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  • HHV8 associated Kaposi's sarcoma during triple immunosuppressive treatment with cyclosporin A, azathioprine, and prednisolone for ocular Behçet's disease and complete remission of both disorders with interferon α

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Case report
HHV8 associated Kaposi's sarcoma during triple immunosuppressive treatment with cyclosporin A, azathioprine, and prednisolone for ocular Behçet's disease and complete remission of both disorders with interferon α
  1. I Köttera,
  2. C Aepinusb,
  3. F Graeplerc,
  4. V Gärtnerb,
  5. A K Ecksteind,
  6. N Stübigere,
  7. B Kaskasc,
  8. M Zierhute,
  9. B Bültmannb,
  10. R Kandolfb,
  11. L Kanza
  1. aUniversity Hospital, Department of Internal Medicine, Haematology, Oncology, Immunology and Rheumatology, Otfried-Müller-Str 10, D-72076 Tübingen, Germany, bInstitute for Pathology, Liebermeisterstr 8, D-72076 Tübingen, cUniversity Hospital, Department of Internal Medicine, Gastroenterology, Infectious Diseases, Otfried-Müller-Str 10, D-72076 Tübingen, dUniversity Hospital, Department of Ophthalmology, Hufelandstr 55, Essen, eUniversity Hospital, Department of Ophthalmology, Schleichstr 12, 72076 Tübingen
  1. Dr Kötterina.koetter{at}med.uni-tuebingen.de

https://doi.org/10.1136/ard.60.1.83

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    Behçet's disease is a multisystem vasculitis with oral and genital aphthous ulcers, cutaneous vasculitis, uveitis, and arthritis as its main features.1 Ocular manifestations, especially panuveitis, have a poor prognosis, resulting in blindness in most patients after five years, irrespective of treatment. Recent reports have shown significant improvement of visual prognosis by early and aggressive immunosuppressive treatment.2 However, immunosuppressive treatment bears a risk of opportunistic infections, especially herpesvirus reactivation and increased incidence of malignancies.3

    Kaposi's sarcoma is a rare malignant tumour (incidence 0.01%) of endothelia and vascular smooth muscle cells which has a markedly increased incidence in patients with AIDS (0.9%)4 and iatrogenic immunosuppression (0.52%).5 Recently, human herpesvirus 8 (HHV8) was identified as the causative agent.

    We describe a patient with severe ocular Behçet's disease who developed disseminated, HHV8 positive Kaposi's sarcoma during triple immunosuppressive treatment with prednisolone, cyclosporin A, and azathioprine.

    Case report

    In 1990 HLA-B51 positive Behçet's disease was diagnosed in a 29 year old man of Turkish origin. He had a history of severe painful oral aphthosis. Panuveitis of both eyes with hypopioniritis was first diagnosed in June 1989. In July 1990 he was doing well while receiving 50 mg prednisolone daily. Clinical examination showed no signs of Behçet's disease, except ocular inflammation. No serological evidence of infection with HIV, Epstein-Barr virus (EBV), herpes simplex virus (HSV), and human cytomegalovirus (HCMV) was found. Cyclosporin A (5 mg/kg body weight orally daily) was added to prednisolone owing to occlusive retinal vasculitis. Because of relapses of bilateral uveitis with further loss of vision azathioprine (150 mg orally daily) was added in November 1992. This resulted in complete remission of ocular inflammation. In December 1993 the patient complained of epigastric pain, had lost 10 kg in weight, and was anaemic. Gastroscopy disclosed a patchy reddish induration of …

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    • Financial support: None.