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https://www.readbyqxmd.com/read/28690517/erythema-gyratum-repens-like-purpura-in-a-patient-with-sj%C3%A3-gren-syndrome
#1
Miyuki Fukunaga, Kazutoshi Harada, Kenichiro Mae, Kanae Wakamatsu, Noriko Kiriyama, Ryoji Tsuboi, Yukari Okubo
The etiology of purpura in Sjögren syndrome (SS) includes cryoglobulinemia, hypergammaglobulinemia, and leukocytoclastic vasculitis (LCV). The clinical symptoms of LCV associated with SS comprise palpable or nonpalpable purpura and urticarial vasculitis. Here, we report a case of LCV presenting as erythema gyratum repens (EGR)-like purpura in a 62-year-old woman with rheumatoid arthritis and SS. EGR-like skin lesions, characterized by annular lesions with expanding concentric pattern and coalescing to form a zebra-like pattern or grain of wood pattern, can appear in various autoimmune conditions; however, EGR-like eruption in SS is extremely rare...
May 2017: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28689593/dermatologic-manifestations-of-chronic-hepatitis-c-infection
#2
REVIEW
Mehmet Sayiner, Pegah Golabi, Freba Farhat, Zobair M Younossi
Chronic hepatitis C virus (HCV) infection is associated with various extrahepatic manifestations, including dermatologic involvement mostly caused by immune complexes. Mixed cryoglobulinemia has a strong relationship with HCV with 95% of these patients being infected with HCV. Lichen planus is a disease of the squamous epithelium and may affect any part of the skin, with 4% to 24% of patients with lichen planus reported to have chronic HCV infection. Porphyria cutanea tarda is the most common form of porphyria, and it is thought that HCV interferes with iron stores, which can promote porphyria cutanea tarda...
August 2017: Clinics in Liver Disease
https://www.readbyqxmd.com/read/28689585/rheumatologic-manifestations-of-hepatitis-c-virus-infection
#3
REVIEW
Patrice Cacoub, Cloé Comarmond, Anne Claire Desbois, David Saadoun
Hepatitis C virus (HCV) infection is associated with a morbidity and mortality due to liver complications. HCV infection is also frequently associated with rheumatic disorders, such as arthralgia, myalgia, cryoglobulinemia vasculitis, and sicca syndrome, as well as the production of autoantibodies. The treatment of HCV infection with interferon alpha (IFN) has been contraindicated for a long time in many rheumatologic autoimmune/inflammatory disorders. New oral IFN-free combinations offer an opportunity for HCV-infected patients with extrahepatic manifestations, including rheumatologic autoimmune/inflammatory disorders, to be cured with a short treatment duration and a low risk of side effects...
August 2017: Clinics in Liver Disease
https://www.readbyqxmd.com/read/28688880/igg3-subclass-a-possible-trigger-of-mixed-cryoglobulin-cascade-in-hepatitis-c-virus-chronic-infection
#4
Umberto Basile, Francesca Gulli, Laura Gragnani, Elisa Fognani, Cecilia Napodano, Krizia Pocino, Anna Linda Zignego, Gian Ludovico Rapaccini
HCV is a hepatotropic and lymphotropic virus and is the most frequent cause of "benign" mono-oligoclonal B-lymphocyte proliferation, observed in mixed cryoglobulinemia (MC). The study aims to investigate the presence, prevalence and characteristics of the subclasses of cryoglobulins in HCV-patients to look for a relationship with MC. Fifty HCV-infected patients with cryoglobulins were enrolled. IgG subclasses were characterized in cryoprecipitate, and serum IgG and IgM Rheumatoid Factor (RF) were determined...
