Budd-Chiari syndrome and renal failure in behcet disease: Report of a case and review of the literature

doi:10.1016/0002-9343(83)90364-9

The American Journal of Medicine

Volume 75, Issue 3, September 1983, Pages 541-550

https://doi.org/10.1016/0002-9343(83)90364-9 Get rights and content

Abstract

A case of Behcet syndrome associated with three rare complications is presented. The patient initially presented with asymmetric polyarticular arthritis, subcutaneous nodules, conjunctivitis, and episcleritis. Necrotizing vasculltis and renal failure subsequently evolved. Despite immunosuppressive therapy, Budd-Chiari syndrome with high grade hepatic obstruction developed. A Denver shunt procedure failed to alter the terminal course. At postmortem examination, skin lesions, carditis, hepatic vein thrombosis, gastric ulcerations, and focal proliferative glomerulonephritis were found. The immunoglobulins and complement present in skin and renal tissue suggest an immune complex pathogenesis.

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Cited by (47)

Budd-Chiari syndrome in the course of Behcet's disease: Clinical and laboratory analysis of four cases
2007, Joint Bone Spine
Citation Excerpt :
Many case reports [4,8,11,12] have evaluated the clinical consequence and incidence of BCS in BD patients. In most of these reports, factors that confer a predisposition to the development of BCS have not been investigated in detail [4,12]. Therefore, the present study aims to present the clinical and laboratory findings of four patients with BCS caused by BD and to evaluate the factors predisposing them to thrombosis.
Budd–Chiari syndrome (BCS) is an uncommon manifestation of Behcet's disease (BD) characterized by hepatic venous outflow obstruction either within the liver or in the inferior vena cava (IVC). Clinical and laboratory findings, as well as treatment options and prognosis of four BD patients with BCS, were analyzed retrospectively.
Four male patients with BCS due to BD, ranging in age from 22 to 46 (median 24 years), were included in the present study. The presence of BCS was confirmed by Doppler ultrasonography and computed tomography and/or venography. Levels of protein C, protein S, antithrombin III, anticardiolipin antibodies and lupus anticoagulants were evaluated in all patients during the acute stage of BCS and both 6 months and 1 year after. Activated protein C resistance and prothrombin gene mutation were also determined in three patients.
Two of the four patients with BCS had already been diagnosed with BD, while the other two were diagnosed with concurrent BD and BCS upon their admission to our clinic. All patients had deep venous thrombosis and superficial migratory thrombophlebitis prior to development of BCS. Case 1 died due to hepatic failure 1 month after his admission. The overall health state of the other three patients has remained good thanks to anticoagulation and immunosuppressive treatments. Three of these patients had one or more than one factor predisposing to thrombosis.
The number of occluded hepatic veins and right hepatic vein involvement, as well as inferior vena cava thrombosis, can be deemed as a major determinant of prognosis. Although the presence of a thrombophilic condition may not be a factor for the clinical prognosis of BCS on its own, it may still necessitate a long-term anticoagulation for prevention of further thrombotic events.
Budd-Chiari syndrome in the course of Behcet's disease: clinical and laboratory analysis of four cases<sup>{lozenge, open}</sup>{lozenge, open}Pour citer cet article, utiliser ce titre en anglais et sa référence dans le même volume de Joint Bone Spine.
2007, Revue du Rhumatisme (Edition Francaise)
Le syndrome de Budd-Chiari (SBC), qui se définit par une obstruction des veines hépatiques ou de la veine cave inférieure sus-hépatique, survient occasionnellement dans le cadre de la maladie de Behçet. Nous avons rétrospectivement analysé les signes cliniques et biologiques, les possibilités thérapeutiques et le pronostic chez quatre malades qui avaient une maladie de Behçet compliquée de SBC.
Les quatre malades, tous de sexe masculin, étaient âgés de 22 à 46 ans (médiane : 24 ans). Le diagnostic de SBC a été confirmé par échographie-doppler et tomodensitométrie, avec ou sans phlébographie. Les concentrations circulantes de protéine C, protéine S, anticorps antithrombine III, anticorps anticardiolipine, et anticoagulants lupiques ont été mesurées pendant la phase aiguë du SBC puis 6 et 12 mois plus tard. Une résistance à la protéine C activée et une mutation du gène de la prothrombine ont été recherchées chez trois malades.
