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Marie-Nathalie Kolopp-Sarda, Pierre Miossec
Cryoglobulins are immunoglobulins precipitating in cold condition. They are classified in 3 types according to the Brouet classification and may lead to vasculitis of small and medium size vessels. Vasculitis is related to vessel obstruction by monoclonal cryoglobulin aggregates in type I cryoglobulins and immune complex deposition in type II and III mixed cryoglobulins. This phenomenon is favored by low temperature, especially in skin, joints, and peripheral nerves, or increased cryoglobulin concentration in kidneys...
March 8, 2018: Autoimmunity Reviews
M Rato, F Gil, A F Monteiro, J Aranha, E Tavares
A 55-year-old man presented with a history of asymptomatic, bilateral, figurate dermatosis consisting of erythematous, annular, linear, andarciform, indurated nodules and plaques on the lateral walls of thorax, flanks, and hypogastrium. His medical history was positive for chronic hepatitis C. Skin biopsy was compatible with the diagnosisof interstitial granulomatous dermatitis. Additionalinvestigation revealed positive rheumatoid factor, antinuclear antibodies, and cryoglobulins. Thepatient started treatment with high-potency topical corticosteroid...
January 15, 2018: Dermatology Online Journal
Yasunori Minami, Masatoshi Kudo
Cryoglobulins are produced by the over-stimulated immune system of patients with hepatitis C virus (HCV). HCV-induced cryoglobulinemic vasculitis primarily affects small-sized vessels of systemic organs. The most common symptoms are purpura, joint pain, and peripheral neuropathy or Raynaud phenomenon. The blood tests of patients with HCV may exhibit raised erythrocyte sedimentation rate and C-reactive protein levels, positive rheumatoid factor and cryoglobulins, and decreased complement levels. Therapeutic options for HCV-induced cryoglobulinemic vasculitis, include anti-virals, Rituximab, steroids, immunosuppressant drugs, and plasma exchange...
February 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
Xin-Xin Cao, Zhuang Tian, Lu Lin, Jian Sun, Wei Su, Dao-Bin Zhou, Jian Li
Cryoglobulinemic vasculitis is a rare and frequently fatal type of myocarditis. Cardiac manifestations in type 1 cryoglobulinemic vasculitis have never been reported to our knowledge. We report a rare case of type 1 cryoglobulinemic vasculitis with cardiac involvement in a patient who experienced progressive heart failure during the diagnosis. The diagnosis was made by the presence of cryoglobulins and endomyocardial biopsy results. After bortezomib-containing treatments, plasma cryoglobulin levels returned to normal, and the patient's clinical condition gradually improved...
December 28, 2017: Canadian Journal of Cardiology
Rita Moretti, Paola Caruso, Matteo Dal Ben, Silvia Gazzin, Claudio Tiribelli
BACKGROUND: Peripheral neuropathy is one most common, limiting and invalidating neurological symptom in subjects with hepatitis C virus and mixed cryoglobulinemia. Notably, the medical therapy proposed to eradicate HCV, can frequently exacerbate the painful neuropathy. Therefore, neuropathy therapies are insufficient and inadequate, and comprise immunosuppressive drugs, such as steroid or cyclosporine, intravenous immunoglobulin or plasma exchange. These have shown variable success in case reports, with a presumably temporary effect, but with major side effects...
January 25, 2018: BMC Gastroenterology
Nicole A Sitkin, Ethan Bernstein, Mark A Perazella
Classical polyarteritis nodosa (PAN) is a necrotizing arteritis affecting medium-sized vessels. A 73-year-old gentleman who presented with progressive anemia and scant hemoptysis developed acute kidney injury over the course of hospital admission. Kidney biopsy was initially interpreted as consistent with cephalosporin-associated acute interstitial nephritis (AIN) and acute tubular injury (ATI). After oral prednisone (5 days), the patient was discharged with improving serum creatinine. Two days later, he presented with diffuse alveolar hemorrhage...
