M.H. Houman

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.

Neuropathie périphérique au cours de la maladie de Behçet

Resume Introduction A l’inverse de l’atteinte du systeme nerveux central, l’atteinte du systeme nerveux peripherique au cours de la maladie de Behcet a ete rarement decrite. Nous rapportons une observation de neuropathie peripherique survenant chez un patient atteint d’une maladie de Behcet. Observation Un homme âge de 47 ans, atteint d’une maladie de Behcet diagnostiquee en 1997, a recu un traitement par prednisolone et colchicine jusqu’a janvier 2001. Deux mois plus tard, il a presente des paresthesies des deux membres inferieurs d’evolution ascendante atteignant secondairement les membres superieurs, associes a une faiblesse musculaire. En janvier 2003, il s’est plaint d’une diarrhee motrice et d’une dysfonction erectile. L’electromyogramme avec mesure des vitesses de conduction nerveuse a montre une polynevrite avec atteinte neurogene peripherique sensitivomotrice. La biopsie neuromusculaire a revele une neuropathie axonale moderee. La fibroscopie œsogastroduodenale etait normale et la coloscopie avec biopsie colique n’a pas montre d’aspect en faveur d’un entero-Behcet. Le patient a ete traite par prednisone, colchicine et carbamazepine avec une diminution des paresthesies. Conclusion Cette observation est particuliere par l’association chez un sujet atteint d’une maladie de Behcet d’une polyneuropathie sensitivomotrice, d’une diarrhee d’origine dysautonomiques et d’une dysfonction erectile. La neuropathie peripherique peut etre secondaire a une vascularite des vasa nervorum ou a la prise de colchicine.

Microscopic polyangiitis presenting with peripheral and central neurological manifestations

Although peripheral neuropathy is a common complication of microscopic angiitis, manifestations involving the muscle and the central nervous system have been rarely reported. We describe a 48-year-old man who rapidly developed a clinical picture of mononeuritis multiplex. A month after the appearance of the primary symptoms, he became comatose and had left hemiplegia in relation with a massive cerebral haematoma. Laboratory data revealed signs of inflammation, glomerular dysfunction with microhaematuria, and positive myeloperoxidase-antineutrophil cytoplasmic antibodies. The neuromuscular biopsy disclosed a small-vessel vasculitis, consisting with microscopic angiitis, associated with myositis and extensive axonal loss. The patient had surgical evacuation of the haematoma and received immunosuppressive therapy with good outcome. Thus, microscopic angiitis should be considered as a differential diagnosis in cases of myositis and intracerebral haemorrhage.

Thrombus intracardiaque au cours de la maladie de Behçet: À propos de trois cas

Resume Les thrombi intracardiaques constituent une atteinte exceptionnelle au cours de la maladie de Behcet, et a notre connaissance moins de 50 cas ont ete rapportes dans la litterature. Nous rapportons trois observations de patients presentant un thrombus intracardiaque dans une cohorte de 180 patients successifs atteints d’une maladie de Behcet selon les criteres de l’International study group for Behcets disease. Ils etaient tous de sexe masculin, la moyenne d’âge au moment du diagnostic des thrombi intracardiaques etait de 27 ans et le delai entre le debut de la maladie et la survenue des thrombi etait de 4,8 ans. Les thrombi intracardiaques se sont manifestes par une hemoptysie dans deux cas et par un tableau d’insuffisance cardiaque dans un cas. Deux patients ont eu une thrombectomie chirurgicale d’emblee avec recidive du thrombus apres la chirurgie disparaissant secondairement apres traitement par prednisone, colchicine, immunosuppresseur et antivitamine K. Le thrombus a persiste malgre le traitement medicamenteux chez le troisieme patient, celui-ci refusant la thrombectomie chirurgicale. Les thrombi intracardiaques constituent une manifestation tres rare de la maladie de Behcet qui doit etre evoquee devant toute masse intracardiaque survenant chez un sujet jeune. Leur traitement repose classiquement sur les agents immunosuppresseurs, les corticoides et les anticoagulants. En cas d’inefficacite du traitement medical ou de thrombus massif ou extensif, une thrombectomie chirurgicale peut etre proposee. Les recidives apres traitement chirurgical sont possibles et necessitent un renforcement du traitement medicamenteux. La thrombolyse peut dans certains cas constituer une alternative interessante au traitement chirurgical.Intracardiac thrombus formation is exceptional in Behçets disease (less than 50 cases had been reported). Among 180 patients with Behçets disease (according to the criteria of the international study group for Behçets disease), three were diagnosed as having intracardiac thrombus. All were male, the mean age at the time of the diagnosis of the intracardiac thrombus was 27 years and the mean disease duration was 4.8 years. The presenting symptom was hemoptysis in two patients and heart failure in the third. Surgery was the first attempted treatment in two patients but thrombus recurred and resolved after medical treatment. The third patient was given corticosteroids and anticoagulants as the first line treatment. The thrombus did not resolve and the patient declined surgery. Intracardiac thrombus formation should be considered when a young Behçets disease patient presents with an intracardiac mass. Medical treatment including corticosteroids, immunosuppressive drugs and anticoagulants should be considered as the first line treatment and surgery should be considered when there is no resolution of the thrombus or when it becomes massive and extensive. In certain cases, thrombolytic treatment becomes an interesting alternative to surgery.

