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Cryoglobulinemic vasculitis

Jana Kazandjieva, Dimitar Antonov, Jivko Kamarashev, Nikolai Tsankov
Purpuric lesions appear in acral distribution in a variety of conditions and often provide clues to the clinical diagnosis. Purpuric means "hemorrhagic"-that is, the lesions do not blanch from pressure. This review focuses on dermatoses that produce hemorrhagic lesions in acral distribution from the large groups of the vasculitic diseases and their mimics. Cutaneous small vessel vasculitis is confined to the skin, involves mainly postcapillary venules, and has the hallmark manifestation of palpable purpura...
January 2017: Clinics in Dermatology
Paul Oykhman, Marlene A Hamilton, Stephen L Aaron
No abstract text is available yet for this article.
December 2016: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Shivali R Berera, Alexandra Gomez, Kevin Dholaria, Leopoldo R Arosemena, Marco A Ladino-Avellaneda, Laura Barisoni, Kalyan R Bhamidimarri
Intestinal involvement of cryoglobulinemia is an uncommon manifestation and marker of severe vasculitis. We describe the case of a woman admitted to our service for management of acute renal failure and progressive gastrointestinal symptoms after initiating hepatitis C virus treatment with ribavirin and sofosbuvir 4 weeks prior. With an undetectable hepatitis C viral load and persistent symptoms despite hepatitis C virus therapy cessation, an upper endoscopy revealed duodenal sloughing, erythema, and bleeding, sparking suspicion for recurrence of cryoglobulinemic vasculitis...
August 2016: ACG Case Reports Journal
C Giménez-Roca, E Iglesias, M A Vicente, R Bou, J Calzada-Hernández, C Prat, M García, J Antón
No abstract text is available yet for this article.
October 28, 2016: Dermatologic Therapy
Martín Bonacci, Sabela Lens, María-Carlota Londoño, Zoe Mariño, Maria C Cid, Manuel Ramos-Casals, Jose María Sánchez-Tapias, Xavier Forns, José Hernández-Rodríguez
BACKGROUND & AIMS: Cryoglobulins (circulating immune complexes of polyclonal IgG, monoclonal IgM, and rheumatoid factor) are detected in the circulation of 40% to 60% of patients with chronic hepatitis C virus infection, and cryoglobulinemic vasculitis (CV) is observed in approximately 10% of patients. We aimed to assess the clinical and immune effects of direct-acting antiviral treatment. METHODS: We performed a prospective study of 64 patients with HCV infection with circulating cryoglobulins receiving direct-acting antiviral therapy at a single center in Barcelona, Spain, from January 2014 through April 2016...
October 8, 2016: Clinical Gastroenterology and Hepatology
B Emmanuel, N Sidique, X Zhang, B Poonia, M C Sneller, S Kottilil
Mixed cryoglobulinemic vasculitis is associated with the monoclonal expansion of pathognomonic B cells in chronic hepatitis C. Recently, treatment with B-cell depletion, including rituximab, a CD20 monoclonal antibody, has been successful in achieving remission from the active disease. We investigated whether B-cell depletion therapy has an impact on activation of non-B cells in the periphery. Results demonstrated that B-cell depletion therapy is associated with a statistically significant decline in activated T cells, from pretherapy to follow-up while on rituximab therapy: CD4+ CD38+ HLA-DR+ (DR+), CD8+ CD38, CD8+ CD38+ DR+, and CD8+ DR+...
September 25, 2016: Journal of Viral Hepatitis
Konstantinos Thomas, Dimitrios Vassilopoulos
PURPOSE OF REVIEW: To review recent evidence for infection rates in patients with systemic vasculitides, the role of specific infectious agents in the pathogenesis of vasculitis and recent breakthroughs in the treatment of virus-associated vasculitides. RECENT FINDINGS: In well designed recent studies, infections were found to be common during the first 6-12 months in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) and giant cell arteritis (GCA) and to contribute significantly to increased mortality during this period...
January 2017: Current Opinion in Rheumatology
Bertrand Lioger, Nicole Ferreira-Maldent, Jean Philippe Cottier, Séverine Debiais, Emmanuel Gyan, François Maillot
No abstract text is available yet for this article.
