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Unlabelled: In the most cases the causes of systemic vasculitis are unknown and treatment is symptomatic (corticosteroids often associated with immunosuppressive agents). We report three cases of systemic vasculitis associated with infections for which dramatic improvement was observed without cortico-therapy (in two patients). CASE REPORT 1: A previously overweight 72-year-old woman was admitted because of a one-year history of fever, fourteen kilogram weight loss, vascular purpura, and polyneuropathy. Abnormal laboratory values included inflammatory syndrome [erythrocyte sedimentation rate (ESR): 80mm/first hour, thrombocytosis: 500,000/microliter, hypereosinophilia (1200/microliter) and positive perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) [anti-myelo-peroxydase antibodies: 30 U (normal < 7)]. Neuromuscular biopsy showed necrotizing vasculitis involving small and medium arteries. Further studies revealed a florid diverticulosis of the colon and no other severe visceral involvement. Treatment was started then with sigmoidectomy. Within six weeks her general condition improved dramatically without corticotherapy: regression of all systemic manifestations, the ESR normalized, and p-ANCA became negative. Ten months later she was still asymptomatic. CASE REPORT 2: An 50-year-old-man developed a progressive fifteen-kilogram weight loss (within 2 months), severe polyneuropathy of all four limbs. His ESR was 120 mm/first hour, and C-reactive protein 200 mg/l. Neuromuscular biopsy showed necrotizing vasculitis affecting small vessels in the nerve and no immune deposits. Stomatologic examination revealed a multiple foci of dental infections. The extraction of all these foci of infections associated with antibiotics improved dramatically all systemic manifestations (within eight weeks), once more without corticotherapy. Eight months later the patient remained asymptomatic. CASE REPORT 3: A 30-year-old-woman was admitted because of five-week history of fever, myalgias, polyarthritis, and cutaneous nodules in her limbs. Abnormal laboratory values included inflammatory syndrome, proteinuria of 0.7 g/day, and a significant rise in Chlamydia trachomatis antibodies titres from 1/64 to 1/256 over a 5 week period. She had a previous history of genital condyloma. The prednisone initialed (0.5 mg/kg/day) twelve days prior to admission was gradually reduced (stopped within 2 months) and treatment with doxycycline (200 mg/day) was initiated. Within six weeks of antibacterial treatment we assisted to a total regression of the initial clinical manifestations and laboratory values became normalized. Ten months later she remained asymptomatic., Conclusion: In systemic vasculitis, investigations in a search of foci of infections are of dual interest: possible etiologic agent like our case reports (strong evidence for an infectious association) and, from a therapeutic perspective, we must identify the microbes behind vasculitis syndromes, since treatment with corticosteroids may have serious consequences if the patient has an active infectious disease.

Raynaud's phenomenon is mainly linked with cold provoked vasomotor perturbations, but also with rheological alterations since blood viscosity is enhanced by lowering temperature. Several methods are available for studying distal vascularization: peri-ungual capillaroscopy, digital plethysmography and laser-Doppler. Digital arteriography must be reserved to serious ischemia regarding the general anesthesia needed to avoid spasm. All these methods explore especially the vessel wall. Conservely, blood viscosity which has been developed for 25 years investigates the content of the vessel. Since 1965, numerous hemorheological studies pointed out the rheological disorders, especially those concerning plasma and blood viscosity. The most usual viscometry abnormalities revealed erythrocyte hyperaggregation, red cell hypodeformability, blood and plasmatic hyperviscosity. In a comparative study, 46 patients with Raynaud's phenomenon were studied: we performed peri-ungual capillaroscopy, plethysmography and viscosity measurements. The results demonstrated a link between capillaroscopy and thixotropy. Both investigations are never normal at the same time in connectivites and never abnormal at the same time in Raynaud's disease (primary Raynaud's phenomenon). In conclusion hemorheological studies showed nearly normal rheological parameters in Raynaud's disease, but abnormal rheological parameters in secondary Raynaud's phenomenon.

