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https://www.readbyqxmd.com/read/28339364/clinical-and-prognostic-features-of-korean-patients-with-mpo-anca-pr3-anca-and-anca-negative-vasculitis
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
https://www.readbyqxmd.com/read/28286108/international-therapeutic-guidelines-for-patients-with-hcv-related-extrahepatic-disorders-a-multidisciplinary-expert-statement
#7
REVIEW
Anna Linda Zignego, Manuel Ramos-Casals, Clodoveo Ferri, David Saadoun, Luca Arcaini, Dario Roccatello, Alessandro Antonelli, Anne Claire Desbois, Cloe Commarmond, Laura Gragnani, Milvia Casato, Peter Lamprecht, Alessandra Mangia, Athanasios G Tzioufas, Zobair M Younossi, Patrice Cacoub
Hepatitis C virus (HCV) is both hepatotrophic and lymphotropic virus that causes liver as well extrahepatic manifestations including cryoglobulinemic vasculitis, the most frequent and studied condition, lymphoma, and neurologic, cardiovascular, endocrine-metabolic or renal diseases. HCV-extrahepatic manifestations (HCV-EHMs) may severely affect the overall prognosis, while viral eradication significantly reduces non-liver related deaths. Different clinical manifestations may coexist in the same patient. Due to the variety of HCV clinical manifestations, a multidisciplinary approach along with appropriate therapeutic strategies are required...
March 7, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28285838/incidental-findings-on-pediatric-abdominal-magnetic-resonance-angiography
#8
Nattinee Leelakanok, Matthew A Zapala, Emily A Edwards, Andrew S Phelps, John D Mackenzie, Jesse Courtier
RATIONALE AND OBJECTIVES: Abdominal magnetic resonance angiography (MRA) has gained favor in pediatric patients owing to its lack of ionizing radiation and noninvasive nature. Reports exist regarding incidental findings on body MRA in adult patients. However, the incidental findings in pediatric abdominal MRA have not been previously reported. Our study aims to determine the frequencies, characteristics, and categories of incidental findings in pediatric patients undergoing abdominal MRA...
March 9, 2017: Academic Radiology
https://www.readbyqxmd.com/read/28285286/necrotizing-polyarteritis-nodosa-like-vasculitis-in-a-child-with-systemic-lupus-erythematosus
#9
Ritambhra Nada, Joseph L Matthews, Sagar Bhattad, Anju Gupta, Surjit Singh
A 10-year-old child presented with prolonged fever, lymphadenopathy, weight loss, oral ulcers, alopecia and parotitis. She later developed arterial thrombosis, poly-serositis, nephritis, myocarditis, sacro-ilitis, autoimmune hemolytic anemia and refractory thrombocytopenia. Though anti-dsDNA was negative, she was diagnosed to have systemic lupus erythematosus (SLE). Terminally, she had pulmonary symptoms and succumbed to her illness. The autopsy showed lupus nephritis-Class II, polyserositis, myocarditis, inflammatory myositis, immune mediated vasculitis involving renal, coronary, pancreatic, adrenal, dermal and intramuscular arteries, and pulmonary hemorrhages and edema...
February 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28260443/outcome-of-renal-transplant-in-recipients-with-vasculitis
#10
Samia Barbouch, Meriam Hajji, Raja Aoudia, Monther Ounissi, Asma Zammouri, Rym Goucha, Fathi Ben Hamida, Mohammed Mongi Bacha, Ezzedine Abderrahim, Taieb Ben Abdallah
OBJECTIVES: End-stage renal disease develops in a high percentage of patients with vasculitis, in whom kidney transplant has become a therapeutic option. However, limited data are available on the prognosis and outcomes after kidney transplant in these patients. We aimed to compare the long-term graft survival and graft function in 8 renal transplant recipients with vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, Goodpasture syndrome, and Henoch-Schonlein purpura) with the other kidney recipients at a single center...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28250302/henoch-sch%C3%A3-nlein-purpura-nephritis-associated-with-intravesical-bacillus-calmette-guerin-bcg-therapy
#11
Hiroyuki Tsukada, Hiroshi Miyakawa
Henoch Schönlein purpura (HSP), also known as IgA vasculitis (IgAV), is a systemic small-vessel vasculitis that predominantly affects adolescents and is rare in adults. In many cases, the onset of HSP has been causally linked to an infectious disease. We encountered a case of HSP with severe renal involvement diagnosed by renal biopsy following bacillus Calmette-Guerin (BCG) therapy for bladder cancer. This is of clinical relevance, as intravesical BCG administration is becoming an established therapy for superficial bladder cancer and is supposed to be safe...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28240590/a-meta-analysis-of-avascular-necrosis-in-systemic-lupus-erythematosus-prevalence-and-risk-factors
#12
Tatiana Nevskaya, Maeve P Gamble, Janet E Pope
OBJECTIVES: To determine the prevalence of and risk factors for avascular necrosis (AVN) in systemic lupus erythematosus (SLE). METHODS: MEDLINE, CINAHL, Web of Science, EMBASE and Cochrane Library were searched from inception to July, 2015 and a random effects model was used to combine frequencies; study quality was assessed using STROBE. RESULTS: 2,041 citations identified 62 articles. Many results had high heterogeneity. The prevalence of symptomatic AVN was 9% (range 0...
