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Summary Background Iloprost, prostacyclin (PGI2) analogue, effective in treatment of peripheral arterial disease, secondary Raynaud's phenomenon (RP) to connective tissue disease (CTD), vasculitis, pulmonary hypertension, is usually infused through peristaltic pump, or recently through a flow regulator. Materials and methods We tested a new portable syringe pump (Pompa Infonde®, Italfarmaco S.p.A., Cinisello Balsamo, Milano) on 120 patients affected by RP to CTD and cryoglobulinaemia, in iloprost therapy with a flow regulator. Results Iloprost infused through portable syringe pump is better tolerated, better appreciated by the patients and nurses and no difference was observed on therapeutic effects, with a lower incidence of side effects statistically significant. Only 3 patients were unable to tolerate the device (2 for changes in pressure and 1 for fear) and shifted to traditional method of iloprost infusion. Conclusions Iloprost infusion through the portable syringe Pompa Infonde® appears to be safe, better tolerated, more acceptable and equally effective compared to infusion through a flow regulator.

Summary Introduction Iloprost is a potent prostacyclin (PGI2) analogue that is effective in the treatment of peripheral arterial disease, vasculitis, pulmonary hypertension, and secondary Raynaud's phenomenon. Intravenous infusions are generally administered with the aid of a peristaltic pump to reduce the risk of adverse reactions caused by unintentional increases in the infusion rate. This increases the cost of care in terms of equipment and personnel and may limit the use of this drug. Materials and methods We retrospectively analyzed 18,432 iloprost infusions administered between 1999 and 2009 to 272 patients with systemic sclerosis (n = 253) and 19 with peripheral arterial disease (n = 19). All infusions were administered in the day hospital over 6 h with a normal IV set-up with a roller flow regulator. Flow rates were set to deliver iloprost at 1-2 ng/kg/min. Rates were verified by direct drop counts during the first 15-20 minutes of the infusion and at each subsequent check. Results There were no adverse events that were fatal, life-threatening, or associated with prolongation of hospitalization and very few events requiring intensive care or continuous monitoring. The latter included 4 cases of tachycardia/arrhythmia (extrasystoles in most cases), 3 cases of hypotension (systolic pressure 170/100 mmHg). All other adverse reactions were mild, reversible, and similar to those seen with iloprost infusion with peristaltic pump. Only one patient had to be switched to another prostanoid (due to intolerance). Discussion Iloprost infusion administered with a normal IV flow regulator appears to be as safe, well tolerated, and effective as traditional infusion with a peristaltic pump.

Objective Rheumatoid arthritis (RA) with concomitant hepatitis B virus (HBV) infection represents a therapeutic challenge due to the risk of HBV reactivation under immunosuppressive treatment. To date there are few data coming from anecdotal case reports that concern HBV reactivation following treatment with abatacept. This observational retrospective study was aimed to assess the safety profile of abatacept in this particular clinical setting. Methods Eleven Italian rheumatologic centers provided data from patients with RA and positive HBV serology treated with intravenous abatacept. HBV markers and clinical and laboratory data were checked at followup visits every 3 months. Results In total, 72 patients were included in the study: 47 inactive carriers, 21 occult carriers, and 4 chronic active carriers for HBV. At baseline all of the patients had normal liver function tests and low or undetectable HBV DNA levels, except for those with chronic active hepatitis. Thirteen patients received prophylaxis with lamivudine, and 4 received treatment with adefovir or tenofovir. At the end of the 24-month followup period, 49 patients were being treated. Data from 316 followup visits showed that abatacept was safe. No patients experienced reactivation of hepatitis B. Treatment withdrawals (23 patients) were due to lack of efficacy, subject decision/lost at followup, or adverse events not related to HBV infection. Conclusion Our study provides reassuring data about the safety profile of abatacept in RA with concomitant HBV infection without universal antiviral prophylaxis. Further prospective studies are needed to confirm these preliminary results.

Objective To assess fetal and maternal outcomes in women with systemic sclerosis (SSc). Methods Prospectively collected data on 99 women with SSc from 25 Italian centers were analyzed retrospectively. Women with SSc were observed during 109 pregnancies (from 2000 to 2011), and outcomes were compared to those in the general obstetric population (total of 3,939 deliveries). The maternal age at conception was a mean ± SD 31.8 ± 5.3 years, and the median disease duration at conception was 60 months (range 2–193 months). Results SSc patients, compared to the general obstetric population, had a significantly increased frequency of preterm deliveries (25% versus 12%) and severe preterm deliveries (

We describe a case of acute ischemia of the 2nd, 3rd and 4th fingers of the right hand secondary to peripheral vascular occlusive disease induced by repeated intra-arterial cocaine injections. The complete occlusion of the distal arteries resolved following treatment with iloprost, a synthetic stable analogue of prostacyclin PGI2, in addition to anticoagulants and antibiotics.

Background Many studies and review have been published concerning the use of hight does of immunoglobulin (IVG) in treatment of polymiositis (PM) or dermatomyositis (DM). Usually this treatment is not employed as first-line therapy, but in relapsing or in no response to conventional immunomodulator therapies. Normally the standard doses of IVG is 2 g/kg in 5 days, very rarely in 2 days and the treatment is usually continued for 3-6 months. Adverse effects are generally few and generally tolerable. Objectives We report our case series in which IVG were used a first therapeutic choice in order to induce a faster remission in acute PM-DM presentation. Methods We observed 15 patients (pts) (4M/11F) mean age 42,6 y (28-72 y) affected by acute PM – DM. Nobody developed a paraneoplastic syndrome. At the onset the mean values of serum creatin kinase (CPK) were 2980 U/l (nv All patients after an initial treatment with corticosteroids (3 pts received also high dose 6-metilprednisolon 1gr/day for 3 days) were treated her with immunoglobulin at a dose of 2g/kg distributed in 5 days for 6 months. Results After 3 infusions the CPK and MG levels were reduced to 70% compared to baseline and normalized after 5 infusions. The mean dosage of corticosteroid was gradually reduced and some immunosuppressant agents (Cyclosporin A in 2 pts, Mycophenolate Mofetile in 2 pts, Azathioprine in 3 pts), were gradually introduced while the other patients continued with a minimum dose of prednisone (5 mg/day), maintaining a complete remission. Dysphagia, short breath, mydriasis were completely resolved, a mild legs weakness persisted until the 6ht month, at HRCT also reticular pattern were improved. Conclusions The treatment with IVG in severe or rapidly progressive PM-DM has been documented in many open-label trials to be effective, especially in pts with lung involvement and esophageal involvement. Adverse effects were generally tolerable and therefore, it may propose the use of IGV use is suggested to induce a faster remission and to allow a faster tapering of the corticosteroid and introduction of immunosuppressive drugs. References Wan DX et al. Rheumatology 2012;31:801-6. Danieli MG et al. Autoimmun Rev 2014;13:1048-54. Disclosure of Interest None declared