August 2016: Neurology® Neuroimmunology & Neuroinflammation
Shiber Shachaf, Molad Yair
BACKGROUND: Cryoglobulinemia is an immune-complex-mediated small vessel vasculitis that classically involves the skin, kidneys and peripheral nerves. Antiphospholipid syndrome (APS) is an autoimmune hypercoagulable disorder which causes blood vessel thrombosis. It can present as a multi-organ microthrombotic disorder which is called catastrophic APS. OBJECTIVE: In this case series we aim to describe the diagnostic and management challenges that arise when these two severe disorders simultaneously present in the same patient...
January 2016: Revista Brasileira de Reumatologia
Hussein El-Fishawy, Gamal Saadi, May Hassaballa, Mohamed Hussein, Wahid Doss, Gaafar Ragab, Rashad Barsoum
Egypt, the single country with highest incidence of HCV infection in the world, has embarked on a government-sponsored mass treatment program using several combinations of DAAs. Recognizing the importance of extrahepatic manifestations, independently of the hepatic, a subcommittee was assigned to develop national guidelines for respective prioritizing indications and protocols. It evaluated the benefit of treating patients with different extrahepatic manifestations, and reviewed relevant clinical trials and guidelines concerning DAA combinations available in Egypt...
May 2016: Journal of Advanced Research
Siba Prasad Dalai, Lalit Kumar Meher, Samir Kumar Behera, Sachidananda Nayak, Sujit Kumar Tripathy
Cryoglobulins are monoclonal or polyclonal immunoglobulins that undergo reversible precipitation at low temperatures. Cryoglobulinemia is associated with HCV infection in more than 90% cases, the remaining 10% being called as Essential Cryoglobulinemia which is generally associated with a severe course and suboptimal response to conventional therapies. As the digital vessels are more prone to colder temperatures, hyperviscosity in those vessels can initiate local thrombosis and may manifest as ischemic ulceration and gangrene...
April 2016: Journal of Clinical and Diagnostic Research: JCDR
Mari Kamiya, Peter Y Shane, Makoto Soejima, Shuji Tohda, Nobuyuki Miyasaka, Hitoshi Kohsaka
A 68-year-old man was admitted because of weakness of the left leg, dysesthesiae of the extremities and bilateral lower extremity purpura. A neurological examination showed mononeuritis multiplex with laboratory evidence of hypocomplementemia, cryoglobulinemia and leukocytoclastic vasculitis in the biopsy of a skin specimen. The patient also exhibited bilateral submandibular gland swelling, elevated serum IgG4 levels and infiltration of a large number of IgG4-positive plasma cells in the submandibular glands...
2016: Internal Medicine
Giuseppe Lopalco, Donato Rigante, Vincenzo Venerito, Giacomo Emmi, Maria Grazia Anelli, Giovanni Lapadula, Florenzo Iannone, Luca Cantarini
Inflammation mediated by cells of the immune system and necrosis are the most striking features observed at the histologic level in patients with vasculitides, clinical entities classified according to pathologic findings involving different organs, to etiology, or to size of vessels involved. Small vessel vasculitides (SVV) are a peculiar group of systemic disorders electively involving small intraparenchymal arteries, arterioles, capillaries, or venules and leading to different levels of vascular obstruction, tissue ischemia and risk of infarction; they can be divided into anti-neutrophil cytoplasmic antibody-associated vasculitides and immune complex vasculitides...
June 2016: Current Rheumatology Reports
Cesare Mazzaro, Luigino Dal Maso, Teresa Urraro, Endri Mauro, Laura Castelnovo, Pietro Casarin, Giuseppe Monti, Valter Gattei, Anna Linda Zignego, Gabriele Pozzato
BACKGROUND: Cryoglobulinemic vasculitis (CV) related to Hepatitis-B Virus (HBV) is rare and its treatment is ill-defined. AIMS: To describe clinical and treatment characteristics of HBV-related CV patients. In addition, the efficacy of treatment with antiviral agent nucleotide (NUC), including Entecavir, Adefovir, and Lamivudine, was explored. METHODS: In four Italian centres, 17 HBV-positive CV patients (median age 56 years, range 45-70) were enrolled...
