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https://www.readbyqxmd.com/read/28416004/clinical-study-of-children-with-takayasu-arteritis-a-retrospective-study-from-a-single-center-in-china
#1
Ye Feng, Xuemei Tang, Mingyue Liu, Juan Zhou, Xiaodong Zhao, Qiu Li
BACKGROUND: Delayed diagnosis of childhood Takayasu arteritis (TA) is common due to its atypical symptoms. The objective of the present study was to summarize the clinical features of childhood TA to raise awareness and improve management. METHODS: Eleven children diagnosed with TA at our hospital were enrolled. Clinical information, diagnosis, treatment, and outcome were then examined retrospectively. The Pediatric Vasculitis Activity Score (PVAS) and the Indian Takayasu Clinical Activity Score (ITAS2010) were used to assess disease activity...
April 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28414152/rituximab-for-immunologic-renal-disease-what-the-nephrologist-needs-to-know
#2
REVIEW
Andreas Kronbichler, Martin Windpessl, Herwig Pieringer, David R W Jayne
Rituximab (RTX), a chimeric, monoclonal anti-CD20 antibody, is increasingly used in immune-mediated renal diseases. While licensed in the induction treatment of ANCA-associated vasculitis, it represents one of the most commonly prescribed off-label drugs. Much of the information regarding its safety has been drawn from experience in hematology and rheumatology. Ample evidence illustrates the safety of RTX, however, rare but serious adverse events have emerged that include progressive multifocal leucoencephalopathy and hepatitis B reactivation...
April 13, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28397718/clinical-predictors-of-outcome-in-patients-with-anti-neutrophil-cytoplasmic-autoantibody-related-renal-vasculitis-experiences-from-a-single-center
#3
Lei Pu, Gui-Sen Li, Yu-Rong Zou, Ping Zhang, Li Wang
BACKGROUND: Primary anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a chronic autoimmune disease associated with multisystem dysfunction. Renal involvement is common and closely associated with outcome. The purpose of this study was to investigate the clinical determinants of mortality of patients with AAV-related renal injury in the first 2 years after diagnosis in a single West Chinese center. METHODS: Demographic and laboratory parameters of 123 consecutive patients with AAV-related renal injury diagnosed in Renal Division and Institute of Nephrology, Sichuan Provincial People's Hospital between 2004 and 2012 were collected retrospectively...
April 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28391344/is-there-a-role-for-tnf%C3%AE-blockade-in-anca-associated-vasculitis-and-glomerulonephritis
#4
Stephen P McAdoo, Charles D Pusey
Tumour necrosis factor alpha (TNFα) is a cytokine that is pivotal in the inflammatory response. Blockade of TNFα has been shown to be effective in a number of human autoimmune diseases, including rheumatoid arthritis, raising the question of whether this approach may be effective in inflammatory kidney disease, such as ANCA-associated vasculitis (AAV). In AAV, there is considerable evidence for the role of TNFα in the pathophysiology of disease, including increased expression of TNFα mRNA in leucocytes and in renal tissue...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28382598/patients-with-anca-associated-vasculitis-admitted-to-the-intensive-care-unit-with-acute-vasculitis-manifestations-a-retrospective-and-comparative-multicentric-study
#5
Julien Demiselle, Johann Auchabie, François Beloncle, Philippe Gatault, Steven Grangé, Damien Du Cheyron, Jean Dellamonica, Sonia Boyer, Dimitri Titeca Beauport, Lise Piquilloud, Julien Letheulle, Christophe Guitton, Nicolas Chudeau, Guillaume Geri, François Fourrier, René Robert, Emmanuel Guérot, Julie Boisramé-Helms, Pierre Galichon, Pierre-François Dequin, Alexandre Lautrette, Pierre-Edouard Bollaert, Ferhat Meziani, Loïc Guillevin, Nicolas Lerolle, Jean-François Augusto
PURPOSE: Data for ANCA-associated vasculitis (AAV) patients requiring intensive care are scarce. METHODS: We included 97 consecutive patients with acute AAV manifestations (new onset or relapsing disease), admitted to 18 intensive care units (ICUs) over a 10-year period (2002-2012). A group of 95 consecutive AAV patients with new onset or relapsing disease, admitted to two nephrology departments with acute vasculitis manifestations, constituted the control group...