Nailfold capillary microscopy patterns in 100 patients aged more than 65 years and free from inflammatory diseases were compared to those of 100 young healthy adults. A higher prevalence of arteriovenous sludge (36% vs 7%, p less than 10(-6)), increase in capillary loop length (12% vs. 0%, p less than 10(-3)) and especially prominent subpapillary plexus (63% vs 12%, p less than 10(-9)) was found in the geriatric group. Such capillary patterns cannot be considered as abnormal in patients aged more than 65 years. Enlargement of capillary loops and loss of capillaries were never encountered. Bushy capillary formations and hemorrhages were very uncommon in both groups.

Venous lesions in Behçet's disease (BD) were defined by Adamantiades and represent one of the most suggestive signs of the disease. They are occasionally the first sign of the disease and are frequently the basis for the diagnosis in a case of recurrent thrombosis in a young subject, the preferential context of BD. Involvement of superficial vessels is virtually constant. Venous vasculitis is responsible for non-specific hypersensitivity and erythema nodosa, which constitute some of the major diagnostic criteria. Ocular periphlebitis is one of the elements responsible for posterior uveitis. The originality of the venous involvement is due to the involvement of deep territories. Any vein may be affected, but the remarkable features are the size of the thrombosed vessels: superior and inferior vena cava, iliofemoral veins and the unusual site of the involvement: supra-hepatic veins, cerebral vessels, etc. Inferior vena cava thrombosis may be associated with aneurysms of the pulmonary arteries in the context of Hughes-Stovin syndrome. Cerebral phlebitis, which can now be identified more easily by means of digital angiography, is responsible for a typical picture: headaches, bilateral papilloedema and raised CSF pressure. The classical pictures of optic chiasmatic arachnoiditis and so-called benign intracranial hypertension actually correspond to unrecognised phlebitis. They may also be associated with other neurological lesions. In one half of cases, phlebitis cutaneous manifestations. However, they may precede the diagnostic signs or may occur very late in the course of the disease. They are recurrent and affect a number of different territories.(ABSTRACT TRUNCATED AT 250 WORDS)

The treatment of Behçet's is essentially symptomatic and depends on the severity of the manifestations. Colchicine is useful in the minor forms, especially the mucocutaneous, forms. It can be safely used at a dose of 1 mg/day as maintenance treatment to prevent or limit the acute episodes. Other immunomodulators have been proposed : levamisole, disulon, thalidomide, the indications for which are limited by the side effects. Non-steroidal anti-inflammatory agents are indicated in the articular forms. Steroid therapy remains essential in the severe neurological and ophthalmological forms at an initial dose of 1 mg/kg/day. It can be preceded by the intravenous bolus administration of high doses of methylprednisone. Immunosuppressants are useful in the same indications. Although some authors propose them right from the start, we prefer to reserve them for second line treatment in the event of steroid dependence or recurrence, because of their short-term infectious and long-term oncogenic risks. Cyclosporin, which has been proven to be effective by randomised studies, is difficult to manage and responsible for complications, in particular renal. It can therefore only be used as second line treatment by experienced users. Plasmapheresis is reserved as an emergency procedure for functionally threatening forms. Venous or arterial thromboses justify longterm anticoagulation, possibly associated which platelet anti-aggregants. In this chronic disease, the quality of follow-up and the patient's cooperation are essential in order to intervene rapidly and to detect as early as possible the complications of the disease and of the treatment.

Etiologic research into caval syndromes enables distinction to be made between superior caval thrombosis, when there is generally a local cause, and inferior caval lesions that are usually of a general origin. Acute disseminated lupus erythematosus and Behçet's disease should be investigated for, particularly in young patients, when an inflammatory syndrome is detected, and when the thrombus lies in the inferior cava territory.

