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treatment of renal vasculitic diseases

Gulen Hatemi, Sinem N Esatoglu, Yusuf Yazici
PURPOSE OF REVIEW: Biomarkers are considered to be helpful in diagnosing, monitoring, predicting treatment response, and prognosis in clinical practice and as outcomes in clinical trials. In this article, we review the recent literature on new biomarkers and the expanding use of older ones in vasculitic conditions. RECENT FINDINGS: In antineutrophil cytoplasmic antibody-associated vasculitis patients antineutrophil cytoplasmic antibody type may be useful as a predictor of relapse and response to rituximab...
January 2018: Current Opinion in Rheumatology
Firas Ghanem, Deepthi Vodnala, Jagadeesh K Kalavakunta, Sridevi Durga, Noah Thormeier, Prem Subramaniyam, Scott Abela, George S Abela
Cholesterol crystal embolic (CCE) syndrome is often a clinically challenging condition that has a poor prognostic implication. It is a result of plaque rupture with release of cholesterol crystals into the circulation that embolize into various tissue organs. Plaque rupture seems to be triggered by an expanding necrotic core during cholesterol crystallization forming sharp tipped crystals that perforate and tear the fibrous cap. Embolizing cholesterol crystals then initiate both local and systemic inflammation that eventually lead to vascular fibrosis and obstruction causing symptoms that can mimic other vasculitic conditions...
January 19, 2017: Journal of Biomedical Research
Alexander K C Leung, Alex H C Wong, Stefani S N Barg
Although proteinuria is usually benign in the form of transient or orthostatic proteinuria, persistent proteinuria may be associated with more serious renal diseases. Proteinuria may be an independent risk factor for the progression of chronic kidney disease in children. Mechanisms of proteinuria can be categorized as glomerular, tubular, secretory, or overflow. A history, a physical examination, and laboratory tests help determine the cause. Transient (functional) proteinuria is temporary. It can occur with fever, exercise, stress, or cold exposure, and it resolves when the inciting factor is removed...
February 15, 2017: American Family Physician
Sophia Lionaki, John N Boletis
BACKGROUND: Pauci-immune glomerulonephritis is the most common cause of aggressive glomerulonephritis and occurs as a renal-limited disease or as a component of systemic necrotizing small-vessel vasculitis. It is characterized by paucity of staining for immunoglobulins, by immunofluorescence along with fibrinoid necrosis and crescent formation by light microscopy, while the vast majority of patients have anti-neutrophil cytoplasmic antibodies (ANCA) in their circulation, which also participate in the pathogenesis of the disease...
March 2016: Kidney Diseases
Troels Ring, Birgitte Bang Pedersen, Giedrius Salkus, Timothy H J Goodship
IgA nephropathy (IgAN) is characterized by a variable clinical course and multifaceted pathophysiology. There is substantial evidence to suggest that complement activation plays a pivotal role in the pathogenesis of the disease. Therefore, complement inhibition using the humanized anti-C5 monoclonal antibody eculizumab could be a rational treatment. We report here a 16-year-old male with the vasculitic form of IgAN who failed to respond to aggressive conventional therapy including high-dose steroids, cyclophosphamide and plasma exchange and who was treated with four weekly doses of 900 mg eculizumab followed by a single dose of 1200 mg...
October 2015: Clinical Kidney Journal
Wei Zheng, Roscoe Warner, Roger Ruggeri, Chunyan Su, Christian Cortes, Athanasia Skoura, Jessica Ward, Kay Ahn, Amit Kalgutkar, Dexue Sun, Tristan S Maurer, Paul D Bonin, Carlin Okerberg, Walter Bobrowski, Thomas Kawabe, Yanwei Zhang, Timothy Coskran, Sammy Bell, Bhupesh Kapoor, Kent Johnson, Leonard Buckbinder
Small vessel vasculitis is a life-threatening condition and patients typically present with renal and pulmonary injury. Disease pathogenesis is associated with neutrophil accumulation, activation, and oxidative damage, the latter being driven in large part by myeloperoxidase (MPO), which generates hypochlorous acid among other oxidants. MPO has been associated with vasculitis, disseminated vascular inflammation typically involving pulmonary and renal microvasculature and often resulting in critical consequences...
May 2015: Journal of Pharmacology and Experimental Therapeutics
Debbie L Cohen, Michael C Soulen
This case presented challenging diagnostic and management issues in a young healthy man who presented with abdominal pain and new-onset hypertension. The differential diagnosis evolved over the course of the clinical presentation. The patient had severe vascular involvement of his renal and basal cerebral arteries that initially was assumed to be due to a vasculitic process or hypercoagulable state. Finally it became apparent that the patient did not have a systemic illness but rather a localized vascular disease most likely due to segmental arterial mediolysis, a rare, under-recognized condition that can potentially be fatal...
