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Cryoglobulin

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https://www.readbyqxmd.com/read/28543916/hcv-rna-genomic-sequences-and-hcv-e2-glycoprotein-in-sural-nerve-biopsies-from-hcv-infected-patients-with-peripheral-neuropathy
#1
Sabino Russi, Domenico Sansonno, Salvatore Monaco, Sara Mariotto, Sergio Ferrari, Fabio Pavone, Gianfranco Lauletta, Franco Dammacco
AIMS: Peripheral neuropathy (PN), the major neurological complication of chronic HCV infection, is frequently associated with mixed cryoglobulinemia (MC) and small-vessel systemic vasculitis. While humoral and cell-mediated immune mechanisms are suspected to act together in an aberrant immune response that results in peripheral nerve damage, the role of HCV remains largely speculative. The possible demonstration of HCV in peripheral nerve tissue would obviously assume important pathogenic implications...
May 24, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28507447/managing-refractory-cryoglobulinemic-vasculitis-challenges-and-solutions
#2
REVIEW
Predrag Ostojic, Ivan R Jeremic
Cryoglobulinemia is thought to be a rare condition. It may be an isolated disorder or secondary to a particular disease. According to immunoglobulin composition, cryoglobulinemia is classified into three types. In mixed cryoglobulinemia (types II and III), vascular deposition of cryoglobulin-containing immune complexes and complement may induce a clinical syndrome, characterized by systemic vasculitis and inflammation - cryoglobulinemic vasculitis (CryoVas). Most common clinical manifestations in CryoVas are skin lesions (orthostatic purpura and ulcers), weakness, peripheral neuropathy, Raynaud's phenomenon, sicca syndrome, membranoproliferative glomerulonephritis, and arthralgia and seldom arthritis...
2017: Journal of Inflammation Research
https://www.readbyqxmd.com/read/28507188/cryoglobulinemia-in-sj%C3%A3-gren-syndrome-a-disease-subset-that-links-higher-systemic-disease-activity-autoimmunity-and-local-b-cell-proliferation-in-mucosa-associated-lymphoid-tissue
#3
Luca Quartuccio, Chiara Baldini, Roberta Priori, Elena Bartoloni, Francesco Carubbi, Alessia Alunno, Saviana Gandolfo, Serena Colafrancesco, Roberto Giacomelli, Roberto Gerli, Guido Valesini, Stefano Bombardieri, Salvatore De Vita
OBJECTIVE: To compare systemic disease activity by validated tools, i.e., the European League Against Rheumatism Sjögren Syndrome Disease Activity Index (ESSDAI) and the Clinical ESSDAI (ClinESSDAI) scores, between primary Sjögren syndrome (pSS) with positive serum cryoglobulins and pSS without serum cryoglobulins. METHODS: There were 825 consecutive patients with pSS who were retrospectively evaluated. RESULTS: The ESSDAI and the ClinESSDAI scores were significantly higher in cryoglobulin-positive patients (p < 0...
May 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28503888/cryoglobulinaemic-glomerulonephritis-artefacts-associated-with-laboratory-diagnosis-and-need-for-renal-biopsy
#4
Ajish Radhamma, Jobert Anjelo, Tony J Elias
Cryoglobulinemia as a cause of renal impairment is uncommon but needs to be considered in viral hepatitis and haematological malignancies. Often detection and estimation of cryoglobulins are confounded by collection and processing errors. This report highlights the need for stringent processing measures if the clinical suspicion is high.
May 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/28466806/hcv-unrelated-cryoglobulinaemic-vasculitis-the-results-of-a-prospective-observational-study-by-the-italian-group-for-the-study-of-cryoglobulinaemias-gisc
#5
Massimo Galli, Letizia Oreni, Francesco Saccardo, Laura Castelnovo, Davide Filippini, Piero Marson, Maria Teresa Mascia, Cesare Mazzaro, Laura Origgi, Elena Ossi, Maurizio Pietrogrande, Piero Pioltelli, Luca Quartuccio, Salvatore Scarpato, Salvatore Sollima, Agostino Riva, Paolo Fraticelli, Roberta Zani, Dilia Giuggioli, Marco Sebastiani, Piercarlo Sarzi Puttini, Armando Gabrielli, Anna Linda Zignego, Patrizia Scaini, Clodoveo Ferri, Salvatore De Vita, Giuseppe Monti
OBJECTIVES: To investigate the clinical and laboratory patterns of HCV-unrelated cryoglobulinaemic vasculitis (CV), and the factors influencing its outcome. METHODS: Prospective study of all anti-HCV and HCV-RNA negative patients with CV who have been observed since January 2004 in 17 centres participating in the Italian Group for the Study of Cryoglobulinaemias (GISC). RESULTS: 175 enrolled were followed up for 677 person-years. The associated conditions were primary Sjögren's syndrome (21...