Le diagnostic de maladie de Behçet a été porté deux fois avant la survenue du SBC et deux fois au moment de l'hospitalisation pour SBC. Des antécédents de thrombose veineuse profonde et de thrombophlébite superficielle migratoire ont été retrouvés dans tous les cas. L'un des malades est décédé d'insuffisance hépatique un mois après son admission. Les trois autres patients sont en bon état général grâce au traitement anticoagulant et immunosuppresseur. Un ou plusieurs facteurs prédisposant aux thromboses ont été mis en évidence chez trois malades.
Le nombre de veines hépatiques occluses, l'atteinte de la veine hépatique droite et la présence d'une thrombose de la veine cave inférieure semblent étroitement liés au pronostic. La présence d'une thrombophilie ne semble pas à elle seule influencer le pronostic clinique du SBC mais peut néanmoins obliger à un traitement anticoagulant au long cours destiné à prévenir les récidives thrombotiques.
Behçet desease: Uncommon etiology of myocardial infarction
2004, Annales de Cardiologie et d'Angeiologie
Les auteurs rapportent un cas d’infarctus du myocarde dans le cadre d’une maladie de Behçet chez un homme de 25 ans n’ayant aucun facteur de risque coronarien. Le diagnostic de la maladie de Behçet a été retenu devant une thrombophlébite récidivante, une aphtose bipolaire, une uvéite postérieure, un test pathergique positif et un typage HLAB51. Il s’agit d’un infarctus de myocarde antérieur compliqué d’une fibrillation ventriculaire réduite par choc électrique externe. La coronarographie montre un réseau coronaire sain. Dans la littérature, une vingtaine de cas d’infarctus du myocarde ont été rapportés au cours de la maladie de Behçet. L’étiopathogénie, l’imputabilité diagnostique ainsi que le traitement sont encore difficiles à préciser.
A 25-year-old man who was known to have Behçet’s syndrome and who has no coronary risk factors suffered an acute anterior wall myocardial infarction which was complicated by a ventricular fibrillation. The diagnosis of Behçet’s syndrome was based on reccurent thrombophlebitis, genital and oral aphtoses, posterior uveitis, positive pathergy test and HLAB51. About 20 cases of myocardial infarction were reported in the litterature but the etiopathogeny, the causal relationsheep and the treatment are yet unknown.
Renal Behçet's disease: A cumulative analysis
2002, Seminars in Arthritis and Rheumatism
Citation Excerpt :
Most reported cases had severe histopathologic abnormalities (crescents and diffuse proliferation), probably a result of overrepresentation of cases with clinically significant renal disease. Immunofluorescence examination of most of the renal biopsies showed deposition of immunoglobulin G (IgG), IgA, immunoglobulin M (IgM), and C3 mainly along the capilleries and in the mesangium (18,20-33,35,36,38,40-43,47-49,53-55). Pauci-immune and ANCA-associated GN have been reported in 2 cases (44,45).
Objective: To analyze cumulated data about renal involvement in Behçet's disease (BD) and to report on 6 patients with BD and renal problems. Methods: We found reports of 159 patients (including our patients) with BD and specific renal disease (amyloidosis 69, glomerulonephritis [GN] 51, renal vascular disease 35, and interstitial nephritis 4) in our survey. Results: The frequency of renal problems among BD patients has been reported to vary between 0% to 55%. Male gender is a risk factor for all types of renal BD. Nephrotic syndrome was present in 83% of patients with amyloidosis, and renal failure was common at the time of diagnosis. The mean interval between the initial manifestation of BD and diagnosis of amyloidosis was shorter in men than in women (P =.02). AA-type amyloid fibrils were shown in all cases studied. Vascular involvement was common in the patients with amyloidosis (60%). The renal findings in GN show a wide spectrum, from asymptomatic hematuria and/or proteinuria to rapidly progressive GN. Several types of glomerular lesions ranging from minor glomerular changes to crescentic glomerulonephritis are observed in BD. The common types of glomerular lesions among the reported cases are crescentic GN, proliferative GN, and immunoglobulin A (IgA) nephritis. Aneurysms may be located throughout the renal artery, from the orifice of the main artery to intrarenal microaneurysms. Another type of renal disease (amyloidosis or GN) and other major vascular involvement were present in all cases with renal vein thrombosis. Hypertension is common among patients with renal artery aneurysm or stenosis. Microscopic vascular disease was described in 4 patients. Conclusions: Based on data in the literature, we suggest that renal involvement in BD is more frequent than has been recognized, although it is most often mild in nature. Amyloidosis is one of the prognostic factors affecting survival. Patients with vascular involvement carry high risk for amyloidosis, and administration of colchicine to these patients may be beneficial. More evidence is needed to accept interstitial nephritis as a manifestation of BD. In spite of some difficulties, hemodialysis and renal transplantation are safe treatment options in BD-related uremia. Semin Arthritis Rheum 31:317-337. Copyright 2002, Elsevier Science (USA). All rights reserved.