February 24, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28229825/anca-associated-pauci-immune-glomerulonephritis-always-pauci-immune
#13
Valeria Scaglioni, Marina Scolnik, Luis J Catoggio, Silvia B Christiansen, Carlos F Varela, Gustavo Greloni, Guillermo J Rosa-Diez, Enrique R Soriano
OBJECTIVES: Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (GN) is considered "pauci-immune" with absent or mild glomerular tuft staining for immunoglobulin (Ig) and/or complement. However, it is not unusual to see some immune deposits (ID) within glomeruli on immunofluorescence (IF). We determined to evaluate the prevalence and clinical significance of immune deposits in ANCA-associated GN. METHODS: We included all patients with ANCA associated vasculitis with renal biopsies between January 2002 and May 2014: granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis and renal limited vasculitis...
January 31, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28228431/right-upper-quadrant-abdominal-pain-as-the-initial-presentation-of-polyarteritis-nodosa
#14
Ricardo Gago, Lee Ming Shum, Luis M Vilá
Polyarteritis nodosa (PAN) is a necrotising vasculitis that involves medium and small vessels. PAN generally presents with constitutional, cutaneous, neurological, renal and gastrointestinal manifestations. However, PAN initially involving a single organ/system is uncommon. Here, we present a 42-year-old man who was hospitalised because of severe right upper quadrant abdominal pain that started 2 months before. Physical examination was remarkable for right upper quadrant abdominal tenderness. Abdominopelvic CT showed lymphadenopathy but no hepatic, gallbladder, pancreatic, intestinal or renal abnormalities...
February 22, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28225490/clinical-characteristics-and-outcome-of-spanish-patients-with-anca-associated-vasculitides-impact-of-the-vasculitis-type-anca-specificity-and-treatment-on-mortality-and-morbidity
#15
MULTICENTER STUDY
Roser Solans-Laqué, Guadalupe Fraile, Monica Rodriguez-Carballeira, Luis Caminal, Maria J Castillo, Ferran Martínez-Valle, Luis Sáez, Juan J Rios, Xavier Solanich, Joaquim Oristrell, Francisco Pasquau, Eva Fonseca, Monica Zamora, Jose L Callejas, Begoña Frutos, Monica Abdilla, Patricia Fanlo, Isabel García-Sánchez, Miguel López-Dupla, Bernardo Sopeña, Almudena Pérez-Iglesias, Josep A Bosch
The aim of this study was to describe the clinical characteristics of ANCA-associated vasculitides (AAV) at presentation, in a wide cohort of Spanish patients, and to analyze the impact of the vasculitis type, ANCA specificity, prognostic factors, and treatments administered at diagnosis, in the outcome.A total of 450 patients diagnosed between January 1990 and January 2014 in 20 Hospitals from Spain were included. Altogether, 40.9% had granulomatosis with polyangiitis (GPA), 37.1% microscopic polyangiitis (MPA), and 22% eosinophilic granulomatosis with polyangiitis (EGPA)...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28222228/neutrophil-to-lymphocyte-ratio-a-biomarker-for-predicting-systemic-involvement-in-adult-iga-vasculitis-patients
#16
Géza Róbert Nagy, Lajos Kemény, Zsuzsanna Bata-Csörgő
BACKGROUND: IgA vasculitis (IgAV) is a small-vessel leucocytoclastic cutaneous vasculitis, often associated with kidney and gastrointestinal (GI) manifestations. Although predictive factors for systemic involvement have been extensively studied in children, there is paucity in the literature regarding adult patients. Neutrophil-to-lymphocyte ratio (NLR) is an inflammatory marker, used to assess systemic inflammation in various diseases. OBJECTIVE: We sought to evaluate whether NLR can be used for predicting renal and GI involvement in adult IgA vasculitis patients...