December 2017: Annals of Intensive Care
https://www.readbyqxmd.com/read/28374407/cutaneous-manifestations-of-diabetes-mellitus-a-review
#6
REVIEW
Ana Luiza Lima, Tanja Illing, Sibylle Schliemann, Peter Elsner
Diabetes mellitus is a widespread endocrine disease with severe impact on health systems worldwide. Increased serum glucose causes damage to a wide range of cell types, including endothelial cells, neurons, and renal cells, but also keratinocytes and fibroblasts. Skin disorders can be found in about one third of all people with diabetes and frequently occur before the diagnosis, thus playing an important role in the initial recognition of underlying disease. Noninfectious as well as infectious diseases have been described as dermatologic manifestations of diabetes mellitus...
April 3, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28356675/thrombotic-microangiopathy-due-to-viperidae-bite-two-case-reports
#7
T Dineshkumar, J Dhanapriya, R Sakthirajan, K Thirumalvalavan, A A Kurien, T Balasubramaniyan, N Gopalakrishnan
Snake bite is mainly an occupational hazard and causes serious health problems in rural India. Acute kidney injury (AKI) occurs in 5-30% cases. Renal pathologic findings include acute tubular necrosis, cortical necrosis, interstitial nephritis, glomerulonephritis, and vasculitis. Thrombotic microangiopathy (TMA) occurrence after a snake bite is reported rarely. Here, we present two patients who developed TMA after viper bite treated with hemodialysis and plasmapheresis. Renal biopsy showed fibrin thrombi in glomeruli and arterioles with cortical necrosis...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28356661/anti-glomerular-basement-membrane-disease-case-series-from-a-tertiary-center-in-north-india
#8
D Prabhakar, M Rathi, R Nada, R W Minz, V Kumar, H S Kohli, V Jha, K L Gupta
Anti-glomerular basement (anti-GBM) disease is an uncommon disorder with a bimodal age of presentation. Patients presenting with dialysis-dependent renal failure have poor renal outcomes. There is limited data regarding the clinical presentation and outcomes of anti-GBM disease from India. We conducted this prospective study to analyze the clinical presentation and outcomes of anti-GBM disease at a large tertiary care hospital in North India over 1½ years. Subjects with a biopsy proven anti-GBM disease (light microscopic examination showing crescents and immunofluorescence examination showing linear deposition of IgG) with or without positive anti-GBM antibodies in serum were included in the study and followed-up for at least 12 months...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28356384/il-26-confers-proinflammatory-properties-to-extracellular-dna
#9
Caroline Poli, Jean François Augusto, Jonathan Dauvé, Clément Adam, Laurence Preisser, Vincent Larochette, Pascale Pignon, Ariel Savina, Simon Blanchard, Jean François Subra, Alain Chevailler, Vincent Procaccio, Anne Croué, Christophe Créminon, Alain Morel, Yves Delneste, Helmut Fickenscher, Pascale Jeannin
In physiological conditions, self-DNA released by dying cells is not detected by intracellular DNA sensors. In chronic inflammatory disorders, unabated inflammation has been associated with a break in innate immune tolerance to self-DNA. However, extracellular DNA has to complex with DNA-binding molecules to gain access to intracellular DNA sensors. IL-26 is a member of the IL-10 cytokine family, overexpressed in numerous chronic inflammatory diseases, in which biological activity remains unclear. We demonstrate in this study that IL-26 binds to genomic DNA, mitochondrial DNA, and neutrophil extracellular traps, and shuttles them in the cytosol of human myeloid cells...
