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T regulator lymphocytes (Tregs) play a key role in the maintenance of immune tolerance and in the development of autoimmune diseases. Expression of Foxp3 is specific for Tregs, and can be used for the identification of these cells. This study investigated the variations of Tregs Foxp3+ in the kidney biopsies inflammatory infiltrate of different lupus nephritis classes compared to that of ANCA glomerulonephritis, acute tubulointerstitial nephritis and nephroangiosclerosis. Sections of paraffin-embedded tissue have been stained by immunohistochemistry with anti-CD3 and anti-FoxP3 antibodies. We find that the ratio of FoxP3+/CD3+ cells is significantly lower in patients with lupus nephritis class IV and in patients with vasculitides than in the course of nephroangiosclerosis, tubulointerstitial nephritis and lupus nephritis class V. The data presented herein demonstrate a decrease of FoxP3+ Treg cells in the inflammatory infiltrate of lupus nephritis, particularly during the most active phases of lupus nephritis, as observed in the course of a IV class nephritis.

Whether tumor necrosis factor-alpha (TNFα) caused beneficial or detrimental cardiovascular effects remains poorly defined. Anti-TNFα agents improved cardiac end points in chronic rheumatic diseases characterized by progressive deterioration of cardiac function. In contrast, anti-TNFα agents did not always improve but actually worsened cardiac function in non-rheumatic patients with heart failure (HF), in spite of that HF usually accompanies with high circulating levels of TNFα. To shed light on these mixed findings, we characterized the effects of TNFα in H9c2 cardiomyocytes. Cells were incubated for 24 h with increasing concentrations of TNFα, hydrogen peroxide, aminotriazole, or etoposide. Posttreatment cell viability was assessed by antimycin A-inhibitable reduction of 3-(4,dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide, and the IC50 value of each test compound was defined. H9c2 cells were also preconditioned with a low non-toxic concentration of TNFα and then re-challenged with increasing concentrations of TNFα and other stressor agents. In re-challenge experiments, all of the IC50 values increased significantly, with the IC50 value of TNFα increasing approximately 16-fold. TNFα preconditioning increased cardiomyocytes shedding of the external portion of transmembrane type 1 and type 2 TNFα receptors [(soluble TNFα receptors (sTNFR)]. Levels of survival-oriented soluble TNFR2 (sTNFR2) always exceeded those of death-oriented sTNFR1. When exposed to TNFα at its IC50 value, preconditioned cardiomyocytes showed an increased release of sTNFR2 but not sTNFR1. These results denoted that preconditioning by "low TNFα" helped cardiomyocyte to withstand toxicity from "high TNFα" or other agents. These results also suggested that beneficial or detrimental effects of anti-TNFα agents might well depend on whether these agents spared or intercepted discrete amounts of TNFα that preconditioned cardiomyocytes and made them more resistant to high concentrations of TNFα.

Schwannomas are benign encapsulated neoplasms of the peripheral nerve sheath. Clinical diagnosis is often difficult, and final diagnosis is usually based on histopathological findings. Imaging techniques, such as magnetic resonance imaging and ultrasonography, can be optimized to improve assessment prior to surgical treatment. The present case demonstrates that high-resolution sonography can be useful in assessing the underlying cause of median nerve pathology.

The purpose of the present study was to determine levels of adipokines and their relationship with stiffness parameters and disease activity index in SLE patients in comparison with healthy controls. Sixty SLE patients and 29 control subjects were enrolled in the study. Serum leptin and adiponectin levels were determined by commercial sandwich ELISA kits. Colour-coded carotid duplex sonography was performed using a Siemens SONOLINE Antares machine equipped with linear 5-13 MHz. SLEDAI, ECLAM and SLICC were evaluated in all patients. Data were analysed by software for statistical analysis (Prism 5.0). Median leptin is higher among SLE patients compared with controls (p 0.035). Median values of vascular stiffness and PSEM are increased in SLE compared with controls (p = 0.0003 and p = 0.007). Vascular strain and vascular distensibility are lower in SLE patients in comparison with controls (p = 0.0001 and p = 0.0006, respectively). Considering SLE patients, leptin levels correlate with vascular stiffness (r = 0.64, p < 0.0001) and PSEM (r = 0.63, p < 0.0001). Adiponectin levels correlate with vascular strain (r = 0.28, p 0.039) and negatively correlate with vascular stiffness (r = -0.38, p 0.039). Leptin levels correlate with disease activity (SLEDAI and ECLAM) and cumulative damage (SLICC) indexes. This study demonstrates higher values of leptin in SLE patients. Moreover, SLE patients show increased levels of vascular stiffness and PSEM and reduced values of vascular strain and distensibility. These results globally indicate a decline in arterial elasticity. We find a positive correlation of leptin with stiffness parameters. According to its atheroprotective action, adiponectin inversely correlates with stiffness parameters.

