Treatment of Cogan's syndrome with FK 506: a case report.
Year: 1991
Type: article
Study design: Case Report
Abstract: OGAN'S syndrome is characterized by ocular inflam- mation.most commonly interstitial keratitis.vestibuloauditory symptoms.and (in about 50% of cases) systemic vasculitis.A recent review!cites the evidence for an autoimmune pathogenesis of this rare disease.This includes the presence of lymphocytes in ocular and vestibuloauditory 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 costeroids,4 while other immunosuppressive agents.including cyclophosphamide and cyclosporine (CyA),s have proved useful in severe cases.FK 506 is a powerful new immunosuppressive 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 trials.it has proved highly effective in the prevention 7 and reversals of organ allograft rejection, with a reduced incidence of rejection and fewer side effects compared with CyA.FK S06 also shows considerable promise for the treatment of certain autoimmune diseases.9-11including 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 secondary 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).Fluorescence Treponima antibody absorption test.rheumatoid factor.antinuclear antibodies.and Lyme titer were negative; and the complete blood count was normal.Audiogram 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.Cardiology assessment revealed aortic insufficiency.She became
Source: PubMed
Institution: University of Pittsburgh
Language: English
Subfield: Ophthalmology
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W314174061