November 22, 2017: Clinical Nephrology
Kabeerdoss Jayakanthan, And Nikhil Gupta, John Mathew, Raheesh Ravindran, Gowri Mahasampth, Debashish Danda
Objective: Anti-C1q antibodies (Anti-C1q Ab) are seen in hypocomplementemic urticarial vasculitis syndrome (HUVS), infection-associated vasculitis such as hepatitis C virus-related vasculitis and in autoimmune diseases such as rheumatoid vasculitis, polyarteritis nodosa, giant cell arteritis, vascular Behcet's disease, and cryoglobulin associated vasculitis. Aim of this study is to evaluate the presence of Anti-C1q Ab in vasculitis and to determine if any difference exists between primary and secondary vasculitis in relation to this antibody...
November 2017: International Journal of Health Sciences
Nelson Leung, Maria E Drosou, Samih H Nasr
Dysproteinemia is characterized by the overproduction of an Ig by clonal expansion of cells from the B cell lineage. The resultant monoclonal protein can be composed of the entire Ig or its components. Monoclonal proteins are increasingly recognized as a contributor to kidney disease. They can cause injury in all areas of the kidney, including the glomerular, tubular, and vascular compartments. In the glomerulus, the major mechanism of injury is deposition. Examples of this include Ig amyloidosis, monoclonal Ig deposition disease, immunotactoid glomerulopathy, and cryoglobulinemic GN specifically from types 1 and 2 cryoglobulins...
January 6, 2018: Clinical Journal of the American Society of Nephrology: CJASN
Adrienne Lenhart, Alireza Meighani, Mona Hassan, Stuart Gordon
Chronic hepatitis C virus infection (HCV) can present with cryoglobulinemic vasculitis, which is primarily associated with type II/III cryoglobulins. Type I cryoglobulins are usually seen in lymphoproliferative disease, and large vessel involvement with this type of vasculitis is rare. A 70-year-old man with chronic HCV presented with abdominal pain, leukocytosis, and rash. Computed tomography angiography showed thickening of the abdominal aorta consistent with large-vessel vasculitis. He was found to have type I cryoglobulinemia and was treated with corticosteroids and ledipasvir/sofosbuvir with rapid resolution of his aortitis...
2017: ACG Case Reports Journal
Victoria Alegría-Landa, Lorenzo Cerroni, Heinz Kutzner, Luis Requena
Cutaneous manifestations secondary to paraprotein deposits in the skin include a group of different disorders that although rare, may be the first clinical manifestation of the underlying hematologic dyscrasia. In this article we review the clinical manifestations and histopathologic findings of the processes that result from specific deposition of the paraprotein in different structures of the skin. Paraneoplastic processes frequently associated with hematologic malignancies will not be covered in this review...
December 2017: Journal of the American Academy of Dermatology
Jesse Kimman, Xavier Bossuyt, Daniel Blockmans
BACKGROUND: Patients with Sjögren's syndrome (SS) have a considerable higher risk of lymphoma development. OBJECTIVES: To determine the incidence of lymphoma and the value of biomarkers to predict lymphoma development in patients with SS. METHODS: Clinical files of all patients with a presumed diagnosis of SS between 1991 and 2016 were retrospectively reviewed for the development of lymphoma. Biochemical data were plotted as a function of the relative time before and after the lymphoma diagnosis (for patients who developed lymphoma) or before the last available blood test (for patients who did not develop lymphoma)...
October 3, 2017: Acta Clinica Belgica
Jung-Lung Hsu, Ming-Feng Liao, Hui-Ching Hsu, Yi-Ching Weng, Ai-Lun Lo, Kuo-Hsuan Chang, Hong-Shiu Chang, Hung-Chou Kuo, Chin-Chang Huang, Long-Sun Ro
OBJECTIVE: To investigate the clinical characteristics of patients with uncommon distal symmetric painful small-fiber neuropathy (DSPSFN). METHODS: From September 2012 to September 2014, participants between 18-70 years of age that had DSPSFN defined by clinical signs/symptoms and ID pain > 2 or DN4 > 4 on questionnaires for more than 1 month were included. Participants who had previous historical or laboratory evidence of common etiologies of DSPSFN were excluded...
