ABSTRACT

Cogan’s syndrome (CS) is a rare chronic autoimmune vasculitis that can have significant systemic consequences and progress with progressive and inflammatory eye (interstitial keratitis, scleritis, episcleritis, uveitis) and audiovestibular (sensorineural hearing loss, vertigo, tinnitus) involvement. Vasculitis is observed in approximately 15% of patients with CS and may present as aortitis, large-vessel vasculitis (Takayasu-like), moderate arteritis, or other vasculitic syndromes. In this case report, we present the development of severe aortic and mitral valve insufficiency in a patient diagnosed with CS who was admitted to the hospital with episcleritis and sensorineural hearing loss.

INTRODUCTION

Cogan’s syndrome (CS) is a rare chronic autoimmune vasculitis that can have significant systemic consequences and progress with progressive and inflammatory eye (interstitial keratitis, scleritis, episcleritis, uveitis) and audiovestibular (sensorineural hearing loss, vertigo, tinnitus) involvement (1). Vasculitis is observed in approximately 15% of patients with CS and may present as aortitis, large-vessel vasculitis (Takayasu-like), moderate arteritis, or other vasculitic syndromes.

In this case report, we present the development of severe aortic and mitral valve insufficiency in a patient diagnosed with CS who was admitted to the hospital with episcleritis and sensorineural hearing loss. Written and verbal consent was obtained from the patient and his relatives.

CASE REPORT

A 44-year-old female patient was admitted to our clinic with progressive bilateral sensorineural hearing loss and episcleritis for approximately two years. The patient had increased dyspnea, exertional dyspnea, and high acute-phase responses in recent years. In the patient's examination. Blood pressure was 140/90 mmHg, hear beat was 110 beats/min, and breath rate was 18 beats/min. Physical examination revealed no features other than a decrease in breath sounds in the right lung base. Cardiological examination of the patient revealed findings consistent with severe aortic and mitral valve insufficiency. CS was considered in the foreground in the patient. The patient's treatment was started with methylprednisolone at a dose of 0.5 mg/kg/day and methotrexate at a dose of 15 mg/week. Heart valve replacement was then performed via cardiovascular surgery because of severe valve insufficiency. Histopathological findings consistent with CS were also observed in the pathological examination of the aortic root (Figure 1 and 2). Because of the lack of adequate clinical and laboratory responses in the patient, whose cardiac function improved significantly during follow-up, azathioprine at a dose of 3 mg/kg/day was added to the treatment. Severe stenosis was found in the left renal artery during the evaluation of the patient who developed intermittent hypertensive attacks and continued acute phase elevation during follow-up. Infliximab (5 mg/kg/4 weeks) was added to the treatment regimen. Currently, the patient is still under treatment with infliximab to prevent disease relapse.

Tumor necrosis factor-alpha (TNFa) is a cytokine released during infection and inflammation. It is mainly produced by activated macrophages. However, other cell types have also been shown to produce TNFa at various stages of inflammation (2). Other cells known to produce TNFa include lymphocytes and even fibroblast and endothelial cells. In recent years, anti-TNFa drugs have been evaluated for systemic vasculitis and different results have been obtained. Disease Modifying Anti Rheumatic Drug (DMARD) treatments used with varying success rates include cyclophosphamide, methotrexate, cyclosporine, mycophenolate mofetil, and azathioprine (3, 4). As biological DMARD treatments, infliximab therapy has an important potential therapeutic area when used in combination with corticosteroids. When the literature is reviewed, there are CS patients with severe systemic involvement treated with infliximab. Our patient had corticosteroid and methotrexate treatments until aortic insufficiency and cardiac surgery. Following aortic pathological evaluation, azathioprine and then infliximab were added to the patient's treatment. The patient's infliximab treatment was given every four weeks for a total of six months.

In previous studies, aortic valve replacement was required in almost half of the patients diagnosed with CS. Permanent vascular damage can be prevented by early diagnosis of the disease and use of other immunosuppressive agents, in addition to high-dose systemic glucocorticoid therapy to be started urgently (5). Severe aortic insufficiency develops approximately 3 years after diagnosis (6). In the present case, the development of valve insufficiency requiring aortic and mitral valve replacement in the first two years after hearing loss, early diagnosis, and effectiveness of TNF inhibitor therapy in appropriate cases are emphasized (7, 8).