Orthopaedic MD

Polychondritis aka/or Relapsing polychondritis aka/or Myenberg-Altherr-Uehlinger syndrome may cause or feature - Symptoms and Signs Arthropathy Conductive hearing loss Episcleritis Epistaxis Erythema nodosum Saddle nose deformity Stridor Uveitis Cardiac and vascular conditions Aneurysms Aortic valve incompetence Mitral valve incompetence Inflammatory conditions Scleritis Vasculitis Trauma, mechanical and physical conditions Tracheal stenosis belong(s) to the category of Autoimmune diseases Polychondritis: Definition(s) via UMLS..... "An acquired disease of unknown etiology, chronic course, and tendency to recur. It is characterized by inflammation and degeneration of cartilage and can result in deformities such as floppy ear and saddle nose. Loss of cartilage in the respiratory tract can lead to respiratory obstruction."

Author: Nicholas L Compton, MD, Staff Physician, Department of Internal Medicine, University of Washington Medical Center
Contents Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Pictures Bibliography
Synonyms and related keywords: relapsing polychondritis, RP, cartilaginous inflammation, cartilage inflammation, inflamed cartilage, inflamed ear, ear inflammation, inflamed nose, nose inflammation, inflamed laryngotracheobronchial tree, laryngotracheobronchial tree inflammation, airway chondritis, infection secondary to corticosteroid treatment, respiratory compromise, systemic vasculitis, auricular chondritis, seronegative arthritis, non-nodular arthritis, nonnodular arthritis, respiratory tract chondritis, audiovestibular damage, audio-vestibular damage, aortic arch syndrome, abdominal aortic aneurysm, aortic regurgitation, chondrolysis, chondritis, perichondritis

Polychondritis is a rare degenerative disease characterized by recurrent inflammation of the cartilage in the body. Deterioration of the cartilage may affect any site of the body where cartilage is present. Ears, larynx and trachea may become "floppy," and the bridge of the nose can collapse into a "saddlenose" shape. The aortic heart valve may also be affected.

Polychondritis is an auto-immune disease in which the human's body's immune system begins to attack and destroy the cartilage tissues in the body. Wikipedia

Relapsing Polychondritis (RPC) is a rare episodic and progressive multisystem inflammatory rheumatic disease that can be life-threatening, debilitating and can be difficult to diagnose. It was first described in 1923 and is characterized be recurrent, potentially severe and frightening episodes of inflammation of cartilaginous tissues. RPC affects cartilage in multiple systems, such as the ears, nose, larynx, trachea, bronchi and joints. In addition, it can affect proteoglycan rich tissues, such as the eyes, aorta, heart and skin. The classic hallmarks of RPC are repeated episodes of chondritis (inflammation of cartilage) involving the ear, nose and trachea. Inflammation at these sites may lead to cartilage destruction and result in deformity of the ears, the nose, and obstruction of the trachea which leads to difficulty breathing. Other problems include arthritis, eye inflammation, hearing and balance disturbances, vasculitis, endocrine system, skin disorders and cranial nerve or brain involvement.

Patient Support Group Our mission is to provide a gathering place for those afflicted with Polychondritis and also a place of support for their family members. Our goal is to join the world together in a complete understanding of Relapsing Polychondritis. Only when there is understanding, can there be a hope for a cure!

Baylor College of Medicine Grand Rounds Archive. Review of the subject and a case presentation from the Otolaryngologic point of view

Relapsing polychondritis is an uncommon, multisystem disease that can be life-threatening, debilitating, and difficult to diagnose. It is characterized by recurrent, potentially severe, and frightening episodes of inflammation of cartilaginous tissues. All types of cartilage may be involved, including the elastic cartilage of the ears and nose, the hyaline cartilage of peripheral joints, the fibrocartilage at axial sites, and the cartilage in the tracheobronchial tree. Relapsing polychondritis can also inflame other proteoglycan-rich structures, such as the eye, heart, blood vessels, and inner ear. Systemic symptoms (for example, fever, lethargy, and weight loss) are common, and vasculitis affecting skin or internal organs may occur. Patients can present with a wide array of painful symptoms that often pose major diagnostic dilemmas. Because no specific tests for it are available, relapsing polychondritis must be diagnosed on clinical grounds. Pathogenetically, a linkage to HLADR4 and, occasionally, other autoimmune disease suggests that antiself reactions may be operative. Ann Intern Med 1998; 129:114-122. David E. Trentham, MD, and Christine H. Le, MBBS, FRACP