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Saudi Journal of Kidney Diseases and Transplantation
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ORIGINAL ARTICLE Table of Contents   
Year : 2009  |  Volume : 20  |  Issue : 4  |  Page : 618-622
Renal involvement in behcet's disease

1 Department of Nephrology, Tabriz University of Medical Sciences, Tabriz, Iran
2 Department of Rheumatology, Tabriz University of Medical Sciences, Tabriz, Iran
3 Liver and Gastrointestinal Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
4 Tuberculosis and Lung Disease Research Center, Tabriz University of Medical Sciences, Tabriz, Iran

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Date of Web Publication8-Jul-2009


There are conflicting reports about the renal involvement in Behcet's disease (BD). In this study we aimed to study the frequency and type of renal involvement in a group of patients with BD in Azerbaijan province that is one of the prevalent areas of BD in Iran. All cases of BD were prospectively followed between June 2004 and January 2007, and evaluated for renal dys­function (serum creatinine > 1.7 mg/dL), glomerular hematuria and proteinuria. Those patients with proteinuria > 500 mg/day and serum creatinine level > 2 mg/dL, underwent renal biopsy. From a total number of 100 patients, six patients (6%) had obvious renal involvements. Four patients had glome­rular hematuria and proteinuria. Renal biopsy in two of them revealed measangial proliferative glumerulonephritis with IgA deposit in one of them and membranoproliferative glumerolonephritis in another one. Two remaining patients had serum creatinine > 2 mg/dL without any hematuria or proteinuria. Serologic study for viral agents and collagen vascular disease were negative in all patients with renal involvements. In conclusion, renal involvement in BD is not infrequent, although in most cases it is mild in nature and may be missed.

Keywords: Behcet′s Disease, Glomerulonephritis, Hematuria

How to cite this article:
Ardalan MR, Sadreddini S, Noshad H, Ebrahimi A, Molaeefard M, Somi MH, Shoja MM. Renal involvement in behcet's disease. Saudi J Kidney Dis Transpl 2009;20:618-22

How to cite this URL:
Ardalan MR, Sadreddini S, Noshad H, Ebrahimi A, Molaeefard M, Somi MH, Shoja MM. Renal involvement in behcet's disease. Saudi J Kidney Dis Transpl [serial online] 2009 [cited 2022 Jan 16];20:618-22. Available from: https://www.sjkdt.org/text.asp?2009/20/4/618/53251

   Introduction Top

Behcet's disease (BD) is a multi system disor­der that affects younger populationfrom Medi­terranean, the Middle East and Far East region connected with ancient Silk Road. Its major ma­nifestations are recurrent oral and genital ulcers and uveitis. Central nervous system disturbances, thrombophlebitis, large-vessel vaculitis and myo­carditis could occur less frequently. There are conflicting reports about the renal involvement in BD. Four decades ago in 1963, Oshima et al first reported the occurrence of minor proteinu­ria and hematuria in 13 of 65 patients with BD. [1] Twelve years later it was claimed that BD does not involve the kidney. [2] But shortly thereafter Rosenthal et al showed that it is not rare, al­though in most instances it is mild in nature. [3] ,[4] In 1980 the diagnostic criteria of Zhang inclu­ded urinary problems including bladder ulcera­tion, hematuria and epididymitis as minor cri­teria of BD. [5] The finding of severe renal in­volvement in BD led to histological study of the kidney by Hermann et al in 1982. [6]

In most of the later reports, transient asymp­tomatic hematuria and proteinuria was the main presentation. [7] Despite of the weak recognition several important renal problems have been as­sociated with BD including; glomerular and vas­cular involvements and complications of drug therapy. [3] ,[8]

Proliferative glomerulonephritis, IgA nephro­pathy, focal necrotizing glomerulonephritis and renal amyloidosis are the reported findings in BD. [8] The aim of this study is to determine the frequency, clinical and pathologic features of renal involvements in a group of patients with BD from Azerbaijan province in North West of Iran where BD is most prevalent. [9]

