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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2004  |  Volume : 15  |  Issue : 1  |  Page : 57-60
Secondary Amyloidosis, a Fatal Complication of Behcet's Disease: Three Case Reports

Departments of Nephrology and Internal Medicine, Monastir Hospital, Monastir 5000, Tunisia

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Renal amyloidosis (RA) is known to be one of the many complications of Behcet's disease (BD) but its frequency has been often underestimated. In this paper, we report on three cases of RA in a series of 105 patients with BD. RA was clinically suspected in our patients by the presence of edema and/or hypotension. The nephrotic syndrome was present in all of them and one patient had associated renal failure. Renal biopsy showed amyloid deposits type AA in all cases.Our patients received Colchicine at doses of 1 mg per day but with disappointing results. Their prognosis was significantely impaired with our three patients dying at ten years, six months and four weeks after diagnosis of RA. We will try, through this clinical experience and based on the relevant literature to better understand this kind of morbid association.

Keywords: Behcet′s disease, Amyloidosis, Renal Failure.

How to cite this article:
Skhiri H, Mahjoub S, Harzallah O, Achour A, Bouraoui S, Frih A, Dhia NB, El May M. Secondary Amyloidosis, a Fatal Complication of Behcet's Disease: Three Case Reports. Saudi J Kidney Dis Transpl 2004;15:57-60

How to cite this URL:
Skhiri H, Mahjoub S, Harzallah O, Achour A, Bouraoui S, Frih A, Dhia NB, El May M. Secondary Amyloidosis, a Fatal Complication of Behcet's Disease: Three Case Reports. Saudi J Kidney Dis Transpl [serial online] 2004 [cited 2022 Aug 12];15:57-60. Available from: https://www.sjkdt.org/text.asp?2004/15/1/57/32967

   Introduction Top

Behcet's disease (BD) is a systemic vasculitis, affecting with an evident predilection, young males of Mediterranean, middle and far east Asian origin. Its diagnosis is made based on a group of clinical features established by the International Group for Behcet's Disease in 1990. The features typically include reccurrent oral and genital ulcers and ocular manifest­ations; however, this disease can affect other parts of the body such as articular, muscular, cardiovascular and neurological systems and to a less degree the digestive system and the kidney. Thus, renal involvement in BD is rare.

Amyloidosis represents one of its facets. We report here on three cases of renal amyloidosis (RA) in patients having BD.

   Material and Methods Top

Three patients among 105 (2.8 %) established cases of BD who developing the nephrotic syndrome during the follow-up period were included in this study [Table - 1]. They com­prised of two males and one female aged 24 to 46 years. Oral ulcers were the first manifest­ation of the disease.

The interval between the onset of the first symptom of BD and the biopsy diagnosis of amyloidosis ranged from 6 to 24 years. None of the patients had previous history of mediteranean fever. All patients had percu­taneous kidney biopsy. Tissue's specimens were examined by light and polarisation microscopy after staining by Congo red. Immunofluorescence study was performed.

   Results Top

At the time of the renal biopsy, two cases had normal and one had impaired renal function (serum creatinine of 800 µmol/l). Proteinuria ranged from 8 to 15 gm per 24 hours. The serum albumin value was variable between 7 to 22 gm/l. There was no micro­scopic hematuria. Serum cholesterol was elevated and the values were between 425 to 604 mg/dl. All biopsy specimens were positive with congo red and emerald-green birefringence was noted under polarisation microscopy. Two of these patients were started on treatment with colchicine 1 mg per day and acetyl salicylic acid 250 mg per day, which they took irregularly. The third patient recieved only actylsalicylic acid. One of them has received chloraminophene for ocular manifestations which was stopped because of medullar toxicity. All three patients died respectively one month, six months and 10 years after the diagnosis of amyloidosis.

   Discussion Top

Behcet's disease is a vasculitis described first by Hulusi in 1937. It is a multisystem disease which can affect nearly any part of the body. Renal involvment can be in the form of interstitial nephritis, renovascular thrombosis or glomerulonephritis of different histological types. Since first reported, only few cases of RA in BD have been des­cribed in literature and amyloidosis has been considered as an exceptional manifestation, although more recent reviews showe that this complication is not so rare as we believe. [1],[2],[3]

The evolution of the frequency of amyloi­dosis in BD through the two last decades, as well as its main features, is summarized in [Table - 2]. Rosenthal et al [1] described three cases of amyloidosis occuring in 77 cases of BD (3.8%).