March 29, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28352031/vasculitis-and-vasculopathy-in-lupus-nephritis-clinical-variability-outcome-and-new-insight-into-treatment
#10
A Kaul, V Agrawal, D Bhaduaria, Vikas Agrawal, Narayan Prasad, Amit Gupta, R K Sharma
More than 50% of patients with systemic lupus erythematosus (SLE) have renal involvement at presentation or during their illness. Lupus nephritis (LN) encompasses several patterns of renal disease, including glomerular, tubulointerstitial, and vascular pathologies. The presence and significance of renal vascular lesions (VLs) are often overlooked. VLs in LN are not rare with an incidence of 10%-40% on renal biopsies from various studies and their presence is often labeled as poor prognostic markers. The current treatment protocol for LN is mainly based on the glomerular pathology, and no guidelines/consensus exists for treatment of LN with VLs...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28352023/anti-glomerular-basement-membrane-a-rare-cause-of-renal-failure-in-children
#11
Indira Agarwal, Ahmad Al-Ghitany
Anti-glomerular basement membrane (GBM) disease is a rare cause of acute renal failure and known to have bad prognosis regarding renal functions recovery and patient survival specially when diagnosed late and presents with severe renal failure that requires dialysis. We report a case of 11-year-old child with acute renal failure secondary to anti-GBM disease and associated with antineutrophil cytoplasmic antibody-positive vasculitis. He was treated with plasmapheresis, steroids, and cyclophosphamide with recovery of his kidney functions...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28352011/biopsy-of-small-kidneys-a-safe-and-a-useful-guide-to-potentially-treatable-kidney-disease
#12
Kamel El-Reshaid, Wael El-Reshaid, Dalal Al-Bader, Jozsef Varro, John Madda, Hosameldin Tawfik Sallam
Over the past four years, all patients with unexplained rapid progression of their renal disease were subjected to kidney biopsy, despite their small size (<9 cm), to define its etiology. Children, pregnant women, morbidly obese patients, and those with an unstable cardiovascular state, septicemia, bleeding diathesis as well as those kidney size with size <6 cm were excluded from the study. Doppler ultrasound was used to exclude renovascular/ischemic nephropathy. The procedure was performed by an interventional radiologist using a biopsy gun technique and under ultrasound guidance...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28348948/microscopic-polyangiitis-atypical-presentation-with-extensive-small-bowel-necrosis-diffuse-alveolar-hemorrhage-and-renal-failure
#13
Justin M Segraves, Vivek N Iyer
Microscopic polyangiitis is an uncommon systemic vasculitis of varying severity that is associated with myeloperoxidase (MPO) and perinuclear antineutrophil cytoplasmic (p-ANCA) antibodies. The most commonly affected organs are the lungs and kidneys. We report on a very unusual case of microscopic polyangiitis presenting with severe mesenteric ischemia in addition to diffuse alveolar hemorrhage and acute renal failure. The patient was initially diagnosed with acute pancreatitis at an outside facility given his severe abdominal pain and elevated pancreatic enzymes...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28348913/a-rare-case-of-digital-ischemia-and-gangrene-in-anca-associated-vasculitis-with-review-of-the-literature
#14
Richard A Lau, Ramandeep Bains, Duminda Suraweera, Jane Ma, Emil R Heinze, Andrew L Wong, Philip J Clements
This paper describes one patient with Antineutrophil Cytoplasmic Antibody- (ANCA-) associated vasculitis who initially presented with multiple ischemic fingers and toes. On further evaluation, the patient was also found to have pulmonary-renal involvement and episcleritis. The diagnosis was supported with a positive cANCA (anti-proteinase 3) and a bronchoscopy consistent with diffuse alveolar hemorrhage. Although the patient refused a tissue biopsy, clinical presentation including nasal ulceration, sinus congestion, and epistaxis and anti-proteinase 3 antibody were more consistent with Granulomatosis with Polyangiitis (GPA) rather than Microscopic Polyangiitis (MPA) or Eosinophilic Granulomatosis with Polyangiitis (EGPA) based on the recently presented ACR/EULAR Provisional 2017 Classification Criteria for GPA (Luqmani et al...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28339364/clinical-and-prognostic-features-of-korean-patients-with-mpo-anca-pr3-anca-and-anca-negative-vasculitis
#15
Juyoung Yoo, Ho Jae Kim, Sung Soo Ahn, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
OBJECTIVES: We reclassified Korean patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) into 3 categories of AAV including MPO-ANCA, PR3-ANCA and ANCA negative vasculitis, and investigated clinical and prognostic features. METHODS: We reviewed the medical records of 133 patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA), who had having either myeloperoxidase (MPO)-ANCA, proteinase 3 (PR3)-ANCA or no ANCA, and who had ever achieved the first remission...