June 24, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28682959/paraneoplastic-neuropathies
#5
Jean-Christophe Antoine, Jean-Philippe Camdessanché
PURPOSE OF REVIEW: To review recent advances in paraneoplastic neuropathies with emphasis on their definition, different forms and therapeutic development. RECENT FINDINGS: A strict definition of definite paraneoplastic neuropathies is necessary to avoid confusion. With carcinoma, seronegative sensory neuronopathies and neuronopathies and anti-Hu and anti-CV2/Contactin Response Mediator Protein 5 antibodies are the most frequent. With lymphomas, most neuropathies occur with monoclonal gammopathy including AL amyloidosis, Polyneuropathy-Organomegaly-Endocrinopathy-M component-Skin changes (POEMS) syndrome, type I cryoglobulinemia and antimyelin-associated glycoprotein (MAG) neuropathies and Waldenström's disease...
July 5, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28675071/from-the-pathogenesis-to-the-cure-of-indolent-b-cell-lymphoproliferative-disorders-associated-with-hepatitis-c-virus-infection-which-role-for-direct-acting-antivirals
#6
Marcella Visentini, Massimo Fiorilli, Milvia Casato
Hepatitis C virus (HCV) causes monoclonal B cell lymphoproliferative disorders ranging from benign, such as in mixed cryoglobulinemia (MC), to indolent or aggressive lymphomas. MC and indolent lymphomas commonly regress when HCV is eradicated with interferon (IFN) therapy; however, sustained virologic response (SVR) to IFN is achieved only in ~50% of patients. The new all oral direct-acting antivirals (DAA), yielding nearly 100% SVR, promise a breakthrough in the treatment of HCV-associated lymphoproliferative disorders, but experience is still scanty...
July 4, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28661098/efficacy-of-cryofiltration-for-treatment-of-mixed-cryoglobulinemia-a-report-of-four-cases
#7
Yoshihiro Taniyama, Yoshihisa Nakatani, Toshiaki Matsuoka, Miyo Takahashi, Kazuyuki Shimizu, Sachiyo Yamamoto, Yuki Inoue, Sayoko Ohnishi, Norihiro Kobayashi, Yukihito Nakano, Masahiro Takami, Shuji Arima
Cryoglobulinemia can induce systemic vasculitis affecting various organs such as skin, peripheral nerves, and kidney. The disease can induce chronic organ failure and even be life-threatening. Cryofiltration has been applied for the treatment of cryoglobulinemic vasculitis. We have experienced four cases with mixed cryoglobulinemia showing severe and progressive clinical manifestations, including skin purpura, nephrotic syndrome, acute kidney injury, and peripheral neuropathy. Cryofiltration in conjunction with conventional pharmacological therapies appeared to be safe and effective...
June 2017: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/28643451/polyclonal-and-monoclonal-b-lymphocytes-response-in-hcv-patients-treated-with-direct-acting-antiviral-agents
#8
Alvise Schiavinato, Alberto Zanetto, Giorgia Pantano, Francesca Tosato, Mitja Nabergoj, Paola Fogar, Elisa Piva, Martina Gambato, Enrica Franceschet, Annarosa Floreani, Fabio Farinati, Patrizia Burra, Francesco Paolo Russo, Mario Plebani
Hepatitis C Virus (HCV) chronic infection can be associated with extrahepatic manifestations such as mixed cryoglobulinemia and lymphoproliferative disorders that are endowed with increased rates of morbidity and all-cause mortality. In this study we used flow cytometry to evaluate the effect of Interferon-free antiviral treatment on peripheral blood lymphocytes in HCV patients with or without associated lymphoproliferative disorders. Flow cytometry analysis of peripheral blood lymphocytes were performed at baseline and at the end of treatment...
June 22, 2017: Journal of Viral Hepatitis
https://www.readbyqxmd.com/read/28579587/direct-acting-antiviral-agents-improved-nephrotic-syndrome-associated-with-refractory-pleural-effusion-and-ascites-caused-by-hepatitis-c-virus-related-nephropathy-a-case-report
#9
Masatoshi Yamato, Kuniaki Arai, Hajime Takatori, Tetsuro Shimakami, Taro Yamashita, Yoshio Sakai, Tatsuya Yamashita, Eishiro Mizukoshi, Masao Honda, Shuichi Kaneko
A 74-year-old man with hepatitis C virus (HCV)-related cirrhosis was admitted because of dyspnea. Laboratory investigations revealed severe proteinuria (4.0g/day), low serum albumin level, and cryoglobulinemia. Computed tomography showed massive pleural effusion and ascites. Because these effusions were leaky and the hepatic reserve was relatively intact, we thought these were mainly caused by nephrotic syndrome. Renal biopsy revealed membranoproliferative glomerulonephritis with mesangial proliferation and excessive matrix deposition...
