mycophenolic-acid and Polychondritis--Relapsing

Relapsing polychondritis (RP) is a very rare autoimmune disease characterised by a relapsing inflammation of the cartilaginous tissues (joints, ears, nose, intervertebral discs, larynx, trachea and cartilaginous bronchi), which may progress to long-lasting atrophy and/or deformity of the cartilages. Non-cartilaginous tissues may also be affected, such as the eyes, heart, aorta, inner ear and skin. RP has a long and unpredictable course. Because no randomised therapeutic trials are available, the treatment of RP remains mainly empirical. Minor forms of the disease can be treated with non-steroidal anti-inflammatory drugs, whereas more severe forms are treated with systemic corticosteroids. Life-threatening diseases and corticosteroid-dependent or resistant diseases are an indication for immunosuppressant therapy such as methotrexate, azathioprine, mycophenolate mofetil and cyclophosphamide. Biologics could be given as second-line treatment in patients with an active disease despite the use of steroids and immunosuppressive drugs. Although the biologics represent new potential treatment for RP, very scarce information is available to draw any firm conclusion on their use in RP.

Topics: Anti-Inflammatory Agents, Non-Steroidal; Autoimmune Diseases; Azathioprine; Biological Products; Cyclophosphamide; Humans; Methotrexate; Mycophenolic Acid; Polychondritis, Relapsing

Cardiovascular involvement occurring in 20-30% patients is the second most common cause of mortality in patients with relapsing polychondritis. Aortic insufficiency occurs as a result of aortic root dilatation rather than primary valvular involvement. We are reporting a patient of relapsing polychondritis with aortic root dilatation, in whom institution of early and aggressive therapy successfully prevented the progression of aortic insufficiency.

Topics: Aorta; Aortic Diseases; Aortic Valve Insufficiency; Azathioprine; Cyclophosphamide; Dilatation, Pathologic; Drug Substitution; Drug Therapy, Combination; Glucocorticoids; Humans; Immunosuppressive Agents; Male; Methylprednisolone; Middle Aged; Mycophenolic Acid; Polychondritis, Relapsing; Prednisolone

We report a 50 year old man who presented to our clinic with a 5 year history of intermittent bilateral ear pain and underwent 2 biopsies which revealed nonspecific findings. A diagnosis of relapsing polychondritis was made based on positive serum antibodies to type II collagen and a wedge biopsy which revealed areas of cartilage necrosis and focal areas of perichondral inflammation with lymphocytes and histiocytes. He was successfully treated with a prednisone taper and mycophenolate mofetil 3 g per day (increased from the initial dose of 2 g per day). During his last clinic follow up, 17 months after beginning mycophenolate mofetil, our patient had no subjective symptoms and objectively had no inflammation or pain to palpation. He was continued on prednisone 5 mg daily and mycophenolate mofetil 3 g per day.

Topics: Anti-Inflammatory Agents; Diagnosis, Differential; Drug Therapy, Combination; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Polychondritis, Relapsing; Prednisone