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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT  
Year : 2015  |  Volume : 26  |  Issue : 1  |  Page : 132-134
Amyloidosis in Behcet's disease


Department of Internal Medicine, La Rabta Hospital, Tunis, Tunisia

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Date of Web Publication8-Jan-2015
 

   Abstract 

Behcet's disease (BD) is a multisystem vasculitis with protean manifestations. It is characterized by a heightened state of inflammation, although the factors that initiate and sustain this inflammation are not clear. We report some cases of BD-associated amyloidosis and have similar features. The patients developed nephrotic syndrome due to secondary amyloidosis, which was refractory to the immunosuppressive agents. Two patients expired and the third was lost to follow-up during the course. The BD complicated with amyloidosis is associated with high mortality despite the current aggressive therapy.

How to cite this article:
Ghorbel I B, Belfeki N, Salem T B, Lamloum M, Houman M H. Amyloidosis in Behcet's disease. Saudi J Kidney Dis Transpl 2015;26:132-4

How to cite this URL:
Ghorbel I B, Belfeki N, Salem T B, Lamloum M, Houman M H. Amyloidosis in Behcet's disease. Saudi J Kidney Dis Transpl [serial online] 2015 [cited 2021 Mar 4];26:132-4. Available from: https://www.sjkdt.org/text.asp?2015/26/1/132/148761

   Introduction Top


Behcet's disease (BD) is a multisystem vasculitis with protean manifestations. [1] It is characterized by a heightened state of inflammation, although the factors that initiate and sustain this inflammation are not clear. Secondary amyloidosis has been observed in BD. [2] We report some cases of BD-associated amyloidosis and have similar features.


   Case Reports Top


Case 1

A 26-year-old man had recurrent oral aphthae and genital ulcerations at the age of 18 years, followed in 1992 by an attack of iridocyclitis and in 1997 by bilateral thrombophlebitis extended to the inferior vena cava. Pathergy test was positive. The diagnosis of BD was made according to the criteria of the International Study Group for Behcet's Disease (ISG). [3] His medical therapy consisted of steroid therapy, anticoagulation treatment and colchicine. In January 1999, he presented with complaints of dyspnea and chest pain. Physical examination revealed a swelling of the arms and neck and venous collateral structures in the neck and anterior thoracic wall. Computed tomographic angiography revealed thrombosis of the axillary, the innominate veins and the superior vena cava. Uveitis sequela was confirmed on ophthalmologic examination. His medical therapy consisted of steroid therapy, anticoagulation and colchicine. In September 2000, he was sent to our department for a swelling of both legs. Pretibial edema and scrotal scars were observed in the physical examination. Laboratory analyses revealed the following: Erythrocyte sedimentation rate 140 mm/h, white blood cell count 5200/mm 3 , hematocrit 27%, platelet count 350,000/mm 3 , urea concentration 0.29 g/L, creatinine concentration 6 mg/L, albumin concentration 16 g/L, globulin concentration 3.9 g/L, total cholesterol concentration 3.48 g/L and triglyceride concentration 1.66 g/L. Daily proteinuria was 10 g. Histologic examination of the kidney biopsy specimen was diagnostic of amyloidosis. Treatment comprised pulsed intravenous cyclophosphamide 1 g monthly for three months for the severe vascular involvement, but, with the worsening of the nephrotic syndrome, we decided to treat him with chlorambucil. Unfortunately, there was no improvement and the patient died four months later from a renal and hepatic failure.

Case 2

A 28-year-old male patient presented in November 2002 with recurrent oral aphthae and genital ulcerations, positive pathergy test and uveitis. The vascular involvement consisted of a right auricular thrombus complicated by a pulmonary embolism. The diagnosis of BD was made. His medical therapy consisted of steroid therapy, anticoagulation treatment and colchicine. He also presented with pleural and pulmonary Staphylococci infection for three times during 2003. The patient presented in January 2005 with edema anasarca. Laboratory analysis revealed similar profile as case 1. Daily protein excretion was 20 g. A renal biopsy was not performed. A labial salivary gland biopsy showed amyloid A (AA) protein. Unfortunately, the patient refused hospitalization and was lost to follow-up.

Case 3

A 53-year-old male patient presented in 2003 with a similar picture as cases 1 and 2, and he was diagnosed as BD. The treatment comprised steroid therapy, pulsed intravenous cyclophosphamide 1 g monthly for six months followed by azathioprine 150 mg/day that he stopped in 2006. In August 2007, he presented with edema anasarca. His daily proteinuria was 10 g. Creatinine concentration and urea were within normal limits. Abdominal biopsy of subcutaneous fat tissue diagnosed amyloidosis. The patient was treated with cholorambucil and steroids. Unfortunately, there was no improvement and the patient died few months later from his complications.


   Discussion Top


BD is characterized by ocular, mucocutaneous, articular, neurological and vascular abnormalities. [4],[5] Although these features are often the most salient, BD can cause inflammation in almost any organ. Several important renal problems have been associated with BD, including glomerular and vascular involvements and complications of drug therapy. [6] Proliferative glomerulonephritis, IgA nephropathy, focal necrotizing glomerulonephritis and renal amyloidosis can associate with BD. [6],[7]

Amyloidosis in BD is an important but relatively rare complication. Recently, a cumulative analysis published in 2008 included 253 cases with BD and specific renal disease, of whom 108 had amyloidosis. [8] It is reported that the time to the onset of amyloidosis after the onset of disease was nine years (range 5-12 years) and the mean time to the detection of proteinuria after the onset of BD in 14 patients with amyloidosis was 8.1 years (range 3-15 years). [2]

In our cases, the mean time of the onset of amyloidosis was eight years in the first case, two years in the second case and four years in the third case. We did not find that the disease duration affected the occurrence of amyloidosis.