2017: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
https://www.readbyqxmd.com/read/28550239/clinical-conditions-responsible-for-hyperviscosity-and-skin-ulcers-complications
#10
Gregorio Caimi, Baldassare Canino, Rosalia Lo Presti, Caterina Urso, Eugenia Hopps
In this brief review, we have examined some clinical conditions that result to be associated to an altered hemorheological profile and at times accompanied by skin ulcers. This skin condition may be observed in patients with the following condtions, such as primary polycythemic hyperviscosity (polycythemia, thrombocytemia) treated with hydroxyurea, primary plasma hyperviscosity (multiple myeloma, cryoglobulinemia, cryofibrinogenemia, dysfibrinogenemia, and connective tissue diseases), primary sclerocythemic hyperviscosity (hereditary spherocytosis, thalassemia, and sickle cell disease)...
May 19, 2017: Clinical Hemorheology and Microcirculation
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
#11
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/28538208/notch4-and-mhc%C3%A2-class-ii-polymorphisms-are-associated-with%C3%A2-hcv-related-benign-and-malignant-lymphoproliferative-diseases
#12
Laura Gragnani, Elisa Fognani, Valli De Re, Massimo Libra, Adriana Garozzo, Patrizio Caini, Guia Cerretelli, Andrea Giovannelli, Serena Lorini, Monica Monti, Silvia Bagnoli, Irene Piaceri, Anna Linda Zignego
Mixed cryoglobulinemia (MC), is a HCV-related, clinically benign, lymphoproliferative disorder (LPD) that may evolve into a non Hodgkin's lymphoma (NHL). Significant associations were found between two single nucleotide polymorphisms near NOTCH4 (rs2071286) and the HLA class II (rs9461776) genes and HCV-related MC syndrome (MCS). We analyzed NOTCH4 rs2071286 and HLA-II rs9461776 in 3 HCV-related LPD groups (asymptomatic MC, MCS, NHL) with HCV infection without lymphoproliferative disorders.We found a positive relationship between NOTCH4 rs207186 T minor allele frequency (MAF) and patients with HCV-related LPDs at risk of NHL (Chi-square test for trend = 14...
March 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/28536920/clinicopathological-features-of-multiple-mononeuropathy-associated-with-systemic-lupus%C3%A2-erythematosus-a-multicenter-study
#13
Elodie Rivière, Fleur Cohen Aubart, Thierry Maisonobe, François Maurier, Christophe Richez, Bruno Gombert, Marie Gousseff, Daniel Adoue, Alexis Mathian, Miguel Hié, Julien Haroche, Zahir Amoura
Multiple mononeuropathy (MM) occurs rarely during systemic lupus erythematosus (SLE) but may lead to major disability. The aim of this study was to investigate the clinic-pathological presentations of MM during SLE, as well as long-term outcomes. We conducted a multicentric retrospective study that included patients receiving a diagnosis of MM during SLE. Ten patients were included (8 women and 2 men, median age at MM diagnosis: 40.4 years). SLE was diagnosed before MM in 9/10 patients (median time 8.2 years)...