July 2016: Digestive and Liver Disease
N Blank, H-M Lorenz
Cryoglobulinemic vasculitis is a disease of the small arteries and affects the skin, joints, peripheral nerves, kidneys and other organs. Even small amounts of cryoglobulins can lead to severe impairment for the patient but the detection of such low amounts of cryoglobulins can be difficult. The causes of cryoglobulinemic vasculitis include monoclonal hematological diseases, autoimmune diseases and chronic infections. Therapy involves treatment of the underlying disease and glucocorticoids, rituximab and plasmapheresis depending on the severity of the vasculitis...
April 2016: Zeitschrift Für Rheumatologie
Belkiz Ongen, Fehime Benli Aksungar, Bahattin Cicek, Isin Akyar, Abdurrahman Coskun, Mustafa Serteser, Ibrahim Unsal
A 60-year-old male patient presented with jaundice and dark urine for three days, icteric sclerae and skin rash on his legs for six months. Laboratory investigations revealed an atypical cryoglobulinemia with high hepatitis C virus (HCV)-RNA levels. Imaging studies showed cholestasis was accompanying HCV. Capillary zone electrophoresis using immunosubtraction method revealed a polyclonal immunoglobulin G and immunoglobulin A (IgA) monoclonal cryoglobulin and that IgA lambda was absent in immunofixation electrophoresis...
March 16, 2016: World Journal of Clinical Cases
Franco Dammacco, Vito Racanelli, Sabino Russi, Domenico Sansonno
Cryoglobulinemic vasculitis (CV) is a small-to-medium-vessel vasculitis that appears in 10-15 % of patients chronically infected with hepatitis C virus (HCV). The classic symptom triad of CV, purpura/asthenia/arthralgia, is accompanied by clinical features that include glomerulonephritis, neuropathy, interstitial pneumonitis, and cardiomyopathy, ranging in their severity from mild to life threatening. The risk of developing non-Hodgkin lymphoma is also higher. The cumulative 10-year survival rate of CV patients is significantly lower than in the age- and sex-matched general population, with death typically caused by nephropathy, malignancies, liver involvement, and severe infections...
August 2016: Clinical and Experimental Medicine
Marion Corouge, Anaïs Vallet-Pichard, Stanislas Pol
Chronic hepatitis C (CHC) is significantly associated with a risk of renal deterioration over time. Renal impairment, especially stage 4-5 chronic kidney disease, increases the risk of: (i) the prevalence and incidence (in dialysis/transplantation) of hepatitis C virus (HCV) infection; (ii) liver deterioration during kidney transplantation and (iii) allograft failure and patient mortality. HCV-infected dialysis patients have a higher mortality than non-infected dialysis patients and than HCV-infected kidney recipients...
January 2016: Liver International: Official Journal of the International Association for the Study of the Liver
Elane Reyes-Avilés, Lenche Kostadinova, Anne Rusterholtz, Angelica Cruz-Lebrón, Yngve Falck-Ytter, Donald D Anthony
Approximately half of those with chronic hepatitis C virus (HCV) infection have circulating rheumatoid factor (RF), and a portion of these individuals develop cryoglobulinemic vasculitis. B cell phenotype/function in relation to RF in serum has been unclear. We examined B cell subset distribution, activation state (CD86), cell cycle state (Ki67), and ex-vivo response to BCR, TLR9 and TLR7/8 stimulation, in chronic HCV-infected donors with or without RF, and uninfected donors. Mature-activated B-cells of HCV-infected donors had lower CD86 expression compared to uninfected donors, and in the presence of RF they also showed reduced CD86 expression in response to BCR and TLR9 stimulation...
2015: PloS One
Chen Wang, Zi-Yin Ye, De-Hua Zeng, Fei-Lai Xie, Li-Juan Qu, Zhi-Yong Zheng
AIMS: We retrospectively analyzed clinicopathologic features of 8 cases of hepatitis B virus-associated glomerulonephritis with hyaline thrombi, to confirm the diagnosis of cryoglobulinemic glomerulonephritis (CRYGN) associated with HBV infection. METHODS: Retrospective analysis was carried out with demographic information, clinical manifestations, laboratory parameters, pathological and prognostic features. RESULTS: The median age of 8 patients was 30...
2015: International Journal of Clinical and Experimental Pathology
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