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  1. Article ; Online: Diagnosis and management of leukocytoclastic vasculitis.

    Fraticelli, Paolo / Benfaremo, Devis / Gabrielli, Armando

    Internal and emergency medicine

    2021  Volume 16, Issue 4, Page(s) 831–841

    Abstract: Leukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. The leading clinical presentation of LCV is ... ...

    Abstract Leukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. The leading clinical presentation of LCV is palpable purpura and the diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments ("leukocytoclasia"). Several medications can cause LCV, as well as infections, or malignancy. Among systemic diseases, the most frequently associated with LCV are ANCA-associated vasculitides, connective tissue diseases, cryoglobulinemic vasculitis, IgA vasculitis (formerly known as Henoch-Schonlein purpura) and hypocomplementemic urticarial vasculitis (HUV). When LCV is suspected, an extensive workout is usually necessary to determine whether the process is skin-limited, or expression of a systemic vasculitis or disease. A comprehensive history and detailed physical examination must be performed; platelet count, renal function and urinalysis, serological tests for hepatitis B and C viruses, autoantibodies (anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies), complement fractions and IgA staining in biopsy specimens are part of the usual workout of LCV. The treatment is mainly focused on symptom management, based on rest (avoiding standing or walking), low dose corticosteroids, colchicine or different unproven therapies, if skin-limited. When a medication is the cause, the prognosis is favorable and the discontinuation of the culprit drug is usually resolutive. Conversely, when a systemic vasculitis is the cause of LCV, higher doses of corticosteroids or immunosuppressive agents are required, according to the severity of organ involvement and the underlying associated disease.
    MeSH term(s) Diagnosis, Differential ; Humans ; Prognosis ; Vasculitis, Leukocytoclastic, Cutaneous/diagnosis ; Vasculitis, Leukocytoclastic, Cutaneous/therapy
    Language English
    Publishing date 2021-03-13
    Publishing country Italy
    Document type Journal Article ; Review
    ZDB-ID 2454173-4
    ISSN 1970-9366 ; 1828-0447
    ISSN (online) 1970-9366
    ISSN 1828-0447
    DOI 10.1007/s11739-021-02688-x
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  2. Article ; Online: Atypical neutrophilic panniculitis as presentation of BCR-ABL1-negative chronic myeloid leukaemia.

    Fraticelli, Paolo / Benfaremo, Devis / Cardinali, Marco / Gabrielli, Armando

    BMJ case reports

    2019  Volume 12, Issue 10

    Abstract: We report the case of an otherwise healthy 60-year-old man presenting with fever, leucocytosis and a painful swelling of the right calf. We initially performed cultural and cytological examination of the popliteal fossa mass, but the results were ... ...

    Abstract We report the case of an otherwise healthy 60-year-old man presenting with fever, leucocytosis and a painful swelling of the right calf. We initially performed cultural and cytological examination of the popliteal fossa mass, but the results were disappointingly inconclusive. The subsequent development of several erythematous subcutaneous nodules, rapidly evolving to broad ulcerative lesions, prompted us to reconsider the clinical setting as a whole, which included fever, marked leucocytosis and multiple subcutaneous nodules. A biopsy of the ulcerative lesions finally led to the diagnosis of neutrophilic panniculitis, which was sustained by a hybrid myelodysplastic/myeloproliferative disorder like BCR-ABL1-negative atypical chronic myeloid leukaemia. The patient was initially treated with high-dose intravenous corticosteroids, resulting in a dramatic improvement of the skin lesions and normalisation of blood tests. Azacytidine treatment was subsequently started, and the haematological disease remained stable.
    MeSH term(s) Administration, Intravenous ; Adrenal Cortex Hormones/administration & dosage ; Adrenal Cortex Hormones/therapeutic use ; Azacitidine/administration & dosage ; Azacitidine/therapeutic use ; Diagnosis, Differential ; Enzyme Inhibitors/administration & dosage ; Enzyme Inhibitors/therapeutic use ; Fusion Proteins, bcr-abl/metabolism ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive/metabolism ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive/pathology ; Male ; Middle Aged ; Neutrophils/pathology ; Panniculitis/etiology ; Panniculitis/pathology ; Skin Neoplasms/pathology ; Skin Ulcer/pathology ; Treatment Outcome
    Chemical Substances Adrenal Cortex Hormones ; BCR-ABL1 fusion protein, human ; Enzyme Inhibitors ; Fusion Proteins, bcr-abl (EC 2.7.10.2) ; Azacitidine (M801H13NRU)
    Language English
    Publishing date 2019-10-08
    Publishing country England
    Document type Case Reports ; Journal Article
    ISSN 1757-790X
    ISSN (online) 1757-790X
    DOI 10.1136/bcr-2019-232005
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  3. Article ; Online: ANCA-negative EGPA: only eosinophils without vasculitis? Insights from anti-T2 biologics.