Ultrasound (US) may be used to diagnose chronic worsened gout. US confirmed the clinical evidence of tophaceous deposits in the left elbow of a 75-year-old man; it also identified crystalline materials on the cartilage surface of the second metacarpophalangeal joint of the left hand. US may be helpful to detect signs of deposition of monosodium urate crystals in periarticular and intra-articular joints of patients with clinically suspected chronic worsened gout.

Familial Mediterranean fever (FMF) is a recessive, autosomal, auto-inflammatory disorder characterised by brief, recurring, self-limited episodes of fever and serositis resulting in abdominal, chest, joint and muscular pain; it is the most common of the periodic hereditary fevers and mostly affects Mediterranean populations. Daily administration of colchicine, a tricyclic alkaloid with anti-microtubule and anti-inflammatory properties, prevents the recurrence of FMF attacks and the development of secondary (AA) amyloidosis, the major long-tem complication of FMF. Colchicine is generally safe and well-tolerated; nevertheless, 5-10 % of FMF patients do not respond to conventional treatment, while another 2-5 % of patients are colchicine-intolerant because of toxicity issues, leading physicians to search for alternative therapeutic strategies. Recent new insights into the mechanisms of auto-inflammation add further proof to the efficacy of IL-1 targeting drugs in colchicine non-responder/intolerant FMF patients. A systematic study of relevant literature through PubMed/Medline was performed in order to identify publications reporting IL-1β biological treatment of FMF. Treatment methods, comorbidities, clinical response and side effects in literature case reports were analysed, as well as recent advances in the pathogenesis of auto-inflammation mechanisms in FMF and the causes of colchicine resistance or toxicity in common clinical practice. The paradigmatic experience of an FMF patient with severe FMF mutations (M694V/M694V) suffering from colchicine toxicity and successfully treated with anakinra is also reported. The present data show that anti-IL-1β biological treatment is actually a therapeutic option for FMF patients unresponsive or intolerant to colchicine or in FMF patients with concomitant vasculitis.

Over the last decade, a growing number of studies have been published supporting the high prevalence of atherosclerosis in rheumatic patients with chronic inflammatory diseases. Ultrasound (US) represents the imaging technique of choice for revealing early atherosclerotic changes at carotids level. New US software, mainly developed to rectify its operator dependence, requires a comparison with conventional technique. The main aim of the present study was to compare conventional and software-guided US in the assessment of intima-media thickness (IMT) of common carotids. Thirty-two consecutive patients with chronic inflammatory rheumatic conditions, presenting at the outpatient clinics of the Rheumatology Department--Università Politecnica delle Marche, Italy--were enrolled in the present study. The patients underwent a US assessment of the IMT of the posterior wall of common carotids using a My Lab 70 XVG system (Esaote SpA, Genoa, Italy) equipped with a 4-13 MHz linear probe. The US examinations were performed by two sonographers. One of them used the conventional manual approach and the second sonographer adopted a software-guided technique (named RF-QIMT technology). Inter-observer reliability was determined using intra-class correlation coefficient (ICC) and the Bland-Altman plot. Agreement between the two methods was high with ICC value of 0.74 (0.69 and 0.77 for the right and left common carotid, respectively). The Bland-Altman plot showed minimal proportional error and high limits of agreement. Substantial inter-observer agreement rates for the assessment of the IMT were achieved. Further studies are encouraged to confirm these results in a larger cohort of patients.

Interruption or hypoplasia of the inferior vena cava, with associated azygos continuation, is an uncommon congenital vascular malformation (Ellis et al, Comput Radiol 1986;10:15-22). Although this anomaly causes venous stasis, few patients present with history of deep vein thrombosis (DVT). The exact role of coexisting thrombophilic gene mutations, also heterozygotic, is far from being completely understood. However, in these cases, because of a probable additive effect, treatment of complications and careful prophylaxis for recurrent DVT are recommended lifelong. The authors report a case of inferior vena cava interruption with azygos continuation in a 30-year-old woman who presented with a history of recurrent lower limb DVT. In addition, heterozygosis for the H1299R polymorphism of the factor V gene (Factor V HR2), for the C677T polymorphism of the methylenetetrahydrofolate reductase gene (MTHFR C677T) and for the 4G/5G polymorphism of the plasminogen activator inhibitor-1 gene (PAI-1 4G/5G) was found in DNA analyses.