February 21, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28210182/successful-cataract-surgery-in-a-patient-with-refractory-wegener-s-granulomatosis-effectively-treated-with-rituximab-a-case-report
#17
Abdullah M Alfawaz
Wegener's granulomatosis is a granulomatous disorder associated with systemic necrotizing vasculitis. Eye involvement occurs in approximately 50% of Wegener's granulomatosis patients and is an important cause of morbidity. Conventional treatment-related morbidity and failure have led to studies of alternative treatment modalities. In this case of a 35-year-old man with severe Wegener's granulomatosis, conventional therapy failed to induce remission. Despite the standard immunosuppressive therapy, progression of the disease was observed, mainly with ocular manifestations and renal impairment...
July 2016: Saudi Journal of Ophthalmology: Official Journal of the Saudi Ophthalmological Society
https://www.readbyqxmd.com/read/28207635/kidney-transplantation-rates-across-glomerulonephritis-subtypes-in-the-united-states
#18
Michelle M O'Shaughnessy, Sai Liu, Maria E Montez-Rath, Richard A Lafayette, Wolfgang C Winkelmayer
BACKGROUND: Whether kidney transplantation rates differ by glomerulonephritis (GN) subtype remains largely unknown. METHODS: Using the US Renal Data System, we identified all adult patients with ESRD attributed to 1 of 6 GN subtypes who initiated dialysis in the US (1996-2013). Patients with diabetic nephropathy (DN) and autosomal-dominant polycystic kidney disease (ADPKD) served as "external" non-GN comparators. Using Cox proportional hazards regression, with death considered a competing risk, we estimated hazard ratios [HRs (95% confidence intervals)] for first kidney transplantation, controlling for year, demographics, comorbidities, socioeconomic factors, and Organ Procurement Organization (OPO)...
February 15, 2017: Transplantation
https://www.readbyqxmd.com/read/28204946/clinical-outcomes-in-children-with-henoch-sch%C3%A3-nlein-purpura-nephritis-without-crescents
#19
Jean Daniel Delbet, Julien Hogan, Bilal Aoun, Iulia Stoica, Rémi Salomon, Stéphane Decramer, Isabelle Brocheriou, Georges Deschênes, Tim Ulinski
BACKGROUND: Henoch-Schönlein purpura is the most common vasculitis in children. Its long-term prognosis depends on renal involvement. The management of Henoch-Schönlein purpura nephritis (HSPN) remains controversial. This study reports the prognosis of children with HSPN presenting with class 2 International Study of Kidney Disease in Children (ISKDC) nephritis. METHODS: All children with HSPN class 2 diagnosed between 1995 and 2015 in four pediatric nephrology centers were included, and clinical and biological data were collected from the medical files...
February 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28202741/seasonal-influence-on-the-risk-of-relapse-at-a-rise-of-antineutrophil-cytoplasmic-antibodies-in-vasculitis-patients-with-renal-involvement
#20
Michael J Kemna, Jan Willem Cohen Tervaert, Kelly Broen, Sjoerd A M E G Timmermans, Pieter van Paassen, Jan G M C Damoiseaux
OBJECTIVE: The objective of this study was to identify risk factors for a relapse at the time of an increase in antineutrophil cytoplasmic antibodies (ANCA) in patients with renal ANCA-associated vasculitis (AAV). METHODS: All patients between January 2000 and November 2011 with renal AAV having an ANCA rise during remission were included. Differences in time to relapse since the ANCA rise were assessed using a Cox regression model. The level of 25-hydroxy Vitamin D (25(OH)D) was assessed at the ANCA rise and at a subsequent relapse or time-matched during remission...
February 15, 2017: Journal of Rheumatology
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