Budd-Chiari syndrome caused by Behcet's disease: Treatment by side-to- side portacaval shunt
1999, Journal of the American College of Surgeons
Background: Behçet’s disease is a chronic multisystem vasculitis of unknown etiology that involves skin, mucous membranes, eyes, blood vessels, joints, central nervous system, digestive system, and occasionally other organs. Budd-Chiari syndrome from occlusion of the major hepatic veins is a rare and serious complication of Behçet’s disease. Although the mortality rate of Behçet’s disease is only 3% to 4%, development of Budd-Chiari syndrome in patients with Behçet’s disease has been associated with a mortality rate of 61%. This report presents the largest reported experience of Behçet’s disease-related Budd-Chiari syndrome confined to the hepatic veins, and results of treatment by side-to-side portacaval shunt (SSPCS). These results are compared with those we have obtained in Budd-Chiari syndrome confined to the hepatic veins without Behçet’s disease, and with results of treatment of Budd-Chiari syndrome in Behçet’s disease reported in the literature.
Study Design: SSPCS was performed in 5 patients with Behçet’s disease who had developed acute Budd-Chiari syndrome, and 27 patients with Budd-Chiari syndrome from other causes. In all patients, Budd-Chiari syndrome was confined to the hepatic veins without involvement of the inferior vena cava (IVC). Patients were studied prospectively and were followed up at regular intervals for from 1.5 to 26 years (mean 10.6 years, 81% more than 5 years). Followup was 100%. Patients were mainly young adults; mean age was 24.6 years in the patients with Behçet’s disease and 30.0 years in those without Behçet’s disease. All patients had massive ascites, abdominal pain, hepatosplenomegaly, and abnormal liver function. Diagnosis was based on angiographic demonstration of occlusion of the major hepatic veins, and liver biopsy findings of intense hepatic congestion and necrosis. SSPCS was performed within 4 months of the onset of Budd-Chiari syndrome in all but 3 patients. Every year or two in followup, patients underwent liver biopsy and evaluation of SSPCS by Doppler duplex ultrasonography and angiography with pressure measurements. Outcomes criteria included mortality rate, SSPCS patency, maintenance of portal decompression, liver function, presence of ascites, presence of portal-systemic encephalopathy (PSE), need for diuretics, quality of life, and return to work. Our results were compared with those reported in the literature in 42 patients who had Budd-Chiari syndrome with Behçet’s disease.
Results: SSPCS permanently reduced the mean portal vein-IVC pressure gradient (mm saline) from 205 to 7 in the 5 patients with Behçet’s disease, and from 250 to 4 in the 27 without Behçet’s disease. There was only one operative death, a patient without Behçet’s disease. One patient with Behçet’s disease died 2 years postoperatively from diffuse vasculitis, a complication of Behçet’s disease, and the other 4 (80%) remain alive. All 26 operative survivors in the group without Behçet’s disease (96%) are alive. Only one patient developed occlusion of the SSPCS, a man without Behçet’s disease, and he required liver transplantation as a result of hepatic decompensation, PSE, and recurrent ascites. All other patients with or without Behçet’s disease remained free of ascites, required no diuretics, were free of PSE, and had reversal of hepatic dysfunction. Serial liver biopsies showed normal architecture in 60% of patients with Behçet’s disease and 46% of those without Behçet’s disease. Return to fulltime work or housekeeping occurred in 80% of patients with Behçet’s disease and 96% without Behçet’s disease. Comparison of outcomes of our patients with 42 cases of Behçet’s disease with Budd-Chiari syndrome reported in the literature, 79% of whom were treated medically, showed striking differences with an overall mortality rate of 61% in generally shortterm followup.
Conclusion: Because the prognosis for long survival is quite good in Behçet’s disease, early diagnosis of Budd-Chiari syndrome is imperative, and prompt treatment by portal decompression surgery is indicated. SSPCS results in reversal of liver damage and correction of hemodynamic disturbances, prolonged survival, and a life of good quality when it is performed early in the course of Budd-Chiari syndrome.
Renal transplantation in polycythemia vera:- A rare case report from India
2020, Indian Journal of Transplantation