Non-cirrhotic portal hypertension is a poorly understood condition characterized by portal hypertension in the absence of conventional hepatic cirrhosis and described in association with blood coagulation disorders, myeloproliferative and immunological diseases and with exposure to toxic drugs. Very recently, precise classification criteria have been proposed in order to define four distinct subcategories. The present case highlights how the clinical presentation, the confounding results from imaging studies, and the difficulties in the histological evaluation often render cases of non-cirrhotic portal hypertension a real diagnostic challenge. It also underscores the classification problems which can be faced once this diagnosis is performed. Indeed, the different subcategories proposed result from the prevalent subtypes in a spectrum of hepatic regenerative responses to a variety of injuries determining microcirculatory disturbances. More flexibility in classification should derive from this etiopathogenic background.

Leptin is one of the most important hormones secreted by adipocytes, with a variety of physiological roles related to the control of metabolism and energy homeostasis. Since its discovery in 1994, leptin has attracted increasing interest in the scientific community for its pleiotropic actions. One of these functions is the relationship between nutritional status and immune competence. It structurally resembles proinflammatory cytokines, such as IL-6 and IL-12. The cytokine-like structural characteristic of leptin is implicative of its function in regulating immune responses. The role of leptin in regulating immune responses has been assessed in vitro as well as in clinical studies. It has been shown that disease conditions of reduced leptin production are associated with increased infection susceptibility. Conversely, immune-mediated disorders, such as autoimmune diseases, are associated with the increased secretion of leptin and the production of proinflammatory pathogenic cytokines. In this paper, we review the most recent advances of the role of leptin in immune-rheumatological diseases, and we discuss whether strategies aimed at modifying leptin levels could represent innovative and therapeutic tools for autoimmune disorders.

Objectives: An Italian multicentre study was promoted in order to assess the accuracy of four anti-double-stranded DNA (dsDNA) antibody assays for SLE diagnosis and monitoring., Methods: Two hundred and twenty-three patients with established SLE according to ACR classification criteria were enrolled from 9 centres. They included 59 patients at first evaluation (disease duration <12 months) and 164 with longer disease duration (median disease duration 120 months). The sera from 55 healthy subjects and 161 patients with rheumatic, infectious or neoplastic diseases were tested as controls. SLE activity was measured by ECLAM score. Anti-dsDNA antibodies were detected in serum by means of FarrzymeTM assay, fluoroenzymeimmunoassay (EliATM), Crithidia luciliae indirect immunofluorescence (CLIFT) or Farr radioimmunoassay (Farr). Cut-off values of quantitative assays were chosen by ROC curves analysis. Statistics were conducted by SPSS software package., Results: Sensitivity for SLE diagnosis ranged between 66% with Farrzyme to 95% with Farr, with about 90% specificity for all the methods tested. Farrzyme assay was more specific than the others towards patients with non-SLE connective tissue disease. The four methods were moderately concordant and correlated among them, all showing a positive association with active disease, renal or haematologic involvement, and a negative association with central nervous system disease. Whatever the assay used, anti-dsDNA antibody levels correlated with disease activity with r correlation coefficients ranging from 0.336 to 0.425 (p<0.0001)., Conclusions: The diagnostic accuracy for SLE of evaluated anti-dsDNA antibody assays is comparable and potentially improvabile especially in terms of specificity. The clinical adherence of the assays confirms the value of anti-dsDNA antibody for SLE monitoring.

The progress of research on the molecular pathogenesis of liver fibrosis and the consequent discoveries are likely to open new possibilities for therapeutic approaches to the management of this disease in the future. A key step towards this goal is a deeper comprehension of both the complex molecular and cellular mechanisms and the signaling involved in the development of hepatic fibrosis. It is not yet clear, in fact, what role apoptosis, cytokines, oxidants and other molecules play and what relationships exist between them in favouring or delaying the onset of these adverse mechanisms. At present, a unique mechanism is recognized to be the main reason for the cause and development of liver fibrosis: sustained hepatic stellate cell activation and transformation. Therefore, in this review, after considering the cause, development of fibrosis and interrelation between molecular and cellular profibrotic mechanisms, the part played in counteracting both of these actions by some anti-oxidants and anti-fibrotic molecules such as cytokines, prostacyclin and others will be taken into consideration. The gene therapy and the possible therapeutic use of liver stem cells and tissue engineering will also be dealt with briefly. At the moment, however, the efficacy of these novel strategies still needs to be further validated in animal studies and confirmed in clinical trials. Some data that are already available from in vitro and animal studies demonstrating the effectiveness of novel approaches to inhibiting or treating liver fibrosis can only offer moderate hope.

