Renal Behçet's Disease: An Update

• Published in: Seminars in arthritis and rheumatism 2008-12, Vol.38 (3), p.241-248
• Main Authors: Akpolat, Tekin, MD, Dilek, Melda, MD, Aksu, Kenan, MD, Keser, Gökhan, MD, Toprak, Ömer, MD, Ci̇ri̇t, Mustafa, MD, Oğuz, Yusuf, MD, Taşkapan, Hülya, MD, Adibelli̇, Zelal, MD, Akar, Harun, MD, Tokgöz, Bülent, MD, Arici, Mustafa, MD, Çeli̇ker, Hüseyin, MD, Di̇ri̇, Banu, MD, Akpolat, Ilkser, MD
• Format: Article
• Language: English
• Subjects: Adult; amyloidosis; Amyloidosis - complications; Amyloidosis - diagnosis; Behcet Syndrome - complications; Behcet Syndrome - diagnosis; Behçet's disease; Biological and medical sciences; Creatinine - blood; cyclosporine; Diseases of the osteoarticular system; Edema - diagnosis; Edema - etiology; Edema - urine; Female; Glomerulonephritis; Humans; interstitial nephritis; Kidney Diseases - diagnosis; Kidney Diseases - etiology; Male; Medical sciences; Middle Aged; Nephrology. Urinary tract diseases; Nephropathies. Renovascular diseases. Renal failure; renal vascular disease; Rheumatology; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; uremia
• ISSN: 0049-0172; 1532-866X
• DOI: 10.1016/j.semarthrit.2007.11.001

Objective The aims of this study are (1) to report 33 patients with Behçet's disease (BD) having various renal manifestations, and (2) to update current data using our patients and published papers about BD and renal manifestations. Methods The PubMed database was searched using the terms BD or Behçet's syndrome. We found reports of 94 patients (including ours) with BD and specific renal diseases (amyloidosis, 39; glomerulonephritis [GN], 37; renal vascular disease, 19; interstitial nephritis, 1). Results The presentation of renal disease was edema/nephrotic syndrome in 12 patients (36%). Renal disease was incidentally diagnosed by routine urine analysis and measurement of serum creatinine level in 20 patients (61%). Renal failure was present in 23 patients (70%) and 5 of them have had cyclosporine treatment. The frequency of renal disease among BD patients has been reported to vary from less than 1 to 29%. Conclusions The clinical spectrum of renal BD shows a wide variation. Amyloidosis (AA type), GN, and macroscopic/microscopic vascular disease are the main causes of renal BD. Patients with vascular involvement have a high risk of amyloidosis and amyloidosis is the most common cause of renal failure in BD. Several types of glomerular lesions are seen in BD. Current treatment options for renal BD are not evidence based. Radiological vascular intervention combined with immunosuppressive drugs can be useful in selected cases. Routine urine analysis and measurement of serum creatinine level are needed for early diagnosis of renal BD.