   Materials and Methods Top

In this prospective study during a period of three years (June 2004 and January 2007), all cases of BD who had the International criteria and were under the follow up in outpatients rheumatologic clinic of Tabriz University of medical science were included. Following in­formation were recorded, including: gender, age, medications, history of renal problem and dura­tion and type of BD. Urine analysis and serum creatinine levels were measured in each indi­vidual during their regular follow up. Patients with a serum creatinine level of greater than 1.7 mg/dL or those with dipstick proteinuria or hematuria underwent further evaluation including; urine examination for dysmorphic red blood cells, urine culture, twenty four hours urine collection for quantifying proteinuria, renal and bladder ultrasound for measuring the renal size and ru­ling out any renal or bladder stones. In those with glomerular hematuria and greater than 250 mg/24 hours proteinuria, serologic examination for hepatitis B virus (HBV), Hepatitis C virus (HCV) and HIV, Autoimmune workup inclu­ding: ANA, dsDNA, complements, ANCA, an­tiphospholipid antibodies, cryoglubulins and anti­basement membrane antibody were performed. In those with accelerated and malignant hyper­tension, flank pain, worsening proteinuria, and renal size asymmetry; Doppler Ultrasound stu­dy for possible renal artery or renal vein throm­bosis was performed. Echocardiography was also performed to rule out endocarditis as a possible cause of secondary glomerulonephritis. A com­plete history of medications was also obtained.

Renal biopsy was considered in those with active urine sediment including dysmorphic red blood, red blood cell casts, 24 hours protein ex­cretion greater than 500 mg/day and serum crea­tinine levels greater than 2 mg/dL.

   Results Top

A total of 103 Behcet patients were enrolled in this study. Two of them withdrew consent at the start and another during the study. In the re­maining 100 patients (M/F 63/37 age: 18-48 years) in 68 patients (68%) had oral and genital aphthous lesion as the main presentation of BD, 27 (27 %) patients had ocular BD, and 5 pa­tients (5%) had various degree of neurological involvements. Glomerular hematuria [Figure 1], proteinuria or renal function impairment were detected in 6 (6%) patients (5M, 1F) only. [Ta­ble 1] A. None of them had any history of mac­roscopic hematuria and the patients and their physicians were unaware about their kidney problems. Urine culture was negative in all these six patients and there was no evidence of renal or bladder stones on ultrasound examination.

Two patients had an elevated serum creatinine levels, without hematuria or proteinuria [Table 1] A: patient no. 5 and 6). One of them (patient 5) was receiving cyclosporine for treatment of ocular-BD (uveitis) for more than one year with a high cyclosporine blood level of 250 ng/mL. The second patient [Table 1] A, patient 6) had a history of oral and genital aphthous ulcer and right knee arthritis and was taking prednisolone and Azathioprine. Ultrasound in this patient re­vealed small left kidney and increased echoge­nicity of right kidney. Doppler ultrasound in this patient was negative for left renal artery stenosis.

Autoimmune workup was negative in all except patient no. 4, who had depressed C3 and CH50 levels [Table 1] B. No evidence was endocarditis was seen on echocardiographic study.

None of our patients had any history of peri­pheral arterial or vein thrombosis, or pulmonary embolism or any recent history of exacerbating or malignant hypertension.

Renal biopsy was performed in two patients [Table 1], patient no. 3 and 4). Light microscopic and immunofluresent studies of renal biopsy disclosed mesangial proliferative glomeruloneph­ritis and a mild granular mesangial deposit of C3 (+1) and more prominant IgA (+3) deposit in in one patient [Table 1] A, patient 3 and [Figure 2].

Membranoproliferative glumerolonephritis with a granular mesangeal deposit of C3 (+2) and IgM (+2) were found in the other patient [Table 1] A, patient 4 and [Figure 3]. Based on the biopsy findings the first patient received angiotensin receptor blockers, angiotensin converting enzyme inhibitors and prednisolone and the other was prescibed intravenous cyclophosphamide pulse (500 mg monthly/for six months). None of these patients with renal involvement reached an end stage renal failure during the study period.