Background: Iloprost, a prostacyclin analog, reduces hepatic microcirculatory damage after ischemia-reperfusion injury in animal liver models. The objective of this study was to evaluate whether the portal flow velocity changes after Iloprost infusion in patients with systemic sclerosis and Raynaud's phenomenon, who usually have increased risk of microvascular thrombosis and transient liver disturbances., Patients and Methods: Fifteen patients (3 males and 12 females, median age 58 years, range 47-66 years), with systemic sclerosis and Raynaud's phenomenon, were exclusively treated with an infusion of Iloprost (2 ng/kg/min, 6 h/day) for 5 days. In each subject, the portal flow velocity (PV, cm/sec) and portal flow volume (PFV, mL/min) were obtained by using portal color Doppler ultrasonography equipment., Results: Iloprost administration significantly (p<0.001) increased both the PV (23.6+/-3.4 cmlsec vs. 29.1+/-3.9 cm/sec) and PFV (1748.8+/-310. 7 mL/min vs. 2254.9+/-404.1 mL/min) values., Conclusion: Hepatic perfusion significantly improved after Iloprost administration, suggesting that such treatment might be useful in preventing vascular complications in patients with systemic sclerosis. Iloprost improves the portal hemodynamics, favoring local microvascular patency, and its effectiveness may be safely monitored by using portal color Doppler ultrasonography.

Objective: To quantitatively evaluate the diagnostic accuracy of antibodies to ribosomal P proteins (anti-P) for neuropsychiatric systemic lupus erythematosus (NPSLE) in general, for psychosis, mood disorder, or both, and for other diffuse manifestations., Methods: This international meta-analysis combined standardized data from 1,537 lupus patients contributed by 14 research teams. Weighted estimation of sensitivity and specificity with fixed-effects and random-effects models, as well as summary receiver operating characteristic (SROC) curve analysis, was used to summarize test performance. The robustness of the overall estimates was examined in sensitivity analyses that included additional studies published up to November 1, 2004 in the Medline, EMBase, and Cochrane databases., Results: Combining the data from the 14 teams, the weighted sensitivity and specificity estimates for the diagnosis of NPSLE were 26% (95% confidence interval [95% CI] 15-42%) and 80% (95% CI 74-85%), respectively. For psychosis, mood disorder, or both, the sensitivity and specificity were 27% (95% CI 14-47%) and 80% (95% CI 74-85%), respectively. For other diffuse manifestations, the sensitivity was 24% (95% CI 12-42%), and the specificity was 80% (95% CI 73-85%). The proportion of patients with anti-P antibodies did not vary markedly across different presentations of NPSLE. Between-study heterogeneity was substantial, but the SROC curves were consistent with the weighted estimates. In further analyses that included another 24 published studies, only the sensitivity for psychosis and/or mood disorder was slightly improved, but it was still suboptimal (42% [95% CI 30-53%]); the specificity remained essentially the same (81% [95% CI 76-85%])., Conclusion: Anti-P antibody testing has limited diagnostic value for NPSLE, and it is not helpful in differentiating among various disease phenotypes.

