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Renal involvement is not frequent in Behçet’s syndrome (BS) and consists of occasional reports of patients having glomerulonephritis,1 IgA nephropathy2 and renal amyloidosis.3 We present the successful outcome of a renal transplantation in a patient who had end stage renal failure secondary to glomerulonephritis. To our knowledge, this is the first patient with BS to receive an organ transplantation.
The detailed history of this patient at the time of the diagnosis of glomerulonephritis was the subject of a case report in 1991.4 In brief, she was 21 years old when she developed recurrent oral and genital ulcers, bilateral uveitis, erythema nodosum, folliculitis, and intermittent arthritis of the knees. Two years later, she was referred to our centre for further evaluation of eye symptoms. She had no active mucocutaneous lesions at that time, the pathergy reaction was positive and she carried HLA B5. It was decided to prescribe only local drops for her mild eye involvement. Three months later she experienced two ocular episodes resulting in a sharp decline of visual acuity and azathioprine 2.5 mg/kg/day was prescribed. Two weeks later she was admitted to the hospital because of microscopic haematuria. She was ANA negative, the anti-DNA and serum complement levels were within normal range. Her glomerular filtration rate was 67 ml/min. An open renal biopsy showed diffuse proliferative glomerulonephritis and weak focal segmental positivity of IgA and IgM. She was treated with three boluses of 1 g methylprednisone and was discharged prescribed azathioprine 150 mg/day, aspirin 300 mg/day and prednisone 30 mg/day. She was well except for occasional mucocutaneous symptoms and a mild transient neurological episode during the next four years. However her renal function deteriorated progressively despite uninterrupted treatment with azathioprine and changing doses of prednisone and she was put on regular haemodialysis twice a week. In the 14th month of haemodialysis, she received a kidney from her mother. The graft function started immediately and she was prescribed maintenance immunosuppression with azathioprine, cyclosporin A and methylprednisolone. An acute interstitial type rejection on the 11th day of transplantation was treated successfully with pulsed corticosteroids. Now 40 months after transplantation, she has normal renal function and is free of any symptoms of BS except for occasional oral ulcers.
We had some hesitation in performing a renal transplantation in our patient initially because of the lack of any previous experience and particularly because of our concern for the heightened inflammatory response of BS patients to simple penetrating trauma that is best characterised by the pathergy reaction.5 This reaction, however, is not only limited to the skin and development of aneurysms after vascular punctures and episodes of synovitis after arthrocentesis have been observed.6 7 Furthermore, postoperative complications leading to a poor outcome such as occlusions of grafts/anastomoses after the surgical treatment of aneurysms8 or perivalvular leakage and suture breakdown after aortic valve replacement9 have been reported in BS patients. As these complications are probably related to the pathergy phenomenon reaction, you would also reasonably expect problems after an organ transplantation, an operation with arterial and venous anastomoses. On the other hand, we had previously shown that despite the increased inflammation, wound healing after full thickness skin punch biopsies is not changed in BS.10
We have not experienced any of the feared complications after the transplantation procedure in this instance. One reason for this favourable outcome might be that our patient was female. It is known that BS runs a milder disease course in women compared with men.11 Additionally, the rather intensive immunosuppressive/anti-inflammatory post-transplant drug use might also have contributed to the diminished disease activity of our patient as well as to the prevention of a reaction at the site of transplantation. Whatever it might be related to, the outcome in our patient suggests that BS patients can undergo renal transplantation with a satisfactory outcome.
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