March 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28424927/development-of-new-extra-glandular-manifestations-or-associated-auto-immune-diseases-after-establishing-the-diagnosis-of-primary-sj%C3%A3-gren-s-syndrome-a-long-term-study-of-the-antonius-nieuwegein-sj%C3%A3-gren-ans-cohort
#6
E J Ter Borg, J C Kelder
To investigate in a long-term study, the development of new extra-glandular manifestations (EGM) or associated auto-immune diseases (AID) from 1 year after establishing the diagnosis of primary Sjögren's syndrome (pSS). The primary goal was to examine the frequency and type of these manifestations and to find out which demographic, clinical and serological profile was most at risk. All outpatients diagnosed with primary Sjögren's syndrome were included in a retrospective study, with at least one check-up per year, from June 1991 until August 2015...
July 2017: Rheumatology International
https://www.readbyqxmd.com/read/28422865/crystalglobulinemia-manifesting-as-chronic-arthralgia-and-acute-limb-ischemia-a-clinical-case-report
#7
Nobuya Abe, Tomoko Tomita, Miyuki Bohgaki, Hideki Kasahara, Takao Koike
RATIONALE: Crystalglobulinemia is a rare disease caused by monoclonal immunoglobulins, characterized by irreversible crystallization on refrigeration. It causes systemic symptoms including purpura, arthralgia, and vessel occlusive conditions to be exacerbated by exposure to cold. We report a patient with crystalglobulinemia associated with monoclonal gammopathy of undetermined significance (MGUS) manifesting as chronic arthralgia and recurrent acute arterial occlusion. PRESENTING CONCERNS: A 61-year-old man, who had been diagnosed with MGUS and who had arthralgia of unknown origin, presented with recurrent acute limb ischemia after surgical thromboembolectomy...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28416003/characterization-and-risk-estimate-of-cancer-in-patients-with-primary-sj%C3%A3-gren-syndrome
#8
Pilar Brito-Zerón, Belchin Kostov, Guadalupe Fraile, Daniel Caravia-Durán, Brenda Maure, Francisco-Javier Rascón, Mónica Zamora, Arnau Casanovas, Miguel Lopez-Dupla, Mar Ripoll, Blanca Pinilla, Eva Fonseca, Miriam Akasbi, Gloria de la Red, Miguel-Angel Duarte-Millán, Patricia Fanlo, Pablo Guisado-Vasco, Roberto Pérez-Alvarez, Antonio J Chamorro, César Morcillo, Iratxe Jiménez-Heredia, Isabel Sánchez-Berná, Armando López-Guillermo, Manuel Ramos-Casals
BACKGROUND: The purpose of this study is to characterize the risk of cancer in a large cohort of patients with primary Sjögren syndrome (SjS). METHODS: We had analyzed the development of cancer in 1300 consecutive patients fulfilling the 2002 SjS classification criteria. The baseline clinical and immunological characteristics and systemic activity (ESSDAI scores) were assessed at diagnosis as predictors of cancer using Cox proportional hazards regression analysis adjusted for age at diagnosis and gender...
April 17, 2017: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/28415864/-extrahepatic-manifestations-in-chronic-hepatitis-c-infected-patients
#9
Krisztina Hagymási, Anna Egresi, Gabriella Lengyel
The importance of chronic hepatitis C infection is significant. 3% of the World's population is infected. There is at least one extrahepatic manifestation in 50% of HCV patients, which makes the prognosis and mortality worse. The pathomechanisms included are cryoglobulin production, immunmechanisms, and direct viral effects. The authors summarize the main extrahepatic manifestations, as well as treatment possibilities. The aim is to draw attention to this colourful infection in order to improve the recognition in the era of the new effective direct antiviral agents...
April 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28388935/direct-acting-antiviral-agents-in-the-therapy-of-hepatitis-c-virus-related-mixed-cryoglobulinaemia-a-single-centre-experience
#10
Gianfranco Lauletta, Sabino Russi, Fabio Pavone, Angelo Vacca, Franco Dammacco
BACKGROUND: The efficacy and safety of direct-acting antiviral agents (DAAs) were evaluated in a cohort of prospectively enrolled patients with hepatitis C virus (HCV)-related mixed cryoglobulinaemia (MC), an immune complex-mediated vasculitis of small and medium vessels in which the pathogenetic role of HCV has been clearly established. METHODS: Twenty-two patients received DAAs. Clinical and laboratory features were recorded at baseline, every 4 weeks until the end of treatment (EoT), and 12 weeks afterwards...