Objective: To investigate the thrombotic tendency in patients with systemic lupus erythematosus (SLE) by evaluating congenital or acquired abnormalities associated with an increased risk of venous and/or arterial thrombosis., Methods: A total of 57 patients with SLE were included in the study. Twenty-one patients (37%) had a history of arterial and/or venous thrombosis and 36 patients (63%) did not have such a history. Sera from 50 healthy controls were examined. Protein C, protein S, antithrombin, D-dimer, fibrinogen, homocysteine, anticardiolipin antibodies (aCL), lupus anticoagulant (LAC), prothrombin G20210A, and methylenetetrahydrofolate reductase (MTHFR) C677T gene mutation were evaluated., Results: Protein C, antithrombin, fibrinogen, D-dimer, and homocysteine levels were significantly higher in patients with SLE than in controls. A prothrombin mutation was observed in 2 (4%) of 50 controls and in 6 (11%) of 57 patients. A significantly higher prevalence (P = 0.036) of MTHFR homozygous mutation was observed in patients with SLE (14 [25%] of 57) in comparison with controls (4 [8%] of 50). IgG-aCL and IgM-aCL levels were significantly higher in patients with SLE than in controls (P < 0.0001). The presence of medium-high (> or = 20 IgG phospholipid units/ml) IgG-aCL antibody titers was significantly higher (P = 0.005) in patients with thrombosis (11 [52%] of 21) than in patients without (5 [14%] of 36) thrombosis. LAC was present in 22 (38.5%) of 57 patients and in none of 50 controls., Conclusion: In this study, we confirm the association between thrombosis and IgG-aCL at medium-high titers and suggest that the coexistence of other risk factors can affect the expression of thrombosis in patients with SLE.

Chronic viral liver disease may evolve to cirrhosis. The medical treatment to slow down this passage is based on anti-viral and anti-fibrotic properties of interferon. Recently, we evidenced significant increase of portal vein flow velocity and volume after a prostacyclin analog (iloprost) infusion in subjects without and with chronic viral hepatitis. On the basis of these results and considering both the pathophysiology of viral liver disease and the mechanism of action of iloprost in portal microcirculation, we hypothesize that it may be of some efficacy in chronic liver disease ameliorating the portal hemodynamics and producing an anti-oxidant liver effect.

Background/aims: Experimental radiofrequency ablation has already been performed in healthy livers of porcine models, but not in less expensive and easy-to-manage rats, with devices capable of delivering radiofrequency ablation in the 20-30 g liver of such small animals being so far unavailable., Methodology: We experimented with a modified system of radiofrequency ablation of liver tissue in rat models developing a custom-made needle-microelectrode of very small dimensions (0.3x2 mm) and an electrode-tip cooling technique, based on saline solution infusion. We adjusted duration (seconds) and power (watts) of radiofrequency ablation letting them range between 5-50 seconds and 5-25 W, respectively, to obtain the greatest lesions with the least side effects. After sacrificing the animals, an accurate histological examination of the liver was made., Results: It is possible to establish beforehand the diameter of thermal liver lesion on the basis of joules of applied energy. The greatest increase of liver thermal lesion diameter (8 mm) is obtained with a 250-joule (10 W for 25 seconds) thermal energy cooling the electrode-tissue interface., Conclusions: Experimental radiofrequency ablation in rat liver is an effective and cheap way to study its effects on healthy hepatic tissues. It might be the first step to treat experimentally caused liver tumors.

Liver cirrhosis is characterized by a severe impairment of the growth hormone/insulin-like growth factor-1 (GH-IGF-1) axis, that is, acquired GH resistance. The condition of the GH-IGF-1 axis in the phase of chronic liver disease (CLD) preceding cirrhosis, however, remains uncertain. The origin of GH resistance during CLD is multifactorial, and to date, the liver functional mass is considered to play a major role. Although proinflammatory cytokines, tumor necrosis factor-alpha (TNF-alpha), interleukin-6 (IL-6), and IL-1beta, were found to be elevated in patients with CLD and were shown to induce a state of GH resistance in other disease models, their involvement in the pathogenesis of GH resistance during CLD has never been investigated. We characterized the GH-IGF-1 axis by analyzing the individual components of the axis (GH, IGF-1, IGF-binding protein-3 [IGFBP-3], acid-labile subunit [ALS]) and the corresponding ratios (GH/IGF-1, GH/IGFBP-3, and GH/ALS) and verified the links with circulating proinflammatory cytokines (TNF-alpha, IL-1beta, and IL-6), in 34 patients with CLD and 12 healthy controls. Evolution of CLD from chronic hepatitis (CH, n = 17) to cirrhosis (CIR, n = 17) was associated with a progressive increase of GH resistance indices (e.g., GH/IGF-1 ratio: controls 0.5 +/- 0.9, CH 15.9 +/- 31.2, p < 0.01 vs. controls; CIR 188.4 +/- 282.7 mU/nmol, p < 0.001 vs. CH and controls), indicating its onset also in the early stages of CLD. The progressive increase in GH resistance indices matched the increase of circulatory TNF-alpha (e.g., TNF-alpha vs. GH/IGF-1, r = 0.54, p < 0.001). A similar trend was found for IL-6 without reaching statistical significance (r = 0.23, p = 0.13). We found undetectable levels of IL-1beta in our sample of patients and controls. We conclude that proinflammatory cytokines play an important role in the pathogenesis of GH resistance in CLD, but TNF-alpha is a major factor. In addition, GH resistance is present in CLD from the early stages. These results could begin new therapeutic lines of attack in the management of CLD.

