Fung, JJ

Jain, A

Roat, MI

Starzl, TE

Thoft, RA

Thomson, AW

PubMed

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Treatment of Cogan's Syndrome With FK 506: A C~se Report M.1. Roat, A.A. Thoft, A.W. Thomson, A. Jain, J.J. Fung, and T.E. Starzi COGAN'S syndrome is characterized by ocular inflam-mation. most commonly interstitial keratitis. vestib-uloauditory symptoms. and (in about 50% of cases) sys-temic vasculitis. A recent review! cites the evidence for an autoimmune pathogenesis of this rare disease. This in-cludes the presence of lymphocytes in ocular and vestib-uloauditory tissue.2 and the detection of serum antibodies against antigens present in the cornea and inner ear. l Successful treatment can be achieved with corti coste-roids,4 while other immunosuppressive agents. including cyclophosphamide and cyclosporine (CyA),s have proved useful in severe cases. FK 506 is a powerful new immu-nosuppressive agent which shares many of the properties of Cy A. 0 including a very similar inhibitory effect on CD4'" T-Iymphocyte activation and proliferation. In clinical tri-als. it has proved highly effective in the prevention7 and reversals of organ allograft rejection, with a reduced inci-dence of rejection and fewer side effects compared with CyA. FK S06 also shows considerable promise for the treatment of certain autoimmune diseases.9-11 including disorders unresponsive to high-dose steroids. We report here on the successful use of FK 506 in the treatment of a patient with Cogan's syndrome. A 48-year-old Caucasian woman presented to the Ocular Immunology service at the Eye and Ear Institute with a 9-year history of ocular inflammation. She also had a 20-year history of hearing loss. She had lost all vision in the left eye from intractable glaucoma. which was second-ary to uncontrolled uveitis and scleritis. At the time of presentation. she had active, diffuse anterior scleritis. anterior uveitis. and interstitial keratitis of the right eye and mild arthralgias of the knees. She was on low-dose oral steroids (0.25 mgikg per day). Fluores-cence Treponima antibody absorption test. rheumatoid factor. antinuclear antibodies. and Lyme titer were nega-tive; and the complete blood count was normal. Audio-gram was consistent with severe. bilateral sensorineural hearing loss. She was diagnosed as having Cogan's syndrome and her oral steroids were increased to I mg/kg per day. Cardiol-ogy assessment revealed aortic insufficiency. She became dependent on prednisone to.S mg/kg per day) to control her ocular inflammation. As the steroid side effects accumu-lated. she entered our prospective. clinical trial comparing the efficacy and safety of oral FK 506 (0.15 mg/kg b.i.d.) with conventional immunosuppressive drugs for the treat-ment of ocular autoimmune disease. She was randomized to FK 506. Early during treatment. she experienced a pruritic papular rash on her chest. Her ocular inflammation began to improve in 3 weeks and was mostly resolved in 3 months. Over a 100month period. oral steroids were ta-pered and discontinued. She has had no progression of her hearing loss or recurrent arthralgias and has been free of ocular inflammation for 6 months. Only minimal systemic side effects of FK 506 (> 10 mg BID) have been observed. Specifically, there was a transient rise in creatinine to 158 mmoVL compared with a pretreatment level of 79 mmoVL. Renal function responded to a decrease in FK 506 dose; her current creatinine level is 106 mmoVL. No change in blood pressure. hepatic function. electrolytes. amylase. or blood glucose was observed. This case report demonstrates the powerful immunosup-pressive activity and safety of FK 506. It suggests that FK 506 may prove valuable in the management of this and other ocular inflammatory disorders. as well as showing promise for the treatment of other autoimmune diseases. REFERENCES I. Anonymous: Lancet 337:1011. 1991 2. Haynes BF. Kaiser-Kupfer Ml. Mason P. et al: Medicine <Baltimore' 59:426. 1980 3. Arnold W. Gebbcrs JO: Laryngol Rhinol Otol (Stuttgart) 63:428. 1984 4. Haynes BF. Pikus A. Kaiser-Kupfer M. et al: Arthrit Rheum 24:501. 1981 5. Allen NB. Cox CC. Cobo M. et al: Am J Med 88:296. 1990 6. Macleod AM. Thomson AW: Lancet 337:25.1991 7. Todo S. Fung JJ. Tzakis A. et al: Transplant Proc 23:1397. 1991 8. Stan! TE. Todo S. Fung JJ. et a1: Lancet ii: 1000. 1989 9. McCauley J. Tzakis AG. Fung JJ. et a1: Lancet 335:674. 1990 10. Murase N. Lieberman 1. Nalesnik M. et a1: Lancet 336:373. 1990 It. Jegasothy BY. Ackennan CD. Todo S. et al: Arch Derma-tol (in press' From the Department of Ophthalmology, Eye and Ear Institute. and DMsion of Transplantation. Department of Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania. Supponed in part by NIH grants EY06186 and P30 EY0898. Address reprint requests to Dr M.I. Roat. Eye & Ear Institute. 203 Lothrop Street. Pittsburgh, PA 15213. ~ 1991 by Appleton & Lange 0041-13451911$3.001 +0 Transplantation Proceechngs, Vol 23. No 6 (December). 1991: p 3347 3347 

Treatment of Cogan's syndrome with FK 506: A case report

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Treatment of Cogan's syndrome with FK 506: A case report

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