April 8, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28370486/clinical-presentation-and-outcomes-of-patients-with-type-1-monoclonal-cryoglobulinemia
#11
Surbhi Sidana, S Vincent Rajkumar, Angela Dispenzieri, Martha Q Lacy, Morie A Gertz, Francis K Buadi, Suzanne R Hayman, David Dingli, Prashant Kapoor, Wilson I Gonsalves, Ronald S Go, Yi Lisa Hwa, Nelson Leung, Amie L Fonder, Miriam A Hobbs, Steven R Zeldenrust, Stephen J Russell, John A Lust, Robert A Kyle, Shaji K Kumar
We describe a series of 102 patients diagnosed from January 1, 1990 to December 31, 2015 with Type 1 monoclonal cryoglobulinemia (MoC). Symptoms were seen in 89 (87%) patients, including: cutaneous symptoms in 64 (63%) patients, with purpura (n = 43, 42%) and ulcers/gangrene (n = 35, 34%) being most common; neurological findings in 33 (32%) patients, most frequently sensory neuropathy (n = 24, 24%); vasomotor symptoms, mainly Raynaud's phenomenon in 25 (25%); arthralgias in 24 (24%); and renal manifestations, primarily glomerulonephritis in 14 (14%) patients...
July 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28368978/cryoglobulinemia-vasculitis-how-to-handle
#12
Anne C Desbois, Cloe Comarmond, David Saadoun, Patrice Cacoub
PURPOSE OF REVIEW: More than 50% of hepatitis C virus (HCV) infected patients produce a mixed cryoglobulin and two-third of them will develop a symptomatic cryoglobulinemia vasculitis (CryoVas). In the present review, we aim at summarizing the most recent advances in diagnosis and treatment of HCV-CryoVas. RECENT FINDINGS: The treatment of HCV-CryoVas has much changed during the last months. The recent emergence of new direct-acting (DAA) interferon (IFN)-free antivirals, enabling high cure rates with a very good safety profile now permit to cure most patients with HCV-CryoVas...
July 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28348266/it-s-not-what-it-looks-like-atypical-rash-in-cryoglobulinaemic-vasculitis
#13
Tyler Gleason, Sushil Ghimire, Susmita Paladugu
A 48-year-old man with a history of intravenous drug use and chronic, untreated hepatitis C presented to the emergency room with acute bilateral lower extremity swelling, erythema and maculopapular rash. Serum C4 levels were low, but dermatology felt the rash was due to venous stasis dermatitis. The patient was discharged with compression stockings, but returned to the hospital 5 days later with no improvement in his symptoms. A more extensive laboratory workup revealed hepatitis C viral load of 4 million, elevated serum cryoglobulins, and skin biopsy showing leucocytoclastic vasculitis...
March 27, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28339354/the-effects-of-an-educational-meeting-and-subsequent-computer-reminders-on-the-ordering-of-laboratory-tests-by-rheumatologists-an-interrupted-time-series-analysis
#14
Nienke Lesuis, Nathan den Broeder, Nadine Boers, Ester Piek, Steven Teerenstra, Marlies Hulscher, Ronald van Vollenhoven, Alfons A den Broeder
OBJECTIVES: To examine the effects of an educational meeting and subsequent computer reminders on the number of ordered laboratory tests. METHODS: Using interrupted time series analysis we assessed whether trends in the number of laboratory tests ordered by rheumatologists between September 2012 and September 2015 at the Sint Maartenskliniek (the Netherlands) changed following an educational meeting (September 2013) and the introduction of computer reminders into the Computerised Physician Order Entry System (July 2014)...
May 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28332476/new-onset-hepatitis-c-virus-associated-glomerulonephritis-following-sustained-virologic-response-with-direct-acting-antiviral-therapy%C3%A2
#15
Muriel Ghosn, Matthew B Palmer, Catherine E Najem, Danny Haddad, Peter A Merkel, Jonathan J Hogan
Glomerulonephritis (GN) is an important extra-hepatic manifestation of infection with hepatitis C virus (HCV). HCV-associated GN occurs due to HCV-induced lymphoproliferation, leading to the generation of pathogenic immune complexes, including complexes containing cryoglobulins. The management of HCV-associated extra-hepatic disease is focused on viral eradication, with direct-acting antiviral agents leading to high rates of sustained virologic remission. There have been a few reports of relapsing cryoglobulinemic vasculitis after sustained virologic remission was achieved with interferon-based therapies...
