Multidrug-resistant cytomegalovirus infection in a patient with granulomatosis with polyangiitis during immunosuppressive treatment.

Cytomegalovirus (CMV) infection is a major complication in immunocompromised patients, including those with autoimmune diseases. Here, we describe the first case of granulomatosis with polyangiitis treated with steroids and cyclophosphamide, complicated by a multidrug-resistant (MDR) CMV infection in presence of weak antiviral cellular immunity. Since reports regarding CMV infection in rheumatological patients are rarely described and no guidelines on its management exist, the described case contributes to identify potential strategies to predict the risk of CMV disease and developing of MDR-CMV in these patients, through virological and immunological surveillance.

Serum Jo-1 Autoantibody and Isolated Arthritis in the Antisynthetase Syndrome: Review of the Literature and Report of the Experience of AENEAS Collaborative Group.

Anti-Jo-1 is the most frequently detectable antibody in the antisynthetase syndrome (ASSD), an autoimmune disease characterized by the occurrence of arthritis, myositis, and interstitial lung disease (ILD). Recently, we organized an international collaborative group called American and European NEtwork of Antisynthetase Syndrome (AENEAS) for the study of this rare and fascinating disease. The group collected and published one of the largest series of ASSD patients ever described and with one of the longer follow-up ever reported.

Clinical Spectrum Time Course in Anti Jo-1 Positive Antisynthetase Syndrome: Results From an International Retrospective Multicenter Study.

Anti Jo-1 antibodies are the main markers of the antisynthetase syndrome (ASSD), an autoimmune disease clinically characterized by the occurrence of arthritis, myositis, and interstitial lung disease (ILD). These manifestations usually co-occur (for practical purpose complete forms) in the same patient, but cases with only 1 or 2 of these findings (for practical purpose incomplete forms) have been described. In incomplete forms, the ex novo occurrence of further manifestations is possible, although with frequencies and timing not still defined.

CC chemokine receptor 5 polymorphism in Italian patients with Behcet's disease.

Objective: To evaluate the potential role of CC chemokine receptor 5 (CCR5)Δ32 polymorphism in the susceptibility to and clinical expression of Behçet's disease (BD) in a cohort of Italian patients.

Methods: One hundred and ninety-six consecutive Italian patients satisfying the ISG criteria for BD were followed up for 8 years, and 180 healthy age- and sex-matched blood donors were molecularly genotyped for the CCR5Δ32 polymorphism. A standard microlymphocytotoxicity technique was used to serotype HLA-B51.

PLA1/A2 polymorphism of the platelet glycoprotein receptors IIIA in Behçet's disease.

Objectives: To investigate potential associations between the PlA1/A2 polymorphism of the platelet glycoprotein receptor IIIA (GpIIIa) gene and venous thrombosis and other clinical manifestations in Italian patients with Behçet's disease (BD).

Methods: Two hundred consecutive Italian patients satisfying the International Study Group criteria for BD who were followed up for seven years and 241 healthy Italian age- and gender-matched blood donors were molecularly genotyped for the PlA1/A2 polymorphism of the platelet GpIIIa gene; 118 and 117 of the 200 BD patients were also respectively genotyped for factor V Leiden and prothrombin gene G20210A polymorphisms. A standard microlymphocytotoxicity technique was used to type serological HLA class B51.

Epidemiology of systemic sclerosis in a district of northern Italy.

Objectives: To estimate, using both the American College of Rheumatology-ACR 1980 classification criteria and revised LeRoy and Medsger 2001 criteria, the incidence and prevalence of systemic sclerosis (SSc) in an area in north-eastern Italy with a referral base population of about 346,000 inhabitants.

Methods: Retrospective examination of all patients 16 years and older of native Italian origin and resident in the Ferrara district who had either been admitted to hospital or referred to our outpatient clinic with a diagnosis of SSc between 1st January 1999 and 31st December 2007. SSc subjects were identified both by a search of hospital discharge code 710.

Glucocorticoid receptors and bone.

Glucocorticoid Receptors (GRs) have been identified in all bone cells. The molecular structure of human GR is organized into 3 major functional domains: the N-terminal immunogenic domain, the central DNA-binding domain and the C-terminal ligand-binding domain. Human GR is a product of a gene composed of 10 exons, located in the chromosome 5q31-32.

Bronchoalveolar lavage in systemic sclerosis with lung involvement: role and correlations with functional, radiological and scintigraphic parameters.

To evaluate the role and the prognostic value of bronchoalveolar lavage (BAL) in scleroderma patients with interstitial lung disease. We reviewed the records of 79 patients with systemic sclerosis (SSc) who had dyspnea and pulmonary involvement and underwent BAL study. Sixty-two patients were prospectively followed up for 12-36 months and re-evaluated by pulmonary function tests (PFTs).

The tumour necrosis factor-related apoptosis-inducing ligand-osteoprotegerin system in limited systemic sclerosis: a new disease marker?

Objective: To investigate the role of the TNF-related apoptosis-inducing ligand-osteoprotegerin (TRAIL-OPG) system in the pathogenesis of limited SSc (lSSc).

Methods: Circulating levels of TRAIL and of its soluble receptor OPG were measured by ELISA in serum samples obtained from 50 lSSc patients and 50 healthy controls.

Results: TRAIL serum levels in lSSc patients were similar to those of healthy controls, whereas the OPG serum levels were significantly increased (P < 0.

Nicotine-patch therapy on mucocutaneous lesions of Behcet's disease: a case series.

Objective: We report the use of nicotine-patch therapy on active mucocutaneous lesions of Behçet's disease (BD).

Methods: Five BD ex-smoker patients with refractory active mucocutaneous manifestations were treated with nicotine patches for 6 months.

Results: Four out of five patients quickly responded to nicotine-patch therapy and experienced a complete regression of mucocutaneous lesions.

Dysfunctional inflammasome in Schnitzler's syndrome.

Objective: IL-1beta plays a key role in the pathogenesis of Schnitzler's syndrome (SS). We have investigated inflammasome activity in peripheral blood mononuclear cells (PBMCs) from a patient affected by a variant type of SS.

Methods: PBMCs were purified by Ficoll and examined for ability to secrete IL-1beta and -18, expression and function of the P2X(7) receptor and expression of apoptosis-associated speck-like protein containing a caspase recruitment domaine (ASC) and NOD-like receptor protein 3 (NLRP3) before and after the therapy with steroid.

Wegener's granulomatosis and giant cell tumor of tendon sheaths: casual or causal combination? First case report.

Giant cell tumor of tendon sheath (GCTTS) and Wegener's granulomatosis (WG) are rare conditions both characterized by polyclonal cellular proliferation and multinucleated giant cells formation. Here, we report the case of a 27-year-old Caucasian woman affected by WG who experienced the metachrone appearance of two different GCTTSs at the right hand within a time of 3 years. To our knowledge, the combination of GCTT with WG is exceptional and this could probably be the first case reported.

Sarcoidosis appearing during anti-tumor necrosis factor alpha therapy: a new "class effect" paradoxical phenomenon. Two case reports and literature review.

Objectives: To report 2 cases of sarcoidosis that developed during treatment with tumor necrosis factor alpha (TNFalpha) antagonists, infliximab and adalimumab, used for inflammatory rheumatic disease and to review previously reported cases.

Methods: We describe 2 patients, the first with psoriatic arthritis, the second with rheumatoid arthritis, who developed noncaseating granulomas of the lungs consistent with sarcoidosis while being treated with anti-TNFalpha drugs. A retrospective review of the literature was performed using the PubMed database.

Abnormalities of left ventricular function in asymptomatic patients with systemic sclerosis using Doppler measures of myocardial strain.

Objective: To verify whether myocardial impairment can be detected by tissue Doppler imaging (TDI) in patients with asymptomatic systemic sclerosis (SSc), 35 patients with SSc with normal left ventricular (LV) ejection fraction and 35 control subjects were studied.

Methods: Myocardial longitudinal peak systolic velocity, strain, and strain rate (SR) were measured by TDI at a regional level, and for each parameter the average value was calculated using an LV 12-segment model. In addition, the mitral annulus diastolic velocities and the E/Ea ratio were obtained.

Paget's disease of bone: a review.

Paget's disease of bone (PDB) is a condition of unknown etiology characterized by excessive and abnormal bone remodeling. It may be localized to one or several skeletal segments. The disease seldom appears before the age of 40 years, but its prevalence tends to double each decade from the age of 50 onwards, reaching about 10% after ninth decade.

CXCR4 pos circulating progenitor cells coexpressing monocytic and endothelial markers correlating with fibrotic clinical features are present in the peripheral blood of patients affected by systemic sclerosis.

There is still controversy regarding the role of circulating endothelial and progenitor cells (CECs/CEPs) in the pathogenesis of systemic sclerosis (SSc). Using a sequential Boolean gating strategy based on a 4-color flow cytometric protocol, an increased number of CD31(pos)/CD184(pos)(CXCR4)/CD34(pos)/CD45(pos) and CD31(pos)/CD117(pos) (c-kit-R) /CD34(pos)/ CD45(pos) hematopoietic circulating progenitor cells (HCPCs) was detected in SSc patients compared with healthy subjects. In SSc, no circulating mature and progenitor endothelial cells were observed, while an enhanced generation of erythroid progenitor cells was found to be correlated with the presence of CD117+ HCPCs.

Neurological involvement in North Italian patients with Behçet disease.

The aim of this study was to evaluate neurological involvement in a series of 110 North Italian patients with Behçet disease (BD), a multisystemic vasculitis of unknown origin, followed up for a period of 5 years. During this time, 27 (24.5%) patients with neuro-BD were identified.

Vascular endothelial growth factor gene polymorphisms in Behçet's disease.

Objective: To evaluate potential associations of vascular endothelial growth factor (VEGF) gene polymorphisms with Behçet's disease (BD) and disease expression.

Methods: Case patients were 122 consecutive Italian patients with BD followed at the Rheumatology, Ophthalmology, and Neurology Units in Bologna, Ferrara, Milano, Palermo, Potenza, Prato, Reggio Emilia, and Trento over a 3-year period (1997-99) and who satisfied the International Study Group criteria for BD. Also selected as a control group were 200 healthy age and sex matched blood donors.

Effect of low dose methotrexate on bone density in women with rheumatoid arthritis: results from a multicenter cross-sectional study.

Objective: To analyze the influence of low dose methotrexate (MTX) on bone using data from a large multicenter, cross-sectional study on bone mineral density (BMD) in women with rheumatoid arthritis (RA).

Methods: We selected 731 female patients with RA divided into 2 groups on the basis of MTX use: never MTX users (n = 485) and MTX users for at least 6 months (n = 246). Demographic, disease, and treatment related variables were collected for each patient.

Factor V Leiden and prothrombin gene G20210A mutations in Italian patients with Behçet's disease and deep vein thrombosis.

Objective: To evaluate the frequency and type of vascular lesions and to study the association of factor V gene G1691A (Leiden) and prothrombin gene G20210A polymorphisms with venous thrombosis in Italian patients with Behçet's disease (BD).

Methods: Included were 118 consecutive Italian BD patients followed over a 3-year period (1997-1999) who satisfied the International Study Group criteria for BD. The control group consisted of 132 healthy Italian blood donors.