In this study, we evaluated the prevalence and association with thrombosis and/or thrombocytopenia of IgG and IgM antibodies to cardiolipin (aCL), phosphatidic acid (aPA), phosphatidylinositol (aPI), phosphatidylserine, and beta(2)-glycoprotein I (abeta(2)-GPI) in systemic lupus erythematosus (SLE). Sera were obtained from 87 patients affected by SLE (77 of the 87 patients were females), 41 of them with a history of arterial and/or venous thrombosis. Antiphospholipid antibodies and abeta(2)-GPI were evaluated by enzyme-linked immunosorbent assay. IgG-aCL, IgG-aPA, IgG-aPI, IgG-aPS, and IgG-abeta(2)-GPI were found in 53%, 37%, 32%, 38%, and 24% of patients, respectively. IgM-aCL, IgM-aPA, IgM-aPI, IgM-aPS, and IgM-abeta(2)-GPI were detected in 15%, 17%, 18%, 14%, and 16%, respectively. With respect to antibody titer, IgG-aCL strongly correlated with all other antiphospholipid antibodies and abeta(2)-GPI of IgG isotype. Thrombosis was significantly associated with IgG-aPA (p = 0.044), IgG-aPI (p = 0.038), IgG-aPS (p = 0.026), IgG-abeta(2)-GPI, IgM-aPA (p = 0.044), IgM-aPI (p = 0.024), and IgM-aPS (p = 0.01), irrespective of antibody titer, whereas IgG-aCL were associated with thrombosis and thrombocytopenia when taken at medium-high titer (p = 0.009 and p = 0.046, respectively). Our results confirm that, besides aCL and abeta(2)-GPI, other antibodies to negatively-charged phospholipids are present in a large percentage of patients with SLE. However, it remains doubtful whether these other antiphospolipid antibodies actually represent an important parameter predictive of thrombosis and thrombocytopenia in SLE.

Objective: We studied, with color Doppler sonography, portal flow velocity (PV) and volume (PFV) before and after Iloprost infusion., Background: Iloprost is a prostacyclin analogue with arterial vasodilator and platelet aggregation inhibitor properties. Recently, hemodynamic effects after treatment with Iloprost have been demonstrated in subjects with arteriopathy of lower limbs., Methods: We treated 10 subjects (2 males and 8 females; mean age 64 +/- 8.2 years) affected by arteriopathy of lower limbs with intravenous infusion of Iloprost, at a dosage of 2 ng/Kg/min (16 hours/day) for 3 days. In all patients portal vein flow velocity (PV) (cm/s) and volume (PFV) (ml/min) were assessed. PV was directly determined by the Doppler system, whereas PFV was calculated using the formula "CSA x PV", after measuring the portal vein cross sectional area (CSA) (mm2)., Results: The patients showed markedly increased PV and PFV after Iloprost infusion (pre-Iloprost vs post-Iloprost treatment mean portal flow velocity and volume values: 23.12 +/- 3.89 cm/s vs 28.49 +/- 3.90 cm/s, p < 0.01 and 1743.9 +/- 241.7 ml/min vs 2271.7 +/- 333.5 ml/min, p < 0.001, respectively)., Conclusions: This study confirms our previous results about increased PV and PFV values after Iloprost treatment. In the light of these results we suggest some possible therapeutic implications in patients undergoing liver transplantation. However, further studies are necessary to confirm this hypothesis.

The idiopathic hypereosinophilic syndrome is characterized by a prolonged overproduction of eosinophils of unknown cause in addition to specific organ damage due to eosinophil-derivated protein toxicity. Its prognosis is correlated with heart involvement that results in a restrictive cardiomyopathy. This is frequently biventricular. Isolated left ventricular infiltration has rarely been reported. The authors report a case of idiopathic hypereosinophilic syndrome with cardiac involvement obliterating the left ventricular cavity with a favorable clinical outcome following a combination therapy with interferon-alpha, hydroxyurea and prednisone. Data from other studies dealing with the treatment of idiopathic hypereosinophilic syndrome with interferon-alpha are reviewed and discussed.