35 research outputs found
Eosinophilic granulomatosis with polyangiitis (EGPA) and PRES: a case-based review of literature in ANCA-associated vasculitides
Eosinophilic granulomatosis with polyangiitis (EGPA) is a small-sized vessel systemic necrotizing vasculitis and belongs to the family of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. The involvement of central nervous system in this condition is pretty rare. Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiological entity described for the first time by Hinchey et al. (N Engl J Med 334(8):494-500, 1996) and characterized by MRI findings of reversible subcortical vasogenic edema predominantly in the white matter of posterior cerebral lobes. There are few case reports describing the concurrence of PRES with ANCA-associated vasculitides. We describe a case of PRES in a patient with a diagnosis of EGPA with a concise review of the literature. The exact cause of this syndrome is unknown. It has been related to eclampsia, drug-induced hypertension, renal insufficiency and also to rheumatologic diseases. Endothelial injury, hypertension and immunosuppressive medications can compromise the regulation of cerebral blood flow. In ANCA-associated vasculitides, patients presenting with symptoms of PRES represent a challenge to treatment with immunosuppressive medications. However, since an inflammatory process might be implicated, judicious use of these agents along with tight control of blood pressure and a supportive therapy may contribute to the resolution of the encephalopathic syndrome treating at the same time other manifestation related to the rheumatologic disease. Larger clinical studies are warranted to optimize the management of vasculitis-associated PRES
Studio clinico aperto, randomizzato, sulla utilizzazione dell'associazione di βIFN1a ed azatioprina, in pazienti affetti da sclerosi multipla recidivante-remittente
In systemic sclerosis skin perfusion of hands is reduced and may predict the occurrence of new digital ulcers
Systemic sclerosis (SSc) patients are at high risk for the development of ischemic digital ulcers (DUs). The aim of this study was to assess in SSc patients a correlation between skin perfusion evaluated by LDPI and DUs and to evaluate the prognostic value of skin perfusion to predict the new DUs occurrence. Fifty eight (47 female, 11 male) SSc patients were enrolled. Skin perfusion of hands and region of interest (ROIs) was measured by Laser Doppler perfusion Imager (LDPI). The proximal-distal gradient (PDG) was present when the perfusion mean difference between ROI1 and ROI2 was > 30 pU. The skin perfusion of hands is lower in SSc patients than in healthy controls. The skin perfusion decreased with severity of capillaroscopic damage. Both mean perfusion of hand and PDG are significantly (p < 0.01 and p < 0.0001, respectively) lower in SSc patients with new DUs than in SSc patients without DUs. Only 2 of 11 SSc patients (18.2%) with PDG developed new digital ulcers, conversely 36 of 47 (76.6%) SSc patients without PDG developed new digital ulcers (p < 0.001). The ROC curves demonstrated a good accuracy of new DUs prediction for PDG (0.78, p < 0.0001). Using this cut-off value of 30 pU, RR for new DUs development in SSc patients without PDG is 4,2 (p < 0.001). LDPI indices could be used in association to the capillaroscopic and clinical findings or serological tests in the identification of patients at high risk of developing DU
Left ventricular mass and intrarenal arterial stiffness as early diagnostic markers in cardiorenal syndrome type 5 due to systemic sclerosis
Background: Cardiorenal syndrome type 5 (CRS-5) includes a group of conditions characterized by a simultaneous involvement of the heart and kidney in the course of a systemic disease. Systemic sclerosis (SSc) is frequently involved in the etiology of acute and chronic CRS-5 among connective tissue diseases. In SSc patients, left ventricular mass (LVM) can be used as a marker of nutritional status and fibrosis, while altered intrarenal hemodynamic parameters are suggestive of early kidney involvement. Methods: Forty-two consecutive patients with a diagnosis of SSc without cardiac and/or renal impairment were enrolled to assess whether cardiac muscle mass can be related to arterial stiffness. Thirty subjects matched for age and sex were also enrolled as healthy controls (HC). All patients performed echocardiography and renal ultrasound. Results: Doppler indices of intrarenal stiffness and echocardiographic indices of LVM were significantly increased in SSc patients compared to HC. A positive correlation exists between LVM/body surface area and pulsatile index (p < 0.05, r = 0.36), resistive index (p < 0.05, r = 0.33) and systolic/diastolic ratio (p < 0.05, r = 0.38). Doppler indices of intrarenal stiffness and LVM indices were significantly higher in SSc patients with digital ulcers than in SSc patients without a digital ulcer history. Conclusions: SSc is characterized by the presence of microvascular and multiorgan injury. An early cardiac and renal impairment is very common. LVM and intrarenal arterial stiffness can be considered as early markers of CRS onset. The clinical use of these markers permits a prompt identification of organ damage. An early diagnosis allows the appropriate setting of pharmacological management, by slowing disease progression
Autologous hematopoietic stem cell transplantation versus low-dose immunosuppression in secondary-progressive multiple sclerosis
A method to assess the average properties of spacecraft ejecta from hypervelocity impact
NUMBER, SIZE AND SPEED DISTRIBUTION OF PARTICLES EJECTED FROM SPACECRAFT SURFACES DURING HYPERVELOCITY IMPACT
Case report: 3D intracranial vessel wall MRI in Susac syndrome: potential relevance for diagnosis and therapeutic management
EXPERIMENTAL STUDY OF SPACECRAFT MATERIAL EJECTED UPON HYPERVELOCITY IMPACT
Twenty-eight hypervelocity impact experiments were
carried out at CISAS impact facility, with the aim of
assessing the amount of ejecta from three different
targets representative of spacecraft materials, i.e.
simple aluminum-alloy plates, silicon solar cells and
simple aluminum-alloy plates covered by MLI
blankets. Projectiles having different size (1, 1.5 and
2.3 mm diameter) were launched at speed ranging from
4 to 5.5 km/s and impact angle from 0\ub0 to 80\ub0 (the
impact angle dependence was evaluated for simple
aluminium targets only). Experiments pointed out that
the number of ejecta produced after HVI is
significantly high (order of thousands). Moreover, it
was shown that brittle materials produce more
fragments than ductile ones, but the environment
pollution and the damage potential of particles coming
from metals are much more critical, since large and
heavy fragments are prevalent in this case