   Discussion Top

We detected an obvious renal involvement in 6% of our patients with BD. Microscopic glo­merular hematuria and mild proteinuria were the most frequent presentation in our patients. Membranoproliferative glomerulonephritis and mesangial proliferative glomerulonephritis with IgA deposits were the pathological findings that we found in two of our patients.

We did not perform renal biopsy in two pa­tients with urinary findings suggestive of glo­merulonephritis, the third patient was thought to have cyclosporine neohrotoxicity and the fourth was felt to have BD related chronic kidney disease.

Reports of Gharibdoost in 2068 patients and Shahram in a survey of 3153 cases of BD from Iran and a study 4212 patients with BD by Altiparmak et al from Turkey revealed urinary and renal abnormalities in 6% [10] , 11% [11] and 10.8% respectively. The later study had renal biopsy­confirmed GN in seven patients (0.16%) and end stage renal failure developed in only one patient concluding that urinary abnormalities are more frequent in BD; however, serious renal lesions develop in only very few of these pa­tients. [12]

Similar to our study several case reports and case series report the occurrence of various types of Gn in BD. [3],[8],[13],[14],[15],[16],[17]

Glomerulonephritis with nephritic picture is common at early diagnosis of renal disease in BD whereas secondary amyloidosis is a late se­quelae of chronic inflammation. [8] ,[18],[19],[20] In a report from Turkey the most common cause of renal failure in BD was amyloidosis. [19] Shahram et al reported that amyloidosis among the Iranian patients with BD is extremely rare. [9] None of our patients had a nephritic proteinuria or any other sign of systemic amyloidosis.

Although vascular involvement including veno­occlusive, arterial aneurysm and arterial occlu­sion are the leading cause of death in BD, renal vascular disease is found in less than 1% of patients with vascular BD. [21] Renal artery may be involved at any level throughout the renal artery resulting in main renal artery aneurysm or intra renal microaneurysm. [14] ,[8] Microscopic hematuria can result from renal micro-infarcts [22] and proteinuria as a sign of renal vein throm­bosis in these patients. [23]

Renal replacement therapy in BD is also asso­ciated with a high risk of surgical vascular com­plication and failure of A-V fistula. [14] ,[8] Theore­tically peritoneal dialysis could be a more pre­ferred choice. None of our patients reached the end stage renal state.

In conclusion, the present study suggests that renal involvement in BD is not infrequent, al­though in most cases it is mild in nature. Der­matologists, rheumatologists and ophthalmolo­gists taking care of most of these patients should be aware about this possibility of renal involvement and therefore periodic urine exa­mination and serum creatinine level measure­ment should be part of the management pro­tocol.