    Piga, Mario Andrea / Fraticelli, Paolo / Antonicelli, Leonardo / Garritani, Maria Stella / Ghirelli, Giulia / Martini, Matteo / Di Vincenzo, Angelica / Danieli, Maria Giovanna / Moroncini, Gianluca / Bilò, Maria Beatrice

    Frontiers in immunology

    2023  Volume 14, Page(s) 1325299

    Abstract: The pathogenic role of p-ANCA in eosinophilic granulomatosis with polyangiitis (EGPA) is a long-standing matter of debate. In this work, we report our real-life experience with EGPA patients, treated with biologics targeting type 2 (T2)-eosinophilic ... ...

    Abstract The pathogenic role of p-ANCA in eosinophilic granulomatosis with polyangiitis (EGPA) is a long-standing matter of debate. In this work, we report our real-life experience with EGPA patients, treated with biologics targeting type 2 (T2)-eosinophilic inflammation (Mepolizumab, Benralizumab, Dupilumab). Interestingly, we observed EGPA extrarespiratory relapses only in p-ANCA-positive patients (2/5 cutaneous vasculitis, 3/5 constitutional symptoms), with new rise of p-ANCA and normal eosinophil blood count. Notably, revising our cohort with the new ACR 2022 criteria, these five patients were the only ones to satisfy the entry criterion of vasculitis' defined diagnosis at disease onset. These observations may suggest that biologics, selectively turning off T2 inflammation, may have unmasked p-ANCA exclusive role in the pathogenesis of vasculitis in EGPA. Therefore, we raise the question whether EGPA vasculitis exists only in p-ANCA-positive patients, and whether p-ANCA-negative disease is "only eosinophils without vasculitis".
    MeSH term(s) Humans ; Eosinophils/pathology ; Antibodies, Antineutrophil Cytoplasmic ; Churg-Strauss Syndrome ; Granulomatosis with Polyangiitis/diagnosis ; Biological Products ; Inflammation/pathology
    Chemical Substances Antibodies, Antineutrophil Cytoplasmic ; Biological Products
    Language English
    Publishing date 2023-11-27
    Publishing country Switzerland
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2023.1325299
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  4. Article ; Online: Cluster Analysis to Explore Clinical Subphenotypes of Eosinophilic Granulomatosis With Polyangiitis.

    Rubenstein, Emma / Maldini, Carla / Vaglio, Augusto / Bello, Federica / Bremer, Jan Phillip / Moosig, Frank / Bottero, Paolo / Pesci, Alberto / Sinico, Renato Alberto / Grosskreutz, Julian / Feder, Claudia / Saadoun, David / Trivioli, Giorgio / Maritati, Federica / Rewerska, Barbara / Szczeklik, Wojciech / Fraticelli, Paolo / Guida, Giuseppe / Gregorini, Gina /
    Moroncini, Gianluca / Hellmich, Bernhard / Zwerina, Jochen / Resche-Rigon, Matthieu / Emmi, Giacomo / Neumann, Thomas / Mahr, Alfred

    The Journal of rheumatology

    2023  Volume 50, Issue 11, Page(s) 1446–1453

    Abstract: Objective: Previous studies suggested that distinct phenotypes of eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) could be determined by the presence or absence of antineutrophil cytoplasmic antibodies ( ... ...

