M. Lamloum

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.

Characteristics of neurological manifestations of Behcet's disease: A retrospective monocentric study in Tunisia

OBJECTIVE The aim of the present study was to analyze demographic, clinical and genetic features of Behçets disease patients with neurological involvement through a monocentric study of a homogenous group of hospitalized patients observed in the same department and to compare them with those of other ethnic and geographic groups. METHODS Four hundred and thirty Behçets disease (BD) patients were retrospectively studied. Diagnosis of BD was made according to the international study group for Behçets disease criteria. Patients with neurological findings suggestive of involvement of the nervous system by BD were further studied according to clinical examination, laboratory tests and neuroradiological investigations. RESULTS Neurological involvement was observed in 121 patients (28.1%). The mean age at neuro-Behçets disease (NBD) onset was 29.7 years. Average disease duration of BD before neurological manifestations onset was 6.4 years. Male to female ratio was 1.8. Of the 121 NBD patients, parenchymal involvement occurred in 74 patients (61%). Among them 26 (21.4%) presented with brainstem involvement, 24 (19.8%) with hemispheric involvement and 2 (1.6%) with spinal cord involvement. Non-parenchymal NBD occurred in 47 patients (39%). Involvement of the main vascular structures (Vasculo-NBD) was the most common non-parenchymal NBD lesion found in 35 patients (28.9%) consisting of cerebral vein thrombosis (CVT) in 24 cases and cerebral arterial thrombosis in 11 cases. Forty-nine (40.5%) patients with NBD have been followed-up for a median of 3 years (range 1-19 years). Forty-one of them recovered well without significant residual disability, 5 patients made no improvement and are left with severe neurological impairments and 3 died. Male gender and CNS parenchymal lesions occurrence were significantly associated with a poorer prognosis. CONCLUSION Clinical and epidemiological features of NBD are various. In our Tunisian cohort of NBD patients the main characteristic features were male predominance, a relatively high prevalence of CVT, a low prevalence of intra-cranial hypertension and a significant lower frequency of HLA-B51 haplotype.

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.

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.

Pleural myelomatous involvement in multiple myeloma: five cases.

Pleural myelomatous involvement in multiple myeloma (MM) is rare, occurring in less than 1% of cases. We retrospectively studied five cases of patients with MM who developed myelomatous pleural effusions. Three men and 2 women with a mean age of 61 years presented with myelomatous pleural effusion. The pleural fluid electrophoresis revealed a peak of IgG in three cases, of IgA in one case, and of lambda light chains in one case, which were identical to that in the sera of the patients. Detection of typical plasma cells in pleural fluid cytology was contributive, and histologic confirmation by pleural biopsy was positive in four cases. Treatment consisted of chemotherapy. The clinical outcome was initially good, but relapses occurred in all cases early and were complicated by fatal infections. Myelomatous pleural effusion is a rare affection. It is usually a late complication associated with poor prognosis.

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.

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.

Amyloidosis in Behcet′s disease

Behcets disease (BD) is a multisystem vasculitis with protean manifestations. It is characterized by a heightened state of inflammation, although the factors that initiate and sustain this inflammation are not clear. We report some cases of BD-associated amyloidosis and have similar features. The patients developed nephrotic syndrome due to secondary amyloidosis, which was refractory to the immunosuppressive agents. Two patients expired and the third was lost to follow-up during the course. The BD complicated with amyloidosis is associated with high mortality despite the current aggressive therapy.