Dermoscopy in the differential diagnosis between malar rash of systemic lupus erythematosus and erythematotelangiectatic rosacea: an observational study.

BACKGROUND: Malar rash is one of the three cutaneous diagnostic criteria of systemic lupus erythematosus (SLE). Although its clinical recognition is often straightforward, the differential diagnosis with erythematotelangiectatic rosacea may sometimes be challenging. OBJECTIVE: To describe dermoscopic features of SLE malar rash and investigate the accuracy of dermoscopy for the differential diagnosis with erythematotelangiectatic rosacea. METHODS: A representative dermoscopic image of target areas was evaluated for the presence of specific features. Fisher's test was used to compare their prevalence between the two cohorts, and accuracy parameters (specificity, sensitivity, and positive and negative predictive values) were evaluated. RESULTS: Twenty-eight patients were included in the analysis, of which 13 had SLE malar rash and 15 erythematotelangiectatic rosacea. The main dermoscopic features of malar rash were reddish/salmon-coloured follicular dots surrounded by white halos ('inverse strawberry' pattern), being present in 53.9% of the cases, while network-like vessels (vascular polygons) turned out to be the main feature of erythematotelangiectatic rosacea, with a prevalence of 93.3%. The comparative analysis showed that the 'inverse strawberry' pattern was significantly more common in SLE malar rash, with a specificity of 86.7%, while vascular polygons were significantly more frequent in rosacea, with a specificity of 92.3%. CONCLUSION: Dermoscopy may be a useful support to distinguish SLE malar rash and erythematotelangiectatic rosacea by showing peculiar features.

Neumonía eosinofílica aguda: reporte de un caso / Acute eosinophilic pneumonia: case report

Since the initial description as an independent entity in 1989, there are various patients reported in the literature as suffering acute eosinophilic pneumonia. Despite its recognition as etiology of acute respiratory insufficiency, at present still remains weak the ability for diagnosis and treatment, in the opportune moment. The clinical features of a patient that followed algorthms of diagnosis, for acute repiratory insufficiency, are presented. The treatment employed is describe in the article.

Suprarenal inferior vena cava filter implantation.

PURPOSE: This study evaluated the feasibility, safety, and efficacy of the suprarenal implantation of a retrievable filter in patients with renal cell carcinoma (RCC) and renal vein thrombosis (RVT) [extending or not extending to the inferior vena cava (IVC)] undergoing surgery. MATERIALS AND METHODS: Between March 2005 and May 2010, 13 patients (eight men and five women; mean age 67.08 years, range 38-95) with RCC and RVT associated or not with IVC thrombosis underwent implantation of a retrievable suprarenal IVC filter. All patients underwent computed tomography angiography (CTA), which documented RVT and in some cases its extension to the IVC. The level of IVC involvement by the neoplastic thrombus was evaluated on the basis of the Oto classification. Cavography was performed before and after filter implantation. Surgical resection of RCC was performed in all patients. A CTA scan was performed 1 week before filter removal. RESULTS: The procedure had 100% feasibility. All filters were correctly deployed in the suprarenal tract of the IVC. There was no evidence of peri-or postprocedural complications. All patients were monitored for clinical symptoms of pulmonary embolism (PE). There was no evidence of PE in the 30 days after the procedure. All suprarenal IVC filters were removed from 30 to 60 days after surgery. CONCLUSIONS: Implantation of a temporary suprarenal IVC filter is an additional and feasible procedure that can prevent immediate and perioperative PE.

Treatment of inclusion body myositis with cyclosporin-A or tacrolimus: successful long-term management in patients with earlier active disease and concomitant autoimmune features.

OBJECTIVE: Sporadic inclusion body myositis (s-IBM) is a chronic, progressive, inflammatory myopathy of unknown aetiology, generally resistant to immunosuppressive therapy. Given that lymphocyte infiltrates in s-IBM muscle tissue are CD8+ T cells, targeting these cells may represent a valid approach. PATIENTS AND METHODS: Three patients with biopsy-proven s-IBM, high creatine kinase levels at diagnosis, two of whom with associated immune disorders, were treated with either cyclosporin-A (CyA) or tacrolimus, in combination with high doses of corticosteroids (CS), followed by rapid CS tapering. Clinical assessment and laboratory evaluation were performed every three months for the first year and then every six months for the second year. RESULTS: Based on muscle strength assessment and muscle enzyme serum levels, a major clinical response was observed at month +3 in two out of the three patients. A complete clinical response and major clinical response were obtained at month +6, in two and one patient, respectively. Normalization of serum muscle enzymes was observed in all. Steroids could be tapered to very low doses in all patients and were suspended early in one. Laboratory, but not clinical relapse occurred in one patient and was controlled by increasing the CyA dose. Treatment was well tolerated, with no serious adverse events occurring. All three patients are maintaining immunosuppressive therapy. CONCLUSION: Calcineurin inhibitors may represent a useful option for the long-term management of s-IBM, possibly in a subset characterized by a short duration with high disease activity or associated autoimmune manifestations.

B-Lymphocyte stimulator (BLyS) up-regulation in mixed cryoglobulinaemia syndrome and hepatitis-C virus infection.