The presence of genital ulceration, thrombophlebitis, arthritis, a positive pathergy test, uveitis and neuropsychiatric involvement was more frequent among patients with BD and amyloidosis than among patients without amyloidosis. [6],[8] These conclusions are not shared by Kutlay et al, who reported a development of amyloidosis in BD with isolated mucocutaneous involvement. [9]

Akpolat reviewed, in 2008, 39 new cases of BD with amyloidosis, and the epidemiologic analysis revealed that 97% of this population was from the Middle East and Mediterranean countries (Turkey, 33; Morocco, 3; Iran, 2) and only one case from Japan. [8] This is compatible with previous data that implicate genetic/ environmental factors in the pathogenesis of amyloidosis, [6] such as MEFV (gene for familial Mediterranean fever) mutations and serum amyloid A (SAA) protein gene polymorphism. The frequency of MEFV mutations among 19 patients having BD-related amyloidosis was 32% higher than the reported frequency of MEFV mutations in BD, but it was not statistically significant. [10] The SAA1 gene is associated with AA-type amyloidosis and Utku demonstrated that SAA1 α/α geno-type was a risk factor for amyloidosis in BD. [11]

Amyloidosis is one of the prognostic factors affecting survival. BD with amyloidosis had a mortality rate of around 50%. [2] The use of immunosuppressive or colchicine before or after the diagnosis of amyloidosis did not seem to affect the clinical course of amyloidosis. Indeed, there is no evidence that colchicine prevents and/or improves the clinical manifestations of amyloidosis associated with BD, [12] as is the case with other forms of secondary amyloidosis. [13]

Conflict of Interest: None

 
   References Top

1.
Davtchi F, Shahram F, Chams-Davatchi C, et al. Behcet's disease: From East to West. Clin Rheumatol 2010;29:823-33.  Back to cited text no. 1
    
2.
Melikoglu M, Altiparmak MR, Fresko I, et al. A reappraisal of amyloidosis in Behcet's syndrome. Rheumatology 2001;40:2125-5.  Back to cited text no. 2
    
3.
International study group for Behcet's disease: Criteria for diagnosis of Behcet's disease. Lancet 1990;335:1078-80.  Back to cited text no. 3
    
4.
Yazici Y, Yurdakul S, Yazici H. Behcet's syndrome. Curr Rheumatol Rep 2010;12:429-35.  Back to cited text no. 4
    
5.
Houman MH, Neffati H, Braham A, Harzallah O, Khanfir M, Miled M, Hamzaoui K. Behçet's disease in Tunisia. Demographic, clinical and genetic aspects in 260 patients. Clin Exp Rheumatol 2007;25(4 Suppl 45):S58-64.  Back to cited text no. 5
    
6.
Akpolat T, Akkoyunlu M, Akpolat I, Dilek M, Odabas AR, Ozen S. Renal Behcet's Disease: A cumulative analysis. Semin Arthritis Rheum 2002;31:317-37.  Back to cited text no. 6
    
7.
Fernandes PF, Júnior GB, Barros FA, Sousa DC, Franco LM, Patrocínio RM. Behçet's disease and IgA nephropathy: Report of this association in a patient from Brazil and literature review. Invest Clin 2006;47:405-11.  Back to cited text no. 7
    
8.
Akpolat T, Dilek M, Aksu K, et al. Renal Behcet's Disease: An update. Semin Arthritis Rheum 2008;38:241-8.  Back to cited text no. 8
    
9.
Kutlay S, Civriz S, Ensari A, Nergizoglu G, Ates K, Karatan O. Development of amyloidosis in Behçet's syndrome with isolated mucocutaneous involvement. Rheumatol Int 2004;24:37-9.  Back to cited text no. 9
    
10.
Akpolat T, Yilmaz E, Ozdogan H, Melikoglu M, Altiok E, OzenS. Do MEFV mutations play a role in the development of Behcet's disease related amyloidosis? Clin Exp Rheumatol 2005;23:273-4.  Back to cited text no. 10
    
11.
Utku U, Dilek M, Akpolat I, Bedir A, Akpolat T. SAA1 alpha/alpha alleles in Behcet's disease related amyloidosis. Clin Rheumatol 2007;26:927-9.  Back to cited text no. 11
    
12.
Yurdakul S, Tuzuner N, Yurdakul I, Hamuryudan V, Yazici H. Amyloidosis in Behcet's syndrome. Arthritis Rheum 1990;33:1586-9.  Back to cited text no. 12
    
13.
Livneh A, Zemer D, Langevitz P, Shemer J, Sohar E, Pras M. Colchicine in the treatment of AA and AL amyloidosis. Semin Arthritis Rheum 1993;23:206-14.  Back to cited text no. 13
    

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Correspondence Address:
Dr. I Ben Ghorbel
Department of Internal Medicine, La Rabta Hospital, Tunis
Tunisia
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DOI: 10.4103/1319-2442.148761

PMID: 25579733

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