April 7, 2015: Clinical Journal of the American Society of Nephrology: CJASN
Shahid Ilyas, Sumera Salim
Henoch-Schönlein purpura (HSP) is a systemic, small vessel vasculitic disorder that mainly affects joint, skin, gastrointestinal tract and kidneys. It is primarily a disease of children that is typically self-limited, but 10 percent of cases occur in adults where features and outcomes may vary. The underlying pathogenesis of HSP remains unknown. We report a case of HSP that occurred with the onset of acute bacterial endocarditis (ABE) in an otherwise healthy 37-year-old Native American male. The patient presented with fevers, fatigue, abdominal pain and renal failure and was found to have acute left-sided staphylococcal endocarditis...
December 2014: South Dakota Medicine: the Journal of the South Dakota State Medical Association
J M Pego-Reigosa, Í Rúa-Figueroa, F J López-Longo, M Galindo-Izquierdo, J Calvo-Alén, A Olivé-Marqués, V del Campo, M J García-Yébenes, E Loza-Santamaría, R Blanco, R Melero-González, P Vela-Casasempere, T Otón-Sánchez, E Tomero-Muriel, E Uriarte-Isacelaya, M C Fito-Manteca, M Freire-González, J Narváez, A Fernández-Nebro, A Zea-Mendoza, J Rosas, J Carlos Rosas
OBJECTIVES: The objectives of this paper are to study the impact of disease activity in a large cohort of patients with systemic lupus erythematosus (SLE) and estimate the rate of response to therapies. METHODS: We conducted a nationwide, retrospective, multicenter, cross-sectional cohort study of 3658 SLE patients. Data on demographics, disease characteristics: activity (SELENA-SLEDAI), damage, severity, hospitalizations and therapies were collected. Factors associated with refractory disease were identified by logistic regression...
June 2015: Lupus
Charat Thongprayoon, Wisit Cheungpasitporn, Natanong Thamcharoen, Jackrapong Bruminhent
CONTEXT: Henoch-Schönlein purpura (HSP), a systemic IgA vascultitis, is uncommon in adults, with an incidence rate of 0.1 to 1.2 per million in adults over 20 years old. This vasculitic syndrome can present as an uncommon cause of intestinal obstruction in older patients. We report a case of an older woman with HSP presenting with small bowel obstruction and vasculitic rash. CASE REPORT: We report a 67-year-old woman who presented with small bowel obstruction and skin rash...
October 2014: North American Journal of Medical Sciences
Carolyn E Amery, Lui G Forni
PURPOSE OF REVIEW: Acute kidney injury (AKI) is commonplace in most ICUs. In many cases the cause is believed to be multifactorial with sepsis being a major component. However, occasionally intrinsic renal disease will present to the ICU and as such critical care practitioners should be aware of this possibility and the ways in which such conditions may present. RECENT FINDINGS: Although a relatively rare occurrence the treatment for patients with intrinsic renal disease, particularly those who present as part of a vasculitic process, differs considerably from usual organ support employed on intensive care...
December 2014: Current Opinion in Critical Care
Gonzalo De Luna, Benjamin Terrier, Pierre Kaminsky, Alain Le Quellec, François Maurier, Roser Solans, Pascal Godmer, Nathalie Costedoat-Chalumeau, Raphaèle Seror, Pierre Charles, Pascal Cohen, Xavier Puéchal, Luc Mouthon, Loic Guillevin
OBJECTIVE: The aim of this study was to describe the presentation and outcomes of patients with granulomatosis with polyangiitis (GPA) presenting with CNS involvement. METHODS: Patients were included in this nationwide retrospective study if they had GPA according to ACR criteria and/or the European Medicines Agency algorithm and CNS involvement. RESULTS: Thirty-five patients were included in the study. CNS involvement was observed in 51% of patients at GPA diagnosis...
March 2015: Rheumatology
Abdullah Ozkok, Alaattin Yildiz
Hepatitis C virus (HCV) infection is a systemic disorder which is often associated with a number of extrahepatic manifestations including glomerulopathies. Patients with HCV infection were found to have a higher risk of end-stage renal disease. HCV positivity has also been linked to lower graft and patient survivals after kidney transplantation. Various histological types of renal diseases are reported in association with HCV infection including membranoproliferative glomerulonephritis (MPGN), membranous nephropathy, focal segmental glomerulosclerosis, fibrillary glomerulonephritis, immunotactoid glomerulopathy, IgA nephropathy, renal thrombotic microangiopathy, vasculitic renal involvement and interstitial nephritis...