OBJECTIVES: To investigate the role of B-Lymphocyte stimulator (BLyS) in mixed cryoglobulinaemia syndrome (MCsn), a systemic vasculitis associated with a high risk to develop lymphoma, since BLyS up-regulation may favour both autoimmunity and lymphoproliferation. METHODS: BLyS serum levels were analysed by enzyme-linked immunosorbent assay (positive when >0.85 ng/ml) in 66 patients with MCsn, 54 (81.8%) of whom were positive for hepatitis-C virus (HCV) infection. Thirty-three HCV-positive patients without MCsn were also studied. Patients were compared with 48 healthy blood donors (HBDs). BLyS modifications after antiviral therapy were also studied. RESULTS: A significantly higher frequency of BLyS serum positivity was detected both in MCsn patients and in HCV-positive patients without MCsn (37.9 and 30.3%, respectively) when compared with HBDs (4.2%) (P < 0.0001 vs MCsn and P = 0.0026 vs HCV-positive patients without MCsn, respectively). BLyS appeared significantly higher in MCsn (3.70 +/- 2.97 ng/ml) than in HCV-positive patients without MCsn (1.56 +/- 0.63 ng/ml; P = 0.0044). BLyS expression did not correlate with rheumatoid factor levels, cryoglobulin levels or definite MCsn-related systemic features. High BLyS levels were significantly associated only with MCsn-related overt lymphoproliferative disorder. Finally, antiviral treatment significantly increased BLyS levels, independently from HCV-RNA negativization. However, BLyS normalization was noticed after both HCV-RNA negativization and suspension of antiviral therapy by preliminary data. CONCLUSIONS: BLyS is up-regulated and may play a pathogenetic role in a fraction of patients with MCsn, similarly to other autoimmune diseases. HCV infection likely represents the early event leading to BLyS up-regulation in this setting. BLyS is up-regulated during antiviral treatment. Overall, these data provide new insights for BLyS and virus-related autoimmunity, lymphoproliferation and possible treatment strategies.

Rituximab treatment for glomerulonephritis in HCV-associated mixed cryoglobulinaemia: efficacy and safety in the absence of steroids.

OBJECTIVE: Rituximab, an anti-CD20 monoclonal antibody, has been used in lupus nephritis and membranous idiopathic nephropathy and has proved effective in non-renal manifestations of type II mixed cryoglobulinaemia (MC) syndrome. We investigated the possible efficacy and safety of rituximab in the treatment of cryoglobulinaemic nephritis. METHODS: Five patients with active, biopsy-proven, glomerulonephritis in hepatitis C virus (HCV)-related type II MC syndrome were treated with four weekly infusions of rituximab (375 mg/m2) in monotherapy, without steroids whenever possible. Rituximab was the first-line therapy in three cases. RESULTS: A rapid and sustained renal response was observed in all patients, in one of them without retreatment up to the last follow-up (month 21+). Renal biopsy was repeated after 6 months in one patient and histopathological improvement was documented. Three patients relapsed, at months +5, +7 and +12 of follow-up, respectively. Two of them were then retreated with rituximab and again presented a rapid improvement in renal function. Maintenance therapy with rituximab was performed in two patients: nephritis remission was maintained in both. Fc-gamma receptor 3a (FcgammaRIIIa) genotype characterization was consistent with the clinical response observed. Rituximab also proved effective against other active MC manifestations, when present. No major side-effects occurred and steroids were not required in the follow-up. CONCLUSIONS: Rituximab may provide effective and safe therapy in type II MC-related glomerulonephritis, possibly as first-line therapy, avoiding steroids and hazardous immunosuppressive treatment.

Chemical- or radiation-assisted selective dealloying in bimetallic nanoclusters.

A selective dealloying in bimetallic nanoclusters prepared by ion implantation has been found upon thermal annealing in oxidizing atmosphere or irradiation with light ions. In the first process, the incoming oxygen interacts preferentially with copper promoting Cu2O formation, therefore extracting copper from the alloy. In the second process the irradiation with Ne ions promotes a preferential extraction of Au from the alloy, resulting in the formation of Au-enriched "satellite" nanoparticles around the original AuxCu1-x cluster.

Rescue of combination therapy failures using infliximab, while maintaining the combination or monotherapy with methotrexate: results of an open trial.

