Summary
The finding of a focal and segmental glomerulonephritis (GN) in a patient with a Behçet's syndrome led us to perform systematic renal biopsies in ten other patients with the disease. Renal biopsy specimens of 11 patients with Behçet's syndrome (followed for 6 months to 15 years) have been studied by light, electron and immunofluorescence microscopy. In all cases, blood pressure and renal function were normal. Proteinuria was present in five patients. By light and electron microscopy, amyloidosis could not be demonstrated in any case. In one patient, the focal and segmental GN was associated with fibrinoid, electron dense, mesangial and irregular subepithelial deposits. These deposits were also detected in seven other patients but to a lesser degree. Arteriolosclerosis was present in all cases. By immuno fluorescence, small scattered granules of C3 were observed in 10 patients in the mesangium and along the capillary basement membrane. They were diffuse in six cases and focal in four. Small focal deposits containing IgA and/or IgG, Clq were also observed in four cases. Circulating immune complexes found in six out of seven patients in whom they were sought. Rare cases of focal and segmental GN and amyloidosis have been reported in Behçet's syndrome. To our knowledge, glomerular C3 deposits have not been yet reported. These findings with the presence of circulating immune complexes suggest that renal symptoms occasionnally observed in Behçet's syndrome could be related to immune complex deposition.
Similar content being viewed by others
References
Adinolfi M, Lehner T (1976) Acute phase proteins and C9 in patients with Behçet's syndrome and aphtous ulcers. Clin Exp Immunol 25:36–39
Behçet H (1937) Über rezidivierende aphthöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien. Dermatol Wochenschr 105:1152–1157
Beroniade V (1975) Amyloidosis and Behçet's disease. Ann Intern Med 83:904–905
Buge A, Escourolle R, Chomette G, Rancurel G, Dechy H, Hauw JJ, Gray F (1977) Maladie de Behçet avec manifestations neurologiques et fibrose endocardique du coeur droit. Ann Med Interne 128:411–419
Chajek T, Fainaru M (1975) Behçet's disease. Report of 41 cases and a review of the literature. Medicine (Baltimore) 54:179–196
Empey DW, Hale JE (1972) Rectal and colonic ulceration in Behçet's disease. Proc R Soc Med 65:163
Gamble CN, Wiesner KB, Shapiro RF, Boyer WJ (1979) The immune complex pathogenesis of glomerulonephritis and pulmonary vasculitis in Behçet's disease. Am J Med 66:1031–1039
Germuth FG, Rodriguez E (1973) Immunopathology of the renal glomerulus: Immune complex deposit and antibasement membrane disease. Little, Brown & Co, p 165
Gluckman JC, Jacob N, Beaufils H, Baumelou A, Salah H, German A, Legrain M (1978) Clinical significance of circulating immune complexes detection in chronic glomerulonephritis. Nephron 22:138–145
Godeau P, Herreman G, Ben Ismail M, Levassor M, Metzger JP (1972) Syndrome de Behçet. Atteintes péricardique et pulmonaire. Nouv Press Med 1:391–395
Gupta RC, O'Duffy JD, McDuffie FC, Meurer M, Jordon RE (1978) Circulating immune complexes in active Behçet's disease. Clin Exp Immunol 34:213–218
Hamza M, Zribi A, Chadli A, Benayed H (1975) La maladie de Behçet. Etude de 22 cas. Nouv Presse med 4:563–566
Kansu E, Özer FL, Akalin E, Güler Y, Zileli T, Tanman E, Kaplaman E, Müftüoglu E (1972) Behçet's syndrome with obstruction of the venae cavae. Q J Med 41:151–168
Kansu E, Deglin S, Cantor RI, Burke JF, Yon Cho S, Cathart RT The expanding spectrum of Behçet syndrome. A case with renal involvement. J Amer Med Assoc 237:1855–1856
Kawachi-Takahashi S, Tanaka K, Takahashi M, Kawashima T, Shimada K (1975) Determination of serum C9 level by immunodiffusion. Int Arch Allergy Appl Immunol 48:161
Levinsky RJ, Lehner T (1978) Circulating soluble immune complexes in recurrent oral ulceration and Behçet's syndrome. Clin Exp Immunol 32:193–198
Mace BEW, Jones JG (1978) Renal involvement in Behçet's disease. J R Soc Med 71:74
Mason RM, Barnes CG (1969) Behçet's syndrome with arthritis. Ann Rheum Dis 28:95–103
Morel-Maroger L, Leathern A, Richet G (1972) Glomerular abnormalities in non systemic diseases. Am J Med 53:170–184
Movat HZ (1961) Silver impregnation methods for electron microscopy. Am J Clin Pathol 35:528–537
Nazzaro P (1966) Cutaneous manifestations of Behçet's disease. Clinical and histopathological findings. In Int. Symp. on Behçet's Disease, Rome. Karger, Basel New York, p 15
Nestor G, Beroniade V, Carnaru S, Badea I (1970) Behçet's disease with renal involvement (with reference ot three anatomo-clinical cases). Rom Med Rev 14:27–34
O'Duffy JD, Goldstein NP (1976) Neurologic involvement in seven patients with Behçet's disease. Am J Med 61:170–178
O'Duffy JD (1978) Summary of international symposium on Behçet's disease. J Rheumatol 5:229–233
Oshima Y, Shimizo T, Yokohari R, Matsumoto T, Kano K, Kagami T, Nagaya H (1963) Clinical studies on Behçet's syndrome. Ann Rheum Dis 22:36–45
Ota T, Takayama T (1969) To the chronic nephritis like disease specially to Behçet's syndrome. Iryo 23:912–913
Piers A, Pope FM, Seymour W, Wyatt AP (1977) Behçet's disease with arterial and renal manifestations. Proc R Soc Med 70:540–544
Rosenthal T, Bank H, Aladjem M, David R, Gafni J (1975) Systemic amyloidosis in Behçet's disease. Ann Intern Med 83:220–223
Rosenthal T, Weiss P, Gafni J (1978) Renal involvement in Behçet's syndrome. Arch Intern Med 138:1122–1124
Shimada K, Kogure M, Kawashima R, Nishioka K (1974) Reduction of complement in Behçet's disease and drug allergy. Med Biol 52:234
Sobel JD, Haim S, Shafrir A, Gellei B (1973) Cutaneous hyperactivity in Behçet's disease. Dermatologica 146:350–356
Strachan RW, Wigzell FW (1963) Polyarthritis in Behçet's multiple symptom complex. Ann Rheum Dis 22:26–35
Sutherland JC, Vann Markham R, Mardiney MR (1974) Subclinical immune complexes in the glomeruli of kidneys postmortem. Am J Med 57:536–541
Williams BD, Lehner T (1977) Immune complexes in Behçet's syndrome and recurrent oral ulceration. Br Med J 1:1387–1389
Yakohama Y, Kono T, Aoki M (1973) Focal proliferative glomerulonephritis in a patient with Behçet's syndrome. Report of an autopsy case. Ryumachi 13:281–288
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Beaufils, H., Cassou, B., Auriol, M. et al. Kidney involvement in Behçet's syndrome. Virchows Arch. A Path. Anat. and Histol. 388, 187–198 (1980). https://doi.org/10.1007/BF00430687
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00430687