   References Top

1.Oshima Y, Shimizu T, Yokohari R. Clinical studies on Behcet,s syndrome. Ann Rheum Dis 1963;22;36-45.  Back to cited text no. 1    
2.Chajek T, Fainaru M. Behcet disease: report of 41 cases and a review of literature. Medicine 1975;54:179-96.  Back to cited text no. 2    
3.Rosenthal T, Weiss P, Gafni J. Renal involvement in Behcet's syndrome. Arch Intern Med 1978; 138:1122-4.  Back to cited text no. 3    
4.Yver L, Blanchier D, Aouragh F, et al..Renal involvement in Behcet's disease. Case report and review of the literature. Nephron 1996;73:689-91.  Back to cited text no. 4    
5.Rigby AS, Chamberline MA, Bhakta B. Behcet,s disease. Baillieres Clin Rheumatol 1995;9:375-95.  Back to cited text no. 5    
6.Herreman G, Beaufils H, Godeau P, et al. Behcets syndrome and renal involvement; a histologic and immunofluorescent study of eleven biopsies. Am J Med Sci 1982; 284:10-7.  Back to cited text no. 6    
7.Sahin I, Noyan T, Karaman E, et al. .Mild renal injury in Behcet's disease. Ren Fail 2004; 26:55-7.  Back to cited text no. 7    
8.Akpolat T, Akkoyunlu M, Akpolat I, Dilek.M, Odabas AR, Ozen S. Renal Behcet,s Disease. A Cumulative analysis. Seminar in arthritis and rheumatology 2002; 31: 317-37.  Back to cited text no. 8    
9.Shahram F. Behcet's disease. Tehran University press. 2000. (In Persian)  Back to cited text no. 9    
10.Gharibdoost F, Davatchi F, Shahram F, et al. Clinical manifestations of Behcet,s disease in Iran. Analysis of 2068 cases. 6 th international conference on Behcet's disease(abstract): La Revue Medecine Interne 1993;14:28S.  Back to cited text no. 10    
11.Shahram F, Davatchi F, Akbarian M, et al. The 1996 survey of Behcet,s disease in Iran. study of 3153 cases. 7 th international conference on Behcet's disease (Abstract): Rev Rhum Eng Ed 1996;63: 538.  Back to cited text no. 11    
12.Altiparmak MR, Tanverdi M, Pamuk ON, Tunc R, Hamuryudan V. Glomerulonephritis in Behcet's disease: report of seven cases and review of the literature.Clin Rheumatol. 2002;21:14-8.  Back to cited text no. 12    
13.Sakemi T, Yoshiyuki T, Ikeda Y, Suzuki N, Nagasawa K. End-stage renal failure due to crescentic glomerulonephritis in a patient with Behcet's syndrome. Review of the literature. Am J Nephrol 1998;18:321-4.  Back to cited text no. 13    
14.Benekli M. Haznedaroglu IH, Erdem Y. Glo­merular involvement in Behcet's disease. Nephrol Dial Transplant 1998;13:1351-4.  Back to cited text no. 14    
15.Donnelly S, Jothy S, Barre P. Crescentic glome­rulonephritis in Behcet's syndrome results of therapy and review of the literature. Clin Nephrol 1989;31:213-8.  Back to cited text no. 15    
16.El Ramahi KM, Al Dalaan A, Al Shaikh A, Al Meshari K, Akhtar M. Renal involvement in Behcet's disease: review of 9 cases. J Rheumatol 1998;25:2254-60.  Back to cited text no. 16    
17.Hemmen T, Perez Canto A, Distiler A, Offermann G, Braun J. IgA Nephropathy in patients with Behcets syndrome Case report and review of the literature. Br J Rheumatol 1997;36:696-9.  Back to cited text no. 17    
18.Olsson PJ, Gaffney E, Alexander RW, Mars DR, Fuller TJ. Proliferative glomerulonephritis with crescent formation in Behcet's syndrome. Arch Intern Med 1980;140:713-4.  Back to cited text no. 18    
19.Akpolat T, Diri B, Oguz Y, Yilmaz E, Yavuz M, Dilek M. Behcet's disease and renal failure. Nephrol Dial Transplant 2003;18:888-91.  Back to cited text no. 19    
20.Bemelman FJ, Krediet RT, Schipper ME. Arisz L. Renal involvement in Behcet's syndrome. Report of a case and a review of the literature. Neth J Neth Med 1989;34:148-53.  Back to cited text no. 20    
21.Koc Y, Gullu I, Akpek G, et al. Vascular involvement in Behcet's disease. J Rheumatol 1992:19: 402-10.  Back to cited text no. 21    
22.Angotti C, D'Cruz DP, Abbs IC, Hughes GR. Renal microinfarction in Behcet's disease. Rheu­matology (Oxford). 2003;42:1416-7.  Back to cited text no. 22    
23.Malik GH, Sirwal IA, Pandit KA. Behcet's syn­drome associated with minimal change glomerulo­nephritis and renal vein thrombosis. Nephron 1989;52:87-9.  Back to cited text no. 23    

Correspondence Address:
Mohammad Reza Ardalan
Nephrology Department, Tabriz University of Medical Sciences, Tabriz
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