    Abstract Objective: Previous studies suggested that distinct phenotypes of eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) could be determined by the presence or absence of antineutrophil cytoplasmic antibodies (ANCA), reflecting predominant vasculitic or eosinophilic processes, respectively. This study explored whether ANCA-based clusters or other clusters can be identified in EGPA.
    Methods: This study used standardized data of 15 European centers for patients with EGPA fulfilling widely accepted classification criteria. We used multiple correspondence analysis, hierarchical cluster analysis, and a decision tree model. The main model included 10 clinical variables (musculoskeletal [MSK], mucocutaneous, ophthalmological, ENT, cardiovascular, pulmonary, gastrointestinal, renal, central, or peripheral neurological involvement); a second model also included ANCA results.
    Results: The analyses included 489 patients diagnosed between 1984 and 2015. ANCA were detected in 37.2% of patients, mostly perinuclear ANCA (85.4%) and/or antimyeloperoxidase (87%). Compared with ANCA-negative patients, those with ANCA had more renal (
    Conclusion: Although reinforcing the known association of ANCA status with clinical manifestations, cluster analysis does not support a complete separation of EGPA in ANCA-positive and -negative subsets. Collectively, these data indicate that EGPA should be regarded as a phenotypic spectrum rather than a dichotomous disease.
    MeSH term(s) Humans ; Churg-Strauss Syndrome/diagnosis ; Granulomatosis with Polyangiitis/diagnosis ; Antibodies, Antineutrophil Cytoplasmic ; Phenotype ; Cluster Analysis
    Chemical Substances Antibodies, Antineutrophil Cytoplasmic
    Language English
    Publishing date 2023-09-22
    Publishing country Canada
    Document type Journal Article
    ZDB-ID 194928-7
    ISSN 1499-2752 ; 0315-162X
    ISSN (online) 1499-2752
    ISSN 0315-162X
    DOI 10.3899/jrheum.2023-0325
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  5. Article ; Online: A young man with persistent dyspepsia: the unexpected virtue of proton-pump inhibitors.

    Fraticelli, Paolo / Mattioli, Massimo / Benfaremo, Devis / Biondi, Lorenzo / Cardinali, Marco / Gabrielli, Armando

    Acta bio-medica : Atenei Parmensis

    2019  Volume 89, Issue 4, Page(s) 569–572

    Abstract: A 26-year-old man was referred to our department for a 3-year history of dyspepsia responsive to oral pump-inhibitors therapy. During the last year, he underwent a gastroscopy, a colonoscopy and a computed tomography enterography that failed to reveal an ...

    Abstract A 26-year-old man was referred to our department for a 3-year history of dyspepsia responsive to oral pump-inhibitors therapy. During the last year, he underwent a gastroscopy, a colonoscopy and a computed tomography enterography that failed to reveal an underlying organic disease: a diagnosis of functional dyspepsia was made. Because of the persistence of symptoms, he came to our ambulatory where we performed an abdominal ultrasound that revealed the presence of multiple bi-lobar lesions of the liver suspected for metastases and a hypoechoic solid lesion of the pancreas body, confirmed by a contrast enhanced computed tomography. Laboratory tests showed high chromogranin A and gastrin level, and a liver biopsy was consistent with a metastatic pancreatic neuroendocrine tumor. This report aims to underlie the diffuse heterogeneous diagnostic management of some common gastrointestinal symptoms, such as dyspepsia, that are too often approached with the prescription of proton pump inhibitors.
    MeSH term(s) Adult ; Dyspepsia/drug therapy ; Dyspepsia/etiology ; Humans ; Liver Neoplasms/secondary ; Male ; Neuroendocrine Tumors/secondary ; Pancreatic Neoplasms/pathology ; Proton Pump Inhibitors/therapeutic use
    Chemical Substances Proton Pump Inhibitors
    Language English
    Publishing date 2019-01-15
    Publishing country Italy
    Document type Case Reports ; Journal Article
    ZDB-ID 2114240-3
    ISSN 2531-6745 ; 0392-4203
    ISSN (online) 2531-6745
    ISSN 0392-4203
    DOI 10.23750/abm.v89i4.6371
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  6. Article ; Online: Computed tomography assessment of evolution of interstitial lung disease in systemic sclerosis: Comparison of two scoring systems.