A similar proportion has been observed in a series of 183 patients with BD. [2] In a Tunisian report, renal amyloidosis was present in 1.4% of all patients having BD [3] while in another report, only one case of renal amyloidosis has been described in a series of 162 cases of BD. [4]

In general, amyloidosis appears late after the onset of BD. This duration is variable and Ben Moussa, [3] reported on the occurrence of RA after a mean of 9.4 years (5-19 years) of the onset of BD while Tasdemir et al [2] reported a onset duration of 10 ± 3 years (4-16 years). A recent study of 718 cases of BD showed a delay of 8.1 years (9 -15 years). [5]

The diagnosis of amyloidosis in these patients is essentially made by the presence of heavy proteinuria and/or the nephrotic syndrome. Rarely, end-stage renal failure can be present at diagnosis of amyloidosis as described in our series and in Ben Moussa et al [3] and Utas' ones. [6]

A recent cumulative analysis [6] about the renal involvment in BD has reported on 159 cases of BD of whom 69 had amyloidosis.

The overall renal involvment is estimated to occur in 0-55% of all cases. [7] Males seem to be more affected by the renal complications of this vasculitis. The mean interval between the diagnosis of BD and amyloidosis seems to be shorter in men than in women. The nephrotic syndrome was present in 83% of all cases of amyloidosis and renal failure is quite frequent. The vascular manifestations of the disease were present in 60% of all patients with amyloidosis.

The analysis of the fibrillar deposits with potassium permenganate or monoclonal anti­bodies show amyloidosis in all cases. The relationship between BD and amyloidosis type AA is still debated. [8] The presence of cutaneous, articular and vascular inflamma­tory processes would be predisposing factors for amyloidosis.

The evolution of BD with amyloidosis, even if treated with colchicine, is complicated by the occurrence of events such as thrombo­embolic phenomena and/or renal failure often requiring hemodialysis. The overall prognosis in this context is dark. The follow-up of 23 patients showed 10 deaths in the three months following the diagnosis of amyloidosis. [8] A survival exceeding five years as in the case of one of our patients has been reported only in two patients. A global mortality of 50% is described after 3.4 years of evolution. Thus, amyloidosis in BD is a turning point in its evolution. The absence of an efficient thera­peutic drug mandates us to take preventive measures including vigorous treatment of inflammatory processes and practice moni­toring of proteinuria and plasmatic creatinine level at least once a year.

   References Top

1.Rosenthal T, Weiss P, Gafni J. Renal involvment in Behcet's syndrome. Arch Intern Med 1978;138:1122-4.  Back to cited text no. 1    
2.Tasdemir I, Sivri B, Turgan C, et al. The expanding specrtum of a disease. Behcet's disease associated with amyloidosis. Nephron 1989;52(2):154-7.  Back to cited text no. 2    
3.Ben Moussa F, Turki C, Ben Taarit C, Chaabouni C, Goucha R, Ben Maiz Hedi. Renal involvment in Behcet's disease. Proceedings of the seventh international conference on Behcet's disease.Tunis, October 10-11, 1996.  Back to cited text no. 3    
4.Filali Ansary N, Tazi Mezalek Z, Mohattane A, et al. Behcet disease 162 cases. Ann Med Interne 1999;150(3):178-88.  Back to cited text no. 4    
5.Melikoglu M, Altiparmak MR, Fresko I et al. A reappraisal of amyloidosis in Behcet's syndrome. Rheumatology 2001;40:212-15.  Back to cited text no. 5    
6.Utas C, Utas S, Oymak O, Kelestimur F. Behcet's syndrome presenting as end-stage chronic renal failure due to amyloidosis. Nephrol Dial Transplant 1995;10:1472-3.  Back to cited text no. 6    
7.Akpolat T, Akkoyunlu M, Akpolat I et al. Renal Behcet's disease : a cumulative analysis. Semin Arthritis Rheum 2002;31(5):317-37.  Back to cited text no. 7    
8.Akpolat T, Akpolat I, Kandemir B. Behcet's disease and AA-type amyloidosis. Am J Nephrol 2000;20(1):68-70.  Back to cited text no. 8    

Correspondence Address:
Habib Skhiri
Departments of Nephrology and Internal Medicine, Monastir Hospital, Monastir 5000
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Source of Support: None, Conflict of Interest: None

PMID: 18202468

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  [Table - 1], [Table - 2]


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