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Ask the Experts on . . .

Etanercept for Relapsing Polychondritis

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Question

Are there any reports of effectiveness of etanercept (Enbrel) or any other

disease-modifying antirheumatic drug (DMARD) in a patient with relapsing

polychondritis with tracheomalacia? I have a patient who has been

steroid-dependent (about 20 mg daily) for 10 years and refractory or

intolerant to methotrexate, azathioprine, dapsone, mycophenolate mofetil, and

minocycline.

Response

from Terkeltaub, MD, 08/25/00

Laryngotracheobronchial complications of relapsing polychondritis are

potentially serious (and possibly fatal) and can present difficult

pharmacologic and nonpharmacologic management challenges.[1] Because

relapsing polychondritis is uncommon, and because of its frequently

unpredictable course, there are few useful long-term treatment studies using

DMARDs in large numbers of patients. In a recent review, Trentham and Le[2]

cited that in their own experience, methotrexate was effective in 23 of 31

patients with relapsing polychondritis. Their average dose, 17.5 mg per week,

was at the high end of typical pulse weekly oral methotrexate regimens.

However, they reported an average reduction of prednisone dosage from 19 mg

per day to 5 mg per day in association with methotrexate use in their

patients.

You do not mention cyclosporine, which some groups have anecdotally reported

to be efficacious in certain cases of relapsing polychondritis. I am not

aware of published experience with etanercept in relapsing polychondritis. I

suspect you posed this question because etanercept is under formal

investigation as a potential means to help stabilize the course of patients

with Wegener's granulomatosis. It will be interesting to see the results of

that line of investigation. However, such results may not be directly

applicable to relapsing polychondritis, which recent studies suggest to have

a unique immunologic pathogenesis that could include immune responses to the

cartilage matrix protein matrilin-1 (which is uniquely expressed in tracheal,

auricular, and nasal cartilages of adults).[3,4]

References

Tillie-Leblond I, Wallaert B, Leblond D, et al. Respiratory involvement in

relapsing polychondritis: clinical, functional, endoscopic, and radiographic

evaluations. Medicine. 1998;77:168-176.

Trentham DE, Le CH. Relapsing polychondritis. Ann Intern Med.

1998;129:114-122.

Hansson AS, Heinegard D, Holmdahl R. A new animal model for relapsing

polychondritis, induced by cartilage matrix protein (matrilin-1). J Clin

Invest. 1999;104:589-598.

Buckner JH, Wu JJ, Reife RA, Terato K, Eyre DR. Autoreactivity against

matrilin-1 in a patient with relapsing polychondritis. Arthritis Rheum.

2000;43:939-943.

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