June 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28533861/-cutaneous-leukocytoclastic-vasculitis-about-85-cases
#14
Amina Aounallah, Aicha Arouss, Najet Ghariani, Wafa Saidi, Badreddine Sriha, Mohamed Denguezli, Colandane Belajouza, Rafia Nouira
Clinical manifestation, etiology and outcome of leukocytoclastic vasculitis are little studied. The aim of our study was to examine epidemiological, clinical etiological, and evolutionary characteristics of this entity. We conducted a cross-sectional data collection from medical records of 85 patients with leukocytoclastic vasculitis in the Department of Dermatology at the Farhat Hached University Hospital, Sousse between January 2000 and December 2013. Epidemiological, clinical, paraclinical, etiological data sheets had been completed for each patient...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28520564/normothermic-cardiopulmonary-bypass-in-patient-with-waldenstr%C3%A3-m-s-macroglobulinemia-and-cryoglobulinemia-a-case-report
#15
Shiho Satomi, Asuka Kasai, Eisuke Hamaguchi, Yasuo M Tsutsumi, Katsuya Tanaka
Waldenström's macroglobulinemia (WM) manifests as hyperviscosity syndrome. Cryoglobulinemia, which may increase blood viscosity or induce thrombosis in association with decreased body temperature, can occur in combination with WM. We describe the management of an 82-year-old woman with WM, hyperviscosity syndrome, and cryoglobulinemia who required open aortic valve replacement. Decreased body temperature in this patient was prevented during cardiopulmonary bypass by using a forced air warming system and normothermic cardioplegia with continuous warm blood cardioplegia perfusion...
May 17, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28514410/-cryoglobulinemic-vasculitis-in-chronic-hepatitis-c-genetic-aspects
#16
M G Artemova, D T Abdurakhmanov
Cryoglobulinemia (CG) is detected in more than 50% of patients with chronic hepatitis C (CHC); however, only 15-25% of them develop cryoglobulinemic vasculitis (CV) that is a systemic vasculitis due to the formation of immune deposits, which affects small (less than medium-sized) vessels and which is frequently fatal for the patient. The causes of CG only in some patients with CHC and the pathogenesis of CV remain unstudied; however, the accumulated data allow one to identify the special contribution of the patient's genetic factors to the development of the disease...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28509128/a-case-of-rapid-amelioration-of-hepatitis-c-virus-associated-cryoglobulinemic-membranoproliferative-glomerulonephritis-treated-by-interferon-free-directly-acting-antivirals-for-hcv-in-the-absence-of-immunosuppressant
#17
Fumiaki Obata, Taichi Murakami, Junko Miyagi, Sayo Ueda, Taizo Inagaki, Masanori Minato, Hiroyuki Ono, Kenji Nishimura, Eriko Shibata, Masanori Tamaki, Sakiya Yoshimoto, Fumi Kishi, Seiji Kishi, Motokazu Matsuura, Kojiro Nagai, Hideharu Abe, Toshio Doi
Mixed cryoglobulinemic syndrome, which is a systemic vasculitis characterized by the immune complex deposition in small- and medium-sized arteries and most often due to chronic hepatitis C virus (HCV) infection, sometimes clinically manifests as refractory glomerulonephritis or nephritic syndrome. Patients with mixed cryoglobulinemic nephropathy who have a rapidly progressive glomerulonephritis should receive immunosuppressive therapy. After disease stabilization, patients should receive concurrent therapy for the underlying HCV infection...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28507447/managing-refractory-cryoglobulinemic-vasculitis-challenges-and-solutions
#18
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
#19
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/28507081/reversion-of-anergy-signatures-in-clonal-cd21low-b-cells-of-mixed-cryoglobulinemia-after-clearance-of-hcv-viremia
#20
Martina Del Padre, Laura Todi, Milica Mitrevski, Ramona Marrapodi, Stefania Colantuono, Massimo Fiorilli, Milvia Casato, Marcella Visentini
Hepatitis C virus (HCV) causes mixed cryoglobulinemia (MC) by driving clonal expansion of IgM(+)CD27(+) B cells. These cells display both the features of anergy induced by continual engagement of the B cell receptor (BCR), such as high expression of phosphorylated extracellular signal regulated kinase (pERK) and reduced lifespan, and of virus-specific exhaustion such as CD21(low) phenotype and defective response to ligation of BCR and Toll-like receptor 9 (TLR9). Usually MC regresses after eradication of HCV with interferon, whose immunomodulatory activity might contribute to this effect...
May 15, 2017: Blood
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