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^☆: This research was supported in part by the Veterans Administration.

²: From the Naval Air Regional Medical Center, Pensacola, Florida.

¹: From the William S. Middleton Memorial Veterans Administration Hospital, Madison, Wisconsin.

³: From the Tucson Clinic, Tucson, Arizona.

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Case reportBudd-Chiari syndrome and renal failure in behcet disease: Report of a case and review of the literature☆

Abstract

Am J Med

J Allergy Clin Immunol

Arch Intern Med

Uber rezidiverende, apthose, durch ein virus verusachte Geschwure am Mund. Am Auge und an den Genitalien

Dermatol Wochenschr

Behcet's disease with endocarditis and the Budd-Chiari syndrome

J Clin Pathol

Pulmonary manifestations in Behcet syndrome

Arch Intern Med

Involvement of the nervous system in Behcet's syndrome

Arch Neurol

Involvement of veins in Behcet's syndrome

Br J Dermatol

Major vascular complications in Behcet's syndrome

Postgrad Med J

Behcet's syndrome with obstruction of the vena cava

Q J Med

A propos d'un cas d'association de syndrome de Budd-Chiari et de syndrome de Behcet

Case of Behcet's syndrome accompanied by Budd-Chiari syndrome

Naika Hokan

Acute hepatic vein thrombosis in a patient with Behcet's disease

J Pakistan Med Assoc

The immune complex pathogenesis of glomerulonephritis and pulmonary vasculitis in Behcet's disease

Am J Med

Rapidly progressive glomerulonephritis in Behcet's syndrome

JAMA

Applications of immunoperoxidase techniques in surgical pathology

Inhibition of polymorphonuclear leukocyte chemiluminescence for detection of immune complexes in human sera

J Clin Invest

Inhibition of luminoldependent granulocyte chemiluminescence by rheumatoid arthritis serum

Clin Res

The ocular manifestations of Behcet's disease

Arch Ophthalmol

Ocular manifestations of Behcet's disease

J Oral Pathol

Behcet's disease

Acta Derm Venereol

Histological and direct immunofluorescence study of cutaneous hyperreactivity in Behcet's disease

Br J Dermatol

The quantification and significance of mast cells in lesions of Behcet's disease

Br J Dermatol

The quantification and significance of mast cells in lesions of Behcet's disease

Br J Dermatol

Case report
Budd-Chiari syndrome and renal failure in behcet disease: Report of a case and review of the literature☆