Iloprost is useful in the short-term treatment of severe Raynaud's phenomenon and ischaemic ulcers in patients with systemic sclerosis (SSc), but its long-term effects are largely unknown. The aim of this study was to report long-term outcome (median follow-up 36 months) in a prospective observational study of a cohort of 30 consecutive patients with SSc who received iloprost therapy with maintenance infusions every 3 weeks after an initial cycle of 5 consecutive days. At the end of the observation, compared to the pretreatment point, we observed complete healing of digital ulcers in 19/21 patients (90%), a decrease of the Raynaud's phenomenon visual analogue score from 10/10 (25th-75th percentile 7-10) to 5/10 (4-6.75) ( P <0.001) and, in patients with diffuse cutaneous involvement, of the modified Rodnan skin thickness score from 25.5 (16.5-31.5) to 16 (13.5-20) ( P = 0.02), minimal improvement of the Health Assessment Questionnaire from 0.87 (0.68-1.37) to 0.75 (0.62-1.25), which was neither statistically nor clinically significant. The forced vital capacity was not significantly changed, but the diffusion capacity corrected for the alveolar volume decreased from 71% (54-76.7) of the expected value to 62% (51.5-71) ( P = 0.02). In one patient with limited SSc a positive effect on pulmonary hypertension was observed. Six patients, after a median of 25 months of treatment and healing of digital ulcers, discontinued the therapy; after a median of 10 months ulcers did not recur in five of these six. Other reasons for discontinuation were: tolerability (1), disease progression (normotensive renal crisis: 1), and death due to intracranial haemorrhage (1). This same patient had previously suffered a central retinal vein thrombosis. In conclusion, long-term therapy with iloprost in patients with SSc has a durable effectiveness on ischaemic ulcers and Raynaud's phenomenon, but it is not possible to conclude that the natural history of the disease was modified.
The influence of immunodeficiency on the course of hepatitis C virus (HCV) infection is still debated, although a worsening effect has been suggested. We compared the characteristics of hepatitis C in two groups of hematologic patients with different levels of immunocompetence who acquired the same virus strain after treatment with contaminated intravenous immunoglobulins (IVIG). Indications for IVIG therapy were idiopathic thrombocytopenic purpura (ITP) in six patients and hypogammaglobulinemia in 7 patients with various hematologic disorders, who were defined immunodeficient (ID). Infection rate was 100%. Five ID patients never developed HCV antibodies despite serum HCV-RNA positivity. The same HCV genotype was shown in 10 patients tested. Moreover, E1-E2 gene partial nucleotide sequencing, performed in four patients, showed identical or closely related amino acid sequences, thus strongly supporting the hypothesis of a common source of infection. Clinical acute infection did not differ significantly between the two groups, but subsequent liver failure developed in five of the seven ID patients and in none of the ITP patients (P = .04). Liver biopsy, performed in three cases, documented HCV as the only cause of liver damage. Six ID patients died, with liver disease being the primary cause of death in four cases and a contributory cause in two cases. Their median survival after IVIG was 12 months, significantly worse than that of ITP patients (P = .0028). We conclude that immunodeficiency markedly worsens the course of IVIG-acquired HCV infection in hematologic patients.
The natural history of chronic HCV carriers with repeatHepatitis C virus (HCV) carriers with normal aminotransedly normal alanine transaminase (ALT) activities is still unferase levels often show histological chronic hepatitis. This known. Although chronic hepatitis was observed in most study was undertaken to determine the effect of interferon cases, 4-7 the potential progression of liver damage to cirrhosis (IFN) in such patients. Nineteen HCV carriers with normal irrespective of liver enzyme levels cannot be excluded. Furaminotransferase activities and chronic hepatitis were ranthermore, the characteristics of the virological response to domized to receive IFN-a2b (3 million units 3 times weekly antiviral treatment in this group of patients has not been for 12 months) or no treatment. Therapy was monitored by analyzed. These observations suggested a pilot study of the qualitative and quantitative determination of viral RNA. Paresponse to interferon (IFN) in patients with normal ALT tients who did not clear HCV RNA after 6 months disconlevels and chronic hepatitis. None of the subjects included in this study reported alcohol abuse, intravenous drug use, or sexual activity with multiple partThe availability of serological tests for antibodies to hepatiners. Five subjects had previously received blood transfusion(s).tis C virus (anti-HCV) has allowed the identification of Hepatitis B surface antigen was negative in all cases, whereas antichronic infection in subjects without symptoms or biochemibodies to hepatitis B core antigen and hepatitis B surface antigen cal signs of liver disease.
Forty-one percent of anti-HCV-positive donors with persistently normal ALT had active HCV infection. Long-term ALT monitoring allowed the detection of significantly increased enzyme values in only 2 of 16 viremic donors. Reactivity on RIBA-2 or -3, greater age, mean ALT levels in the upper range of normal, higher S:CO ratio on second-generation enzyme-linked immunosorbent assay, and higher gamma globulin levels were predictive of viremia.
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