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.

Successful treatment of refractory adult onset Still’s disease with rituximab

Adult-onset Stills disease (AOSD) is an uncommon inflammatory condition of unknown origin. In chronic disease, joint involvement is often predominant and erosions are noted in one third of patients. Therapeutic strategies derive from observational data. Corticosteroids are usually the first-line treatment. With inadequate response to corticosteroids, methotrexate appears the best choice to control disease activity and allow for tapering of steroid use. For refractory disease, biological therapy seems the most promising. We report here the case of a 38-year-old female patient with AOSD refractory to cytotoxic agents, treated by rituximab infusion therapy with favorable outcome.

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.

Inflammatory optic neuropathy in Behçet’s disease

Inflammatory optic neuropathy (ON) is a rare event in Behçets disease (BD). We report herein a series of ten BD Tunisian patients with ON and describe its clinical features among them. A retrospective review of BD patients (International Study Group for BD criteria) was performed. The patients were divided into two groups: those presenting an inflammatory ON, and those none. The diagnosis of inflammatory ON was based on the clinical examination, visual field and visual evoked potentials. We analyzed the characteristics of the two groups. Ten patients (2.3%) presented an inflammatory ON among our 440 patients. Inflammatory ON was inaugural in 8 cases. Clinical manifestations were as follows: blurred vision (7 cases) and periorbital pain (3 cases). In two cases, the patients did not complain from ophthalmological symptoms. The fundus revealed a papilledema (2 cases), papillary pallor (4 cases), and was normal in 5 cases. Visual field realized in only three patients showed a scotoma in all cases. Visual evoked potentials revealed increased latency in all cases. All patients received corticosteroids associated to an immunosuppressive agent. The comparative study between the two groups revealed that inflammatory ON was significantly more associated to neurological involvement (p<0.0001) and that the disease was more severe in the ON group (p<0.0001). Inflammatory ON in BD is rare and may occur at an early stage of the clinical course of the disease. Its prevalence is certainly underestimated. A systematic visual evoked potential may be interesting as a screening tool.

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.

Intracardiac thrombus in Behçet’s disease

Behçets disease (BD) is a multisystem inflammatory disorder. Intracardiac thrombus (ICT) formation is an uncommon but important complication of BD. Of the cases of Behçets disease, we selected those with ICT. All patients fulfilled the diagnostic criteria of the International Study Group of Behçets disease. The ICT in each case was confirmed by ultrasonography, computed tomography and MRI. Clinical features and laboratory parameters were determined. Among our 518 patients with BD, 8 were diagnosed as having intracardiac thrombus (ICT). All were male; the mean age at the time of the ICT diagnosis was 30.8 years. The main presenting symptoms were hemoptysis, chest pain, and dyspnea. It was associated with pulmonary artery aneurysm and vena cava thrombosis in 3 cases each, pulmonary embolism, and lower limbs deep venous thrombosis in 1 case each. The coexistence of other cardiac complications was as follows: pericarditis in 2 cases, myocarditis, endomyocardial fibrosis, and coronary arteritis with consequent myocardial infarction in one case each. In all cases, echocardiography was sufficient to reach the diagnosis. Chest computed tomography performed in all cases led to the diagnosis of associated pulmonary vasculo-Behçet lesions in 4 cases. All patients received colchicine, anticoagulation, and corticosteroids. Seven patients were on immunosuppressant agents (2 patients received azathioprine and 5 cyclophosphamide). Clinical remission with ICT resolution was observed in 5 cases. Combined immunosuppressive therapy with prednisone and cyclophosphamide might be needed to treat ICT due to BD.