We report a case of Behcet's disease complicated by aortic aneurysm and contiguous vena cava thrombosis due to compression. Arterial aneurysms are uncommon in the course of Behcet's disease and are associated with a poor prognosis owing to the risk of rupture. Vena cava thrombosis is found in 10% of cases; pulmonary embolism is infrequent. Venous and arterial lesions usually evolve independently. In most cases they are consecutive to vasculitis. The case reported herein is uncommon because of simultaneous and contiguous venous and arterial lesions. Eighteen months after aorto bi-iliac graft and inferior vena cava ligature, there is no recurrence of thrombosis nor aneurysm with a treatment including heparin, colchicine and azathioprine.

The microcirculation (terminal vessels with a calibre of less than 30 mu) was examined by means of capillaroscopy which was correlated with histological studies. According to the classification of vasculitis, microvasculitis is a pathological process involved in a number of collagen diseases. The study of the cutaneous lesions of leukocytoclastic angiitis is one of the best method for approaching the study of these microangiopathies, especially in the context of hypersensitivity vasculitis. The study of the microcirculation may also be valuable in other connective tissue diseases (various forms of necrotic vasculitis, lupus, scleroderma, polydermatomyositis) and in Behçet's disease. However, this study is only in its preliminary stages and the data of capillaroscopy need to be interpreted critically.

The treatment of Behçet's disease is essentially symptomatic and depends on the severity of the manifestations. Colchicine is usefull in the minor, particularly mucocutaneous forms. It can be safely used at a dose of 1 mg/day as maintenance treatment to prevent or limit the acute episodes. Other immunomodulators have been proposed: levamisole, disulon, thalidomide, the indications for which are limited by the side effects. Non-steroidal anti-inflammatory agents are indicated in the articular forms. Steroid therapy remains essential in the severe neurological and ophthalmological forms at an initial dose of 1 mg/kg/day. It can be preceded by the intravenous bolus administration of high doses of methylprednisolone. Immunosuppressants are useful in the same indications. Although some authors propose them right from the start, we prefer to reserve them for second line treatment in the event of steroid dependence or recurrence, because of their short-term infectious and long-term oncogenic risks. Cyclosporin, which has been proven to be effective by randomized studies, is difficult to manage and responsible for complications, in particular renal. It can therefore only be used as second line treatment by experienced users. Plasmapheresis is reserved as an emergency procedure for functionally threatening forms. Venous or arterial thromboses justify long-term anticoagulation, possibly associated with platelet anti-aggregants. In this chronic disease, the quality of follow-up and the patient's cooperation are essential in order to intervene rapidly and to detect as early as possible the complications of the disease and of the treatment.

The frequency of thrombotic episodes in patients with Behçet's disease prompted us to study their hemostasis. Seventy patients were investigated and 27 of them (38%) had history of venous thrombosis; they were compared to 27 healthy subjects and to 16 hospitalized patients in whom the diagnosis of Behçet disease was ruled out. Fibrinolytic activity after a 10 minute venous occlusion was significantly decreased while fibrinogen, factor VIII and von Willebrand factor was increased as well as the tPA inhibitor (PAI-I): the last 3 proteins are synthetized by the endothelial cell. No significant difference was observed between patients with or without history of thrombosis. Coagulation and fibrinolysis changes observed are not specific of Behçet disease since they were also found in a population of patients without Behçet disease and without history of thrombosis.

We report a characteristic case of popliteal vein aneurysm which was demonstrated not only by Doppler ultrasonography and venographic examination, but also by CT scan and magnetic resonance imaging. A review of the literature underlines the rarity of these aneurysms, since less than 20 cases have been published. They are always true aneurysms, most often revealed after an episode of pulmonary embolism. Doppler ultrasonography and venography confirm the diagnosis. The place of CT scan and magnetic resonance imaging remains to be defined. Even if asymptomatic, the embolic risk necessitates surgical resection of the aneurysm and restoration of venous continuity.