloride and Aortic-Valve-Insufficiency

Relapsing polychondritis is a rare systemic disease. Clinical manifestations are variable and the condition is frequently associated with other diseases. Chondritis typically involves ears, nose, costal cartilages and upper airways. Other classical features include scleritis and episcleritis, mucocutaneous lesions, arthralgia and constitutional symptoms. Repeated inflammation of cartilaginous structures may lead to deformities of the ears, the nose and the airways. Tracheobronchomalacia and aortitis of the ascending portion resulting in aortic regurgitation or aneurysm are feared complications. Treatment is mainly based on systemic corticosteroids alone or in association with immunosuppressants. Several biological immunosuppressive agents are reported effective in refractory disease.

Topics: Adrenal Cortex Hormones; Aneurysm; Anti-Inflammatory Agents, Non-Steroidal; Aortic Valve Insufficiency; Aortitis; Diagnosis, Differential; Drug Therapy, Combination; Ear, External; Humans; Immunosuppressive Agents; Laryngeal Diseases; Nose; Polychondritis, Relapsing; Scleritis; Skin; Tracheal Diseases; Tracheobronchomalacia; Treatment Outcome

Relapsing polychondritis (RP) is a rare disease characterized by auricular, nasal and respiratory tract chondritis, ocular inflammation, inflammatory polyarthritis and cardiovascular abnormalities. Here we describe a patient with a five-year history of mild nasal and auricular chondritis, which suddenly developed into a severe aortic insufficiency with rest dyspnea. The pathogenesis and the management of this rare connective tissue disease are discussed.

Topics: Adult; Aortic Valve Insufficiency; Dyspnea; Ear, External; Heart Block; Humans; Male; Nose; Polychondritis, Relapsing; Tachycardia; Ultrasonography

Topics: Aorta, Thoracic; Aortic Valve Insufficiency; Cartilage Diseases; Ear; Humans; Male; Middle Aged; Nose; Polychondritis, Relapsing; Urethritis