May 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28291241/efficacy-and-safety-of-direct-acting-antivirals-for-the-treatment-of-mixed-cryoglobulinemia
#16
Joel S Emery, Magdalena Kuczynski, Danie La, Saeed Almarzooqi, Matthew Kowgier, Hemant Shah, David Wong, Harry L A Janssen, Jordan J Feld
OBJECTIVES: Mixed cryoglobulinemia is strongly associated with hepatitis C virus (HCV) infection and ranges from being asymptomatic to causing life-threatening vasculitis. In those with symptoms, treatment with pegylated interferon (pegIFN) and ribavirin (RBV) reduces mortality. However, few data are available on the safety and efficacy of antiviral therapy with direct acting antivirals (DAAs) in the treatment of HCV-related cryoglobulinemia. METHODS: Patients treated for HCV-related cryoglobulinemia with DAA±pegIFN were retrospectively evaluated at a tertiary care center...
March 14, 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/28288791/efficacy-and-safety-of-sofosbuvir-plus-daclatasvir-for-treatment-of-hcv-associated-cryoglobulinemia-vasculitis
#17
David Saadoun, Stanislas Pol, Yasmina Ferfar, Laurent Alric, Christophe Hezode, Si Nafa Si Ahmed, Luc de Saint Martin, Cloé Comarmond, Anne Sophie Bouyer, Lucile Musset, Thierry Poynard, Matthieu Resche Rigon, Patrice Cacoub
Circulating mixed cryoglobulins are detected in 40%-60% of patients with hepatitis C virus (HCV) infection, and overt cryoglobulinemia vasculitis (CryoVas) develops in approximately 15% of patients. Remission of vasculitis has been associated with viral clearance, but few studies have reported the effectiveness of direct-acting antiviral drugs in these patients. We performed an open-label, prospective, multicenter study of the effectiveness and tolerance of an all-oral, interferon- and ribavirin-free regimen of sofosbuvir plus daclatasvir in patients with HCV-associated CryoVas...
March 10, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28274850/direct-acting-antiviral-therapy-restores-immune-tolerance-to-patients-with-hepatitis-c-virus-induced-cryoglobulinemia-vasculitis
#18
Cloé Comarmond, Marlène Garrido, Stanislas Pol, Anne-Claire Desbois, Myrto Costopoulos, Magali Le Garff-Tavernier, Si Nafa Si Ahmed, Laurent Alric, Hélène Fontaine, Bertrand Bellier, Anna Maciejewski, Michelle Rosenzwajg, David Klatzmann, Lucile Musset, Thierry Poynard, Patrice Cacoub, David Saadoun
BACKGROUND & AIMS: Interferon-free direct-acting antiviral (DAA) therapies are effective in patients with hepatitis C virus-induced cryoglobulinemia vasculitis (HCV-CV). We analyzed blood samples from patients with HCV-CV before and after DAA therapy to determine mechanisms of these drugs and their effects on cellular immunity. METHODS: We performed a prospective study of 27 consecutive patients with HCV-CV (median age, 59 y) treated with DAA therapy (21 patients received sofosbuvir plus ribavirin for 24 weeks, 4 patients received sofosbuvir plus daclatasvir for 12 weeks, and 2 patients received sofosbuvir plus simeprevir for 12 weeks) in Paris, France...
June 2017: Gastroenterology
https://www.readbyqxmd.com/read/28149647/hepatitis-c-and-kidney-disease-a-narrative-review
#19
REVIEW
Rashad S Barsoum, Emad A William, Soha S Khalil
Hepatitis-C (HCV) infection can induce kidney injury, mostly due to formation of immune-complexes and cryoglobulins, and possibly to a direct cytopathic effect. It may cause acute kidney injury (AKI) as a part of systemic vasculitis, and augments the risk of AKI due to other etiologies. It is responsible for mesangiocapillary or membranous glomerulonephritis, and accelerates the progression of chronic kidney disease due to other causes. HCV infection increases cardiovascular and liver-related mortality in patients on regular dialysis...
March 2017: Journal of Advanced Research
https://www.readbyqxmd.com/read/28149646/vasculitic-syndromes-in-hepatitis-c-virus-a-review
#20
REVIEW
Gaafar Ragab, Mohamed A Hussein
Vasculitis is a remarkable presentation of the extrahepatic manifestations of HCV. According to the presence or absence of cryoglobulins it is subdivided into two main types: cryoglobulinemic vasculitis and non cryoglobulinemic vasculitis based on the attribution of vasculitis to serum cryoglobulins as a pathogenic factor. The attribution of cryoglobulinemia to HCV represents a success story in the history of immunology, microbiology, and clinical medicine. HCV can bind to and invade lymphocytes, consequently triggering an immune response through different mechanisms...
March 2017: Journal of Advanced Research
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