OBJECTIVE: To assess the possible clinical and biological rescue of rheumatoid arthritis (RA) in 16 patients who were still active despite intensive combination therapy after receiving infliximab following the Anti-Tumour necrosis factor Trial in Rheumatoid Arthritis with Concomitant Therapy (ATTRACT) schedule. METHODS: Sixteen patients who were still active despite combination therapy with optimal doses of methotrexate (MTX 15-17.5 mg/week) and cyclosporin A (CsA 2.5-3.5 mg/day) received infliximab. Ten received their combination plus infliximab (Combi), and six received infliximab plus MTX alone (Mono). The follow-up was carried out for 30 weeks in all patients and for 46 weeks in eight. Efficacy and safety were examined. RESULTS: At entry, the mean disease activity score (DAS) was 5.6 (all patients had a DAS >3.7). After therapy, eight of 10 patients in Combi and four out of six in Mono showed an improvement of >50% in the initial swollen joint count, yet only one patient reached 50% improvement in the initial DAS after 30 weeks, and one patient had a DAS <2.4 (low disease activity). Of the eight patients who reached 46 weeks of follow-up, three showed an improvement in DAS of 50% and two had a DAS <2.4. When considering the change over time, the difference between DAS at entry and at week 30 was statistically significant only in patients receiving MTX plus CsA, while it was not significant in those receiving MTX only. Two patients developed recurrent febrile upper respiratory infections in the Combi therapy group, while two had a single febrile infection in the MTX alone group. Two patients became strongly anti-cardiolipin positive (IgM >40 MPL) and one developed a coronary syndrome. CONCLUSION: Infliximab can be added incrementally to MTX plus CsA, with favourable results in terms of efficacy and safety over time in severe rapidly aggressive and progressive RA. Finally, minor evidence emerged for a stronger efficacy of the Combi treatment compared with Mono.

[Leptin: regulatory role in bone metabolism and in inflammation]. / La leptina: ruolo regolatorio nel metabolismo osseo e nella flogosi.

Leptin is a peptidic molecule synthesized almost exclusively by adipocytes, that regulates appetite and energy expenditure at the hypothalamic level. In the last few years, further actions have been attributed to this molecule, as modulating the immune response and affecting the bone metabolism. We have reviewed if leptin contributes to the metabolic changes leading to cachexia and to the regulation of inflammation, paying attention to the pathogenetic mechanisms of chronic arthritis. Besides, considering the relationship between body mass index (BMI) e bone mineral density (BMD) and the protective role of the obesity towards osteoporosis, we have analysed the role of leptin on the bone metabolism

980-nm diode laser treatment for recurrent urethral strictures.

OBJECTIVES: Our experience with laser treatment of urethral strictures with a 980-nm diode laser is reported. METHODS: 45 consecutive patients with relapsing urethral strictures (mean age 65, range 25-85 years) were treated between February 1995 and February 1998. The fibrous and scar tissue was cut and vaporized at the 6 o'clock position with the laser beam. RESULTS: 44/45 patients were available for follow-up at 1 year and 32/43 at 2 years. At 12 months the average peak flow rates of the patients was 18 ml/s, and at the second year assessment it was 15.5 ml/s. Of the patients 21/44 (47.7%) were extremely satisfied (0-1 of the quality-of-life, QoL, assessment index) with the procedure, 14/44 (31.8%) were satisfied (2 of the QoL assessment index), and 9/44 (20.4%) were not satisfied (> or = 3 of the QoL assessment index) due to the low flow rate or the recurrence of the stricture. (The sum of the first two categories is 79.5%.) At the second year assessment the percentage of satisfied patients reached 78%, with 71% of the patients stable without any dilatation. CONCLUSIONS: Usually in recurrent strictures of the urethra open surgery is suggested, but we believe that there is still a place for endoscopy. With the laser we can make an incision at the 6 o'clock position without significant bleeding, very easily opening the urethra. We had 79.5 and 71% good results at the 1- and 2-year assessments (mean peak flow rate of 18 and 15.5 ml/min), respectively. To date, no definitive conclusions can be drawn, but we believe that laser treatment is indicated for recurrent strictures in high risk or elderly patients and in those who demand a minimally invasive procedure.

Preliminary classification of nonmalignant B cell proliferation in Sjögren's syndrome: perspectives on pathobiology and treatment based on an integrated clinico-pathologic and molecular study approach.

A classification of nonmalignant lymphoproliferation in Sjögren's syndrome is presented, based on the results of international meetings regarding Sjögren's syndrome-associated lymphomagenesis and on our results of a clinico-pathologic and molecular study and long-term follow-up in well-characterized patients. Sjögren's syndrome pathobiology has similarities to hepatitis C virus-related B-cell lymphoproliferation. Antigen stimulation with the preferential expansion of rheumatoid factor-positive clones and specific immunoglobulin gene expression and recombination represent key biologic events in lymphoproliferation. This classification is based on the coupling of molecular and histological studies and may result in more selective treatment approaches.

[Idiopathic retroperitoneal fibrosis: effectiveness of surgery combined with corticosteroid therapy]. / Fibrosi retroperitoneale idiopatica: efficacia della terapia associata chirurgica e corticosteroidea.

Idiopathic retroperitoneal fibrosis (ERF) is a rare disease characterized by non-neoplastic fibroblastic and inflammatory proliferation. We describe a case of a 58-year-old man presenting with abdominal pain, weight loss and abdominal mass on clinical examination. The patient was evaluated by abdominal CT scan and magnetic resonance imaging which disclosed a periaortic soft-tissue mass with marked longitudinal extension below renal arteries origin. The diagnosis of ERF was histologically confirmed after surgical exploration and biopsies followed by extensive ablation of fibrous tissue. After 6 months of low-doses of postoperative steroid therapy retroperitoneal fibrous tissue had almost disappeared on CT examination.