A case report of double filtration plasmapheresis in an acute episode of multiple sclerosis.

Plasma exchange has been proposed as support therapy in both acute and chronic forms of multiple sclerosis (MS). For the first time, we aimed to assess whether double filtration plasmapheresis (DFPP) could be clinically efficacious. We describe the case of a patient affected by MS who developed a severe crisis refractory to conventional steroids, and immunosuppressive and immunomodulating therapy. The patient underwent 12 sessions of DFPP. In each session 3000 mL of plasma was treated. Before and immediately after each session the routine laboratory parameters were assessed. Before the apheresis cycle and one month after the end of treatment, encephalic magnetic resonance imaging (MRI) was performed. A neurological examination and assessment of the extended disability status scale (EDSS) were made once each week from the beginning of treatment until one month after the end of the cycle. No therapy was administered during the course of the apheresis cycle, with the exception of a scaled dose of steroids, that was completely withdrawn half-way through the cycle. The immunoglobulin (Ig) G, IgA and IgM values declined from 465 +/- 104 mg/dL, 69 +/- 18 mg/dL, 34 +/- 16 mg/dL, respectively, pre-apheresis to 331 +/- 76 mg/dL, 42 +/- 5 mg/dL, 15 +/- 6 mg/dL, respectively, post-apheresis; C3 and C4 decreased from 105 +/- 27 mg/dL and 21 +/- 5 mg/dL to 75 +/- 9 mg/dL and 15 +/- 4 mg/dL, respectively; fibrinogen went from 228 +/- 72 mg/dL to 128 +/- 28 mg/dL. The EDSS dropped from a value of 6 before the cycle to 5.5 one month after the end of the treatment. As compared with the pretreatment conditions, post-apheresis MRI showed stabilization of the lesions already present, the reduction of one lesion and a complete absence of enhancement of all lesions. DFPP, adopted for the first time in MS, seems to foster a short-term improvement in both the clinical and magnetic resonance images during an acute MS episode.

Does a reduction of adhesion molecules by LDL-apheresis have a role in the treatment of sudden hearing loss?

Sudden hearing loss (SHL) is a highly disabling affliction that can severely affect the subject's social and relational life. Although the etiology of the complaint is still debated, it is thought that microcirculation disturbances conditioned by an endothelial dysfunction might be the main pathogenetic mechanism. Adhesion molecules favoring interaction between leukocytes and endothelial cells are early markers of endothelial damage. In the present report, we describe a case of SHL that derived evident benefit from a single session of LDL/fibrinogen apheresis, with complete hearing recovery. In this patient, in addition to reducing LDL cholesterol and fibrinogen, the circulating adhesion molecules (sE-selectin, sVCAM-1 and sICAM-1), previously present in higher than normal concentrations, were reduced by the treatment.

LDL-apheresis accelerates the recovery of nonarteritic acute anterior ischemic optic neuropathy.

Nonarteritic acute anterior ischemic optic neuropathy (NAION) is a disabling disease which impairs visual function. It is presumed to result from disturbances of microcirculation in the anterior portion of the optic nerve head due to hemodynamic factors derived from excessive blood viscosity, or restriction of the vasal lumen in hypertensive, hypercholesterolemic, diabetic patients. We aimed to determine whether acute reduction of plasma fibrinogen and serum low-density lipoprotein (LDL) cholesterol is effective for treatment of NAION. We recruited 11 patients (7 females, 4 males) with a mean age of 57.2 +/- 19.6 years. All except one of them presented risk factors for atherosclerosis. The mean values of LDL-cholesterol and fibrinogen before treatment were 144 +/- 32 mg/dL and 341 +/- 80 mg/dL, respectively. All were treated with standard therapy (prednisone, salicylate, pentoxiphyllin) and underwent three sessions of LDL-apheresis (HELP system-B Braun) that can reduce plasma LDL-cholesterol and fibrinogen by more than 50% in a very short time. In all patients we observed a drastic reduction of LDL cholesterol and fibrinogen and a clear improvement in the visual functional data. In fact, mean values of corrected vision increased from 3.7/10 +/- 3/10 to 7.9/10 +/- 2.2/10 (P = 0.002) after the third session, while the scotomatous portion of the visual field regressed after the first session, and in 5 patients further regressed after the third session. This improvement had remained stable after 3 months. Thanks to it's effect of antagonizing hemorheologic disorders of the ocular microcirculation, fibrinogen/LDL-apheresis seems to be an efficacious treatment of NAION.

Renal vascular resistance and renin-angiotensin system in the pathogenesis of early hypertension in autosomal dominant polycystic kidney disease.

Activation of the renin-angiotensin system (RAS) has been proposed to increase renal vascular resistance (RVR) and to play a role in the development of hypertension in autosomal dominant polycystic kidney disease (ADPKD). The aim of this study was to investigate the relationship among RVR, RAS and blood pressure (BP) profile in patients without renal impairment. Thirty-four ADPKD patients underwent ambulatory blood pressure monitoring (ABPM) over a 24-h period and were divided into two groups: 17 hypertensive (group A, day-systolic BP > or = 135 mmHg and/or day-diastolic BP > or = 85 mmHg) and 17 normotensive (group B, day-BP < 135/85 mmHg) patients. The two groups were comparable with respect to age, sex, and renal function. None of the patients assumed therapy. In all subjects the plasma renin activity (PRA) was measured, and the RVR was assessed by measuring resistivity indices (RI). RI was significantly higher in the hypertensive than in normotensive patients (0.67 +/- 0.05 vs. 0.62 +/- 0.03), while PRA was normal in all subjects, and showed no statistical difference between the two groups. Taking all the patients together (group A + group B), a significant positive correlation between RI and 24-h mean arterial pressure (MAP) was discovered, but no correlation was found between RI and PRA or between MAP and PRA. We conclude that in ADPKD patients without renal impairment the MAP values are strictly correlated with the RVR, but not with PRA. Thus factors other than RAS probably contribute to the increase of the RVR and to the early development of hypertension.