March 23, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28337860/clinical-features-and-long-term-outcomes-of-105-granulomatosis-with-polyangiitis-patients-a-single-center-experience-from-north-india
#16
Aman Sharma, Godasi S R S N K Naidu, Manish Rathi, Roshan Verma, Manish Modi, Benzeeta Pinto, Kusum Sharma, Varun Dhir, Manphool Singhal, Mahesh Prakash, Ritambhra Nada, Naresh K Panda, Ranjana W Minz
AIM: To describe the clinical features, treatment and long-term outcomes in north Indian patients with granulomatosis with polyangiitis (GPA). METHODS: Clinical details, Birmingham Vasculitis Activity Score (BVAS-v3), laboratory data, histopathology findings, treatment details and outcomes of all consecutive patients diagnosed as having GPA between April 2005 and April 2016 were retrieved. European Vasculitis Study Group definitions were used to classify patients into localized, early systemic, generalized, severe and refractory categories...
March 24, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28329555/dermatomyositis-clinically-presenting-with-cutaneous-ulcers-with-histopathologic-evidence-of-perforating-collagenosis
#17
Rachel Rosenstein, Kathryn Martires, Mitalee Christman, Vitaly Terushkin, Shane A Meehan, Nicole Seminara, Brian D Golden, Andrew G Franks
Dermatomyositis is a systemic, autoimmune diseasewith a variety of clinical features that often includemyositis and characteristic cutaneous findings. Asubset of patients with dermatomyositis developcutaneous ulcers, often in the setting of vasculitis orvasculopathy. We present a case of dermatomyositiswith cutaneous ulcers that show perforatingcollagenosis on histopathologic examination.Acquired reactive perforating collagenosistypically occurs in the setting of diabetes mellitus,chronic renal failure, and other pruritic conditions,and this case represents a rare association withdermatomyositis, which may ultimately be helpful inelucidating the pathophysiology of this perforatingdisorder...
December 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28302661/severe-co-trimoxazole-induced-hypoglycaemia-in-a-patient-with-microscopic-polyangiitis
#18
Thomas Edward Conley, Atif Mohiuddin, Noshaba Naz
A 69-year-old man presented to the emergency department with lower respiratory tract infection and febrile neutropaenia. He was recently discharged following a 50-day hospital stay with newly diagnosed microscopic polyangiitis, complicated by pulmonary haemorrhage and severe renal dysfunction requiring renal replacement therapy, plasma exchange and immunosuppression (cyclophosphamide and methylprednisolone). High risk of pneumocystis pneumonia (PCP) led to an escalation in treatment from prophylactic to therapeutic oral co-trimoxazole, alongside broad-spectrum antibiotics...
March 16, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28291241/efficacy-and-safety-of-direct-acting-antivirals-for-the-treatment-of-mixed-cryoglobulinemia
#19
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/28289874/renal-vasculitis-presenting-with-acute-kidney-injury
#20
Javier Villacorta, Francisco Diaz-Crespo, Mercedes Acevedo, Teresa Cavero, Carmen Guerrero, Manuel Praga, Gema Fernandez-Juarez
Renal failure secondary to ANCA-associated vasculitis represents a clinical and therapeutic challenge. In this study, we aimed to assess the treatment response rates and long-term outcomes of vasculitis patients presenting with renal failure. This retrospective study included 151 patients with renal vasculitis from three hospitals who underwent a renal biopsy between 1997 and 2014. Patients with renal failure which required dialysis at the onset were compared to those presenting with more preserved renal function...
March 13, 2017: Rheumatology International
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