2017: PloS One
M Ndiaye, B Lebrun-Vignes, N Ortonne, L Fardet
BACKGROUND: A wide variety of drugs can cause cutaneous vasculitis. Herein we report a case of immune complex vasculitis induced by amiodarone. PATIENTS AND METHODS: A 57-year-old patient reported a recent history of pruritus associated with large erythematous, inflammatory, necrotic plaques localized on the lower limbs and back. These cutaneous lesions had appeared less than 2 months after initiation of amiodarone for supra-ventricular arrhythmia. Histological and direct immunofluorescence examinations of a skin biopsy sample revealed vasculitis with the presence of IgM and C3 immune complexes in vessels...
September 18, 2017: Annales de Dermatologie et de Vénéréologie
Praveen K Veerasubramanian, Jayakanthan Kabeerdoss, Pulukool Sandhya, Suresh Devasahayam, Debashish Danda
INTRODUCTION: Maintenance of temperature during collection and transport of blood is an important pre-requisite for cryoglobulin assays. In this manuscript, we describe 'cryodevice', a low-cost device for transportation and/or incubation of vials of whole blood at 37°C. Such a device would reduce false negatives in cryoglobulin assays. METHOD: The 'cryodevice' takes the embodiment of a portable, light, insulated water bath, which can be used as an incubator in a plugged-in state, or as a transport container after it is set up and disconnected from the power supply...
September 11, 2017: International Journal of Rheumatic Diseases
Qiao-Yan Guo, Man Wu, Yang-Wei Wang, Guang-Dong Sun
Hepatitis C virus (HCV) is a major cause of liver-associated morbidity and has an increasing prevalence worldwide. Hepatitis C virus infection may lead to chronic hepatitis, cirrhosis and liver failure. However, it is also associated with a wide range of extra-hepatic complications, such as cryoglobulinemia, an immune complex disease associated with cryoglobulin leading to multiple organ damage and, while the major symptom is vasculitis. The present study reported on a-58-year-old woman who was diagnosed with HCV-associated cryoglobulinemia with skin, kidney and blood system damage and biopsy-proven cryoglobulinemia membrano-proliferative glomerulonephritis...
August 2017: Experimental and Therapeutic Medicine
Tainá Mosca, Simone Sanches Arcuri, Luiz Antônio Miorin, Andrelita de Oliveira Cristiano, Yvoty Alves Dos Santos Sens, Wilma Carvalho Neves Forte
BACKGROUND: The presence of cryoglobulins in patients with chronic kidney disease (CKD) on hemodialysis is well described. However, the generation of cryoglobulins during the dialysis treatment has yet to be established. The aim of the present study was to determine the presence of serum cryoglobulins over time in the dialysis treatment in patients with CKD not infected with hepatitis C virus (HCV). METHOD: Peripheral blood samples were collected at the beginning of dialysis treatment and at 30, 60, 90 and 120 days afterwards...
November 2017: Renal Failure
Silvia Squarza, Alberto Galli, Maurizio Cariati, Federico Alberici, Valentina Bertolini, Fabio Frediani, Carla Uggetti
A 56-year-old man with behavioural disorders and facial-brachio-crural right hemiparesis presented with a brain lesion studied with computed tomography, magnetic resonance imaging and brain biopsy, leading to the diagnosis of cerebral vasculitis. Hepatitis C virus (HCV) infection in a phase of activity, without cryoglobulins, was also detected. Brain biopsy, laboratory analysis and response to a specific therapy supported the diagnosis of central nervous system vasculitis that was HCV related.
January 1, 2017: Neuroradiology Journal
K Balendran, L D S U Senarathne, R D Lanerolle
BACKGROUND: Rheumatoid arthritis is a systemic disorder where clinically significant renal involvement is relatively common. However, crescentic glomerular nephritis is a rarely described entity among the rheumatoid nephropathies. We report a case of a patient with rheumatoid arthritis presenting with antineutrophil cytoplasmic antibody-negative crescentic glomerular nephritis. CASE PRESENTATION: A 54-year-old Sri Lankan woman who had recently been diagnosed with rheumatoid arthritis was being treated with methotrexate 10 mg weekly and infrequent nonsteroidal anti-inflammatory drugs...
July 21, 2017: Journal of Medical Case Reports
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
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
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