    Salaffi, Fausto / Carotti, Marina / Tardella, Marika / Di Carlo, Marco / Fraticelli, Paolo / Fischetti, Colomba / Giovagnoni, Andrea / Gabrielli, Armando

    European journal of internal medicine

    2020  Volume 76, Page(s) 71–75

    Abstract: Background: The aim of this study was to evaluate and compare the internal and external responsiveness of a computed-aided method (CaM) with a conventional visual reader-based score (CoVR) to measure interstitial lung disease (ILD) in patients with ... ...

    Abstract Background: The aim of this study was to evaluate and compare the internal and external responsiveness of a computed-aided method (CaM) with a conventional visual reader-based score (CoVR) to measure interstitial lung disease (ILD) in patients with systemic sclerosis (SSc) on high resolution computed tomography (HRCT).
    Methods: Forty-five patients were evaluated in this retrospective cohort. HRCTs were collected at baseline and after 1 year. HRCT abnormalities were evaluated according to a CoVR (Warrick's method) and a quantitative CaM. Internal 1-year responsiveness was tested with a standardized mean response (SRM). Analyses of the receiver operating characteristic curves (ROCs) evaluated the sensitivity and specificity of the two methods to discriminate between clinically relevant progression and no relevant progression, using expert judgment as the gold standard (external responsiveness).
    Results: In one year, lung involvement was stable/improved in 17 of the 45 patients (37.8%) and worsened in 28 patients (62.2%). HRCT scores changed moderately over the follow-up period. Using SFM, CaM was significantly more responsive in detecting changes due to treatment than the CoVR method. Likewise, in the analysis of the ROC curve, CaM scores showed the highest performance (AUC ROC CaM vs. CoVR, 0.951 vs. 0.807; p = 0.0065).
    Conclusion: Quantitative analysis of CaM was more responsive than the CoVR method to accurately evaluate and monitor SSc-ILD progression or response to therapy.
    MeSH term(s) Humans ; Lung/diagnostic imaging ; Lung Diseases, Interstitial/diagnostic imaging ; Retrospective Studies ; Scleroderma, Systemic/complications ; Scleroderma, Systemic/diagnostic imaging ; Sensitivity and Specificity ; Tomography, X-Ray Computed
    Language English
    Publishing date 2020-02-20
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 1038679-8
    ISSN 1879-0828 ; 0953-6205
    ISSN (online) 1879-0828
    ISSN 0953-6205
    DOI 10.1016/j.ejim.2020.02.009
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  7. Article ; Online: Relationship between interstitial lung disease and oesophageal dilatation on chest high-resolution computed tomography in patients with systemic sclerosis: a cross-sectional study.

    Salaffi, Fausto / Di Carlo, Marco / Carotti, Marina / Fraticelli, Paolo / Gabrielli, Armando / Giovagnoni, Andrea

    La Radiologia medica

    2018  Volume 123, Issue 9, Page(s) 655–663

    Abstract: Objectives: Oesophageal dilatation (OD) has been implicated in the pathogenesis of interstitial lung disease (ILD) in systemic sclerosis (SSc). The aims of this study were to explore the association of the OD and SSc-ILD on chest high-resolution ... ...

    Abstract Objectives: Oesophageal dilatation (OD) has been implicated in the pathogenesis of interstitial lung disease (ILD) in systemic sclerosis (SSc). The aims of this study were to explore the association of the OD and SSc-ILD on chest high-resolution computed tomography (HRCT), and to establish a cutoff point for the OD suggestive for the presence of a significant lung involvement.
    Methods: The widest oesophageal diameter (WOD) was obtained on axial HRCT images. The parenchymal abnormalities on HRCT were coded and scored according to Warrick method. Patient-centred measures, pulmonary function tests and the single breath carbon monoxide diffusing capacity of the lung (DLco) were also obtained. Multivariate regression analysis was performed to identify factors associated with oesophageal diameter.
    Results: 126 subjects with SSc were included. The mean (± SD) WOD was 13.5 (± 4.2) mm, and in 76 (60.3%) participants WOD was ≥ 11 mm. SSc patients with ILD had larger oesophageal diameters than those without lung disease (19.4 vs. 14.1 mm, p < 0.001). We observed a high correlation between WOD and gastro-oesophageal reflux disease questionnaire (GerdQ) (r = 0.886, p < 0.001), Borg score (r = 0.705, p < 0.001), and Warrick score (r = 0.614, p < 0.001). WOD negatively correlated with DLco (r = - 0.508, p < 0.001). Multivariate analysis demonstrated positive associations between WOD and GerdQ (p < 0.0001), Borg score (p < 0.0005), and total Warrick score (p = 0.019).
    Conclusion: An increased oesophageal diameter (> 11 mm) on chest HRCT is associated with pulmonary and oesophageal symptoms, more severe ILD, and lower DLco.
    MeSH term(s) Cross-Sectional Studies ; Dilatation ; Esophagus/diagnostic imaging ; Female ; Gastroesophageal Reflux/complications ; Gastroesophageal Reflux/diagnostic imaging ; Humans ; Lung Diseases, Interstitial/diagnostic imaging ; Lung Diseases, Interstitial/etiology ; Male ; Middle Aged ; Respiratory Function Tests ; Scleroderma, Systemic/complications ; Surveys and Questionnaires ; Tomography, X-Ray Computed/methods
    Language English
    Publishing date 2018-04-23
    Publishing country Italy
    Document type Journal Article
    ZDB-ID 205751-7
    ISSN 1826-6983 ; 0033-8362
    ISSN (online) 1826-6983
    ISSN 0033-8362
    DOI 10.1007/s11547-018-0894-3
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  8. Article: Combination therapy with rituximab and mycophenolate mofetil in systemic sclerosis. A single-centre case series study.

    Fraticelli, Paolo / Fischetti, Colomba / Salaffi, Fausto / Carotti, Marina / Mattioli, Massimo / Pomponio, Giovanni / Gabrielli, Armando

    Clinical and experimental rheumatology

    2018  Volume 36 Suppl 113, Issue 4, Page(s) 142–145

    Abstract: Objectives: To describe a single centre experience using combination therapy with rituximab (RTX) and mycophenolate mofetil (MMF) in a prospective series of systemic sclerosis (SSc) patients with pulmonary and cutaneous involvement, rapidly progressive ... ...

    Abstract Objectives: To describe a single centre experience using combination therapy with rituximab (RTX) and mycophenolate mofetil (MMF) in a prospective series of systemic sclerosis (SSc) patients with pulmonary and cutaneous involvement, rapidly progressive or resistant to conventional therapy.
    Methods: RTX was administered in two different regimens (1000 mg fortnightly x 2 or 375 mg/m2/week for 4 consecutive weeks) at baseline and after 6 months, associated with MMF 2000 mg/day continuously. Cutaneous fibrosis was evaluated assessing modified Rodnan Skin Score (mRSS) and pulmonary involvement was evaluated performing pulmonary function tests, diffusing lung capacity for carbon monoxide and chest high-resolution computed tomography (HRCT). The radiological extension of the interstitial lung disease (ILD) at HRCT, was assessed with the conventional visual reader-based score (CoVR) and with a computerised-aided method (CaM) using a DICOM soft- ware.
    Results: Eighteen SSc patients underwent combination therapy (F/M: 10/8, median age 51 years, median duration of disease 27 months). Data from fifteen patients were available at 12-month follow-up. The mRSS showed a significant improvement; a significant increase in forced vital capacity and forced expiratory volume in the first second were also observed. In addition, a signi cant reduction of the extension of ILD was detected when evaluated with CaM. No serious adverse events were observed during the follow-up period.
    Conclusions: Despite preliminary results and limited to a small number of patients, our data suggest that therapy with RTX and MMF is well tolerated, safe, and potentially effective for cutaneous and pulmonary involvement in SSc.
    MeSH term(s) Adult ; Aged ; Drug Therapy, Combination ; Female ; Humans ; Immunosuppressive Agents/adverse effects ; Immunosuppressive Agents/therapeutic use ; Italy ; Lung/drug effects ; Lung/immunology ; Lung/physiopathology ; Lung Diseases, Interstitial/diagnosis ; Lung Diseases, Interstitial/drug therapy ; Lung Diseases, Interstitial/immunology ; Lung Diseases, Interstitial/physiopathology ; Male ; Middle Aged ; Mycophenolic Acid/adverse effects ; Mycophenolic Acid/therapeutic use ; Prospective Studies ; Recovery of Function ; Rituximab/adverse effects ; Rituximab/therapeutic use ; Scleroderma, Systemic/diagnosis ; Scleroderma, Systemic/drug therapy ; Scleroderma, Systemic/immunology ; Scleroderma, Systemic/physiopathology ; Skin/drug effects ; Skin/immunology ; Skin/physiopathology ; Skin Diseases/diagnosis ; Skin Diseases/drug therapy ; Skin Diseases/immunology ; Skin Diseases/physiopathology ; Time Factors ; Treatment Outcome ; Young Adult
    Chemical Substances Immunosuppressive Agents ; Rituximab (4F4X42SYQ6) ; Mycophenolic Acid (HU9DX48N0T)
    Language English
    Publishing date 2018-09-30
    Publishing country Italy
    Document type Journal Article
    ZDB-ID 605886-3
    ISSN 1593-098X ; 0392-856X
    ISSN (online) 1593-098X
    ISSN 0392-856X
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  9. Article: Provisional recommendations for SARS-CoV-2 vaccination in patients with cryoglobulinaemic vasculitis.

    Scarpato, Salvatore / Sebastiani, Marco / Quartuccio, Luca / Marson, Piero / Fraticelli, Paolo / Castelnovo, Laura / Visentini, Marcella / Candela, Marco / Mazzaro, Cesare / Saccardo, Francesco / Pioltelli, Piero / Casato, Milvia / Filippini, Davide / Monti, Giuseppe / Galli, Massimo

    Clinical and experimental rheumatology

    2021  Volume 39 Suppl 129, Issue 2, Page(s) 149–154

    Abstract: People with cryoglobulinaemic vasculitis (CV) have an increased risk of infections, attributed to different causes: impairment of the immune system due to the disease itself, comorbidities, and immunosuppressive therapy. Therefore, these patients may be ... ...

    Abstract People with cryoglobulinaemic vasculitis (CV) have an increased risk of infections, attributed to different causes: impairment of the immune system due to the disease itself, comorbidities, and immunosuppressive therapy. Therefore, these patients may be at high risk for a more severe course of COVID-19, including hospitalisation and death. Concerns about efficacy, immunogenicity and safety of vaccines, as well as doubts, not yet fully clarified in patients with systemic autoimmune diseases, represent other important factors for a low vaccination rate in people with (CV). Indeed, providing an expert position on the issues related to SARS-CoV-2 vaccination in patients suffering from CV is of critical relevance in order to help both patients and clinicians who are treating them in making the best choice in each case. A multidisciplinary task force of the Italian Group for the Study of Cryoglobulinaemia (GISC) was convened, and through a Delphi technique produced provisional recommendations regarding SARS-CoV-2 vaccination in cryoglobulinaemic patients.
    MeSH term(s) COVID-19 ; COVID-19 Vaccines ; Cryoglobulinemia ; Humans ; Italy ; SARS-CoV-2 ; Vaccination ; Vasculitis
    Chemical Substances COVID-19 Vaccines
    Language English
    Publishing date 2021-04-30
    Publishing country Italy
    Document type Journal Article
    ZDB-ID 605886-3
    ISSN 1593-098X ; 0392-856X
    ISSN (online) 1593-098X
    ISSN 0392-856X
    DOI 10.55563/clinexprheumatol/2v6v5j
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  10. Article ; Online: Occupational Exposures and Smoking in Eosinophilic Granulomatosis With Polyangiitis: A Case-Control Study.

    Maritati, Federica / Peyronel, Francesco / Fenaroli, Paride / Pegoraro, Francesco / Lastrucci, Vieri / Benigno, Giuseppe D / Palmisano, Alessandra / Rossi, Giovanni M / Urban, Maria L / Alberici, Federico / Fraticelli, Paolo / Emmi, Giacomo / Corradi, Massimo / Vaglio, Augusto

    Arthritis & rheumatology (Hoboken, N.J.)

    2021  Volume 73, Issue 9, Page(s) 1694–1702

    Abstract: Objective: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Environmental agents and occupational exposures may confer susceptibility to EGPA, but data are scarce. This ... ...

    Abstract Objective: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Environmental agents and occupational exposures may confer susceptibility to EGPA, but data are scarce. This study was undertaken to investigate the association between occupational exposures (e.g., silica, farming, asbestos, and organic solvents) and other environmental agents (e.g., smoking) and the risk of EGPA.
    Methods: Patients with newly diagnosed EGPA (n = 111) and general population controls (n = 333) who were matched for age, sex, and geographic area of origin were recruited at a national referral center for EGPA. Exposures were assessed using a dedicated questionnaire administered by a specialist in occupational medicine, under blinded conditions. Odds ratios (ORs) and 95% confidence intervals (95% CIs) were calculated.
    Results: Exposures to silica (OR 2.79 [95% CI 1.55-5.01], P = 0.001), organic solvents (OR 3.19 [95% CI 1.91-5.34], P < 0.001), and farming (OR 2.71 [95% CI 1.71-4.29], P < 0.001) were associated with an increased risk of EGPA. Co-exposure to silica and farming yielded an OR of 9.12 (95% CI 3.06-27.19, P < 0.001), suggesting a multiplicative effect between these 2 exposures. Smoking (current and former smokers combined) was significantly less frequent among patients with EGPA compared to controls (OR 0.49 [95% CI 0.29-0.70], P < 0.001). The pack-year index was also lower among patients with EGPA (OR 0.96 [95% CI 0.94-0.98], P < 0.001). The association of silica and farming was primarily aligned with ANCA-positive EGPA, while the association of smoking status and organic solvents was primarily aligned with ANCA-negative EGPA.
    Conclusion: The environment can influence susceptibility to EGPA. Exposure to silica, farming, or organic solvents is associated with an increased risk of EGPA, while smoking is associated with a lower risk. These exposures seem to have distinct effects on different EGPA subsets.
    Language English
    Publishing date 2021-08-09
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2756371-6
    ISSN 2326-5205 ; 2326-5191
    ISSN (online) 2326-5205
    ISSN 2326-5191
    DOI 10.1002/art.41722
    Database MEDical Literature Analysis and Retrieval System OnLINE

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