M. Miled

Budd-Chiari syndrome associated with Behçet's disease.

OBJECTIVES Budd-Chiari syndrome is a rare and serious complication of Behçets disease, and is the result of occlusion of the major hepatic veins, the adjacent inferior vena cava, or both. The aim of this study was to determine the prevalence, clinical and laboratory findings, and treatment and clinical course of Budd-Chiari syndrome associated with Behçets disease. METHODS We analyzed retrospectively the charts of 220 patients fulfilling the international diagnostic criteria of Behçets disease. From them, we selected those with Budd-Chiari syndrome, and analyzed their epidemiological and clinical imaging features and outcomes. RESULTS Seven male patients, mean age 29 years and already diagnosed with Behçets disease, had Budd-Chiari syndrome. The clinical course was from subacute to chronic in all cases. Thrombosis of hepatic veins was associated with inferior vena cava thrombosis in six cases. Four patients had other venous thromboses (superior vena cava and lower limbs) and one also had pulmonary emboli. One patient was positive for anticardiolipin antibodies. All patients had anticoagulation therapy, and six had high-dose corticotherapy associated, in two cases, with monthly cyclophosphamid intravenous pulses. Clinical outcome was favourable in six cases, and one patient died of hepatic failure. CONCLUSION The prevalence of Budd-Chiari syndrome in patients with Behçets disease is 3.2%, confirming that this syndrome is not uncommon in Behçets patients. The inferior vena cava is frequently involved in combination with hepatic veins and often associated with other venous thrombosis. The prognosis may be favorable with medical interventions, including anticoagulation, treatment of the vasculitis and the use of diuretics when required.

Association of right heart thrombosis, endomyocardial fibrosis, and pulmonary artery aneurysm in Behçet’s disease

A 29-year-old man with a 5-year history of Behçets disease was admitted for fever, dyspnae, chest pain, and hemoptysis. A diagnosis of right ventricle and atrial thrombosis associated with a pulmonary artery aneurysm was made. The patient was treated with anticoagulants and prednisone. Since hemoptysis persisted, surgical excision of the intracardiac thrombosis was performed and histological findings were consistent with organizing thrombus and endomyocardial fibrosis. Transesophageal echocardiography 6 months later showed recurrence of the right ventricle thrombosis. A course of 6-monthly boluses of intravenous cyclophosphamide was begun. Currently, at 2 years of follow-up, the patient is asymptomatic.

Contribution of Hla-B51 in the susceptibility and specific clinical features of Behcet's disease in Tunisian patients

BACKGROUND Many researchers have tried to investigate the association of HLA-B51 with the severity and the clinical features of BD with conflicting results. METHODS We aimed at investigating the association of HLA-B51 with demographical and clinical manifestations as well as the severity of BD, by studying 178 native Tunisian BD patients, fulfilling the International Study group criteria for the BD classification recruited from the Department of Internal Medicine, Rabta Hospital in Tunis and compared with 125 native Tunisian healthy age and sex matching volunteers. RESULTS According to our findings, the frequency of HLAB 51 was significantly higher in BD patients than in controls (p<0.001). Positive pathergy test (PPT) (p = 0.01) and retinal vasculitis (p = 0.045), were significantly more frequent in HLA B51(+) patients, while the frequency of arterial aneurysms (p = 0.009) and neurological involvement, especially the parenchymal involvement (p<0.001), were significantly and clearly higher in HLA B51(-) patients. The patients without HLA B51 had a significantly less severe disease (p = 0.001). Discussion/conclusion We conclude that HLA B51 is a predisposing marker for BD in our population as in most ethnic groups. It seems to be associated with a subgroup of BD patients characterized by a higher frequency of ocular involvement and PPT, but a lower frequency of arterial aneurysm and neurological involvement, and a less severe disease course.

Vascularite à anticorps anticytoplasme de polynucléaires neutrophiles induite par le benzylthio-uracile

Resume Introduction De nombreuses observations de vascularites a anticorps anticytoplasme de polynucleaires neutrophiles (ANCA) secondaires aux antithyroidiens de synthese ont ete decrites mais seuls 2 cas ont ete imputes au benzylthio-uracile. Observation Chez une, femme de 36, ans 3 ans apres un traitement par benzylthio-uracile, une insuffisance renale rapidement progressive est apparue, en rapport avec une nephropathie glomerulaire extra-capillaire pauci-immune associee a des lesions de vascularite necrosante. La recherche des p-ANCA etait positive avec une specificite anti-myeloperoxydase. Elle a ete traitee par des corticoides et 6 bolus mensuels de cyclophosphamide puis de l’azathioprine, avec une bonne amelioration de l’insuffisance renale et une diminution significative de la proteinurie. Cependant le taux de p-ANCA n’a pas varie. Conclusion Les vascularites a ANCA sont une complication rare mais grave des traitements par antithyroidiens de synthese de type thio-uraciles. Un dosage des ANCA doit etre effectue en cas d’apparition d’une manifestation systemique en cours de traitement.

MICA Transmembrane Region Polymorphism and HLA B51 in Tunisian Behçeťs Disease Patients

None of MICA microsatellite alleles was significantly increased in Tunisian BD patients. Only HLA-B51 was primarily associated to BD in this population. These data, similar to those found in other ethnics6,9, further support the hypothesis that the amino-acids, common to all HLA-B51 encoding alleles and absent in other HLA-B antigens, probably confer high affinity binding for peptides that may contribute to BD development. However, the possibility that HLA-B contributes to the pathogenesis as an additional or complementary risk factor cannot be excluded.

Une forme disséminée d’histiocytose langerhansienne associant un diabète insipide et un diabète sucré

Resume Introduction. – Lˈhistiocytose langerhansienne est une maladie rare, de cause indeterminee, caracterisee par une heterogeneite clinique, evolutive et therapeutique. Le diabete insipide est une manifestation classique au cours de l’histiocytose. La coexistence d’un diabete insipide et d’un diabete sucre associee a une histiocytose est une eventualite tres rare. Seuls trois cas ont ete rapportes dans la litterature. Exegese. – Nous rapportons lˈobservation dˈune femme atteinte dˈune histiocytose disseminee (hypophyse, osseuse) associee a un diabete sucre qui illustre les difficultes diagnostiques et therapeutiques que pose cette association. Conclusion. – Les differents mecanismes etiopathogeniques pouvant expliquer la survenue dˈun diabete sucre au cours de lˈhistiocytose langerhansienne sont discutes.

Sténose urétérale au cours de la granulomatose de Wegener: Une observation

INTRODUCTION Ureteral stenoses in Wegeners granulomatosis are rare. They usually involve the pelvic portion of the ureter and are caused by vasculitic lesions or granulomatous inflammation. CASE A 38-year-old woman with Wegeners granulomatosis was treated with corticosteroids and monthly intravenous cyclophosphamide pulses. After 4 months, urinary retention developed, accompanied by lumbar pain, associated with protenuria and hematuria, and related to bilateral ureteral stenoses. Treatment by endoscopic dilatation and double J stents led to with clinical and radiological improvement, while the medical treatment continued. CONCLUSION Hematuria in patients with Wegeners granulomatosis suggests renal involvement, but ureteral stenoses must also be considered when these patients present hematuria or urinary tract infections. Surgery should be reserved for those patients in whom medical treatment is not rapidly effective.Recu le 8 aout 2005 Accepte le 20 juin 2006 ■ Summary Ureteral stenosis in Wegeners granulomatosis: Case report Introduction > Ureteral stenoses in Wegeners granulomatosis are rare. They usually involve the pelvic portion of the ureter and are caused by vasculitic lesions or granulomatous inflammation. Case > A 38-year-old woman with Wegeners granulomatosis was treated with corticosteroids and monthly intravenous cyclophospha- mide pulses. After 4 months, urinary retention developed, accompa- nied by lumbar pain, associated with protenuria and hematuria, and related to bilateral ureteral stenoses. Treatment by endoscopic dila- tation and double J stents led to with clinical and radiological impro- vement, while the medical treatment continued. Conclusion > Hematuria in patients with Wegeners granulomatosis suggests renal involvement, but ureteral stenoses must also be consi- dered when these patients present hematuria or urinary tract infec- tions. Surgery should be reserved for those patients in whom medi- cal treatment is not rapidly effective.

Faux anévrysmes artériels iatrogènes au cours de la maladie de Behçet: À propos de deux cas

Resume La maladie de Behcet est une vascularite systemique d’origine inconnue caracterisee par une aphtose buccale et genitale recidivante, une atteinte oculaire et une atteinte cutanee. L’atteinte vasculaire est presente dans 7 a 38% des cas, representes essentiellement par l’atteinte veineuse. L’atteinte arterielle n’est presente que dans 2 a 8% des cas et est associee a une forte mortalite. Elle peut se presenter sous la forme d’anevrysme, de pseudo anevrysme ou d’occlusion arterielle. Le role des traumatismes de la paroi arterielle dans la genese des pseudo anevrysmes est bien connu. Nous rapportons deux observations exceptionnelles de pseudo anevrysmes iatrogenes faisant suite a des ponctions arterielles accidentelles, interessant respectivement l’artere carotide interne et l’artere humerale. Les caracteristiques cliniques et les modalites therapeutiques de l’atteinte arterielle au cours de la maladie de Behcet seront discutees. (J Mal Vasc 2006; 31: 88-92)Behçets disease is a multi systemic vasculitis of unknown origin characterized by recurrent oral and genital ulcerations, uveitis and skin lesions. Vascular involvement mainly interesting the venous system is present in about 7 to 38% of patients. Arterial involvement is present in 2 to 8% of patients and is associated with a high mortality. It may present as aneurysms, pseudo aneurysms or arterial occlusions. Pseudo aneurysm formation has been reported to be sometimes due to invasive imaging techniques, it may also follow arterial repair. We report two exceptional localizations of iatrogenic pseudo aneurysms due to accidental arterial puncture, involving respectively the internal carotid artery and the humeral artery. We discuss the clinical characteristics and therapeutic modalities of this complication.

Microscopic Polyangiitis Associated with Primary Biliary Cirrhosis, Sjogren's Syndrome and Hashimoto's Thyroiditis

The association between microscopic polyangiitis (MPA) and primary biliary cirrhosis (PBC) has seldom been reported. We describe here a patient who presented with sensorimotor neuropathy along with hypothyroidism, renal failure and liver dysfunction. Detection of antinuclear antibodies at a titer of 1/800, anti-SSA, anti-SSB, anti-GP210, anti-microsomial and p-ANCA anti-myeloperoxydase antibodies along with renal, salivary and liver biopsy led to a diagnosis of MPA associated with PBC, Sjogrens syndrome and Hashimotos thyroiditis.

Une gale norvégienne compliquant un traitement immunosuppresseur : à propos d'une observation

Summary Norwegian scabies, also called crusted scabies because of hyperkeratotic lesions, is rare. The infection is frequently overlooked because of its atypical presentations. It is a highly contagious variant of classical scabies, has traditionnally been associated with mentally retarded and physically impaired patients. Recent reports have increasingly linked scabies to immunosuppression, as in acquired immunodeficiency syndrome. Norwegian scabies, an ectoparasitic infestation by Sarcoptes scabiei, features hyperkeratotic lesions of the palm, of the sole, scalp and ears. These lesions contain a great number of mites. The authors report a case of Norwegian scabies in a patient undergoing immunosuppressive therapy for a polyarteritis nodosa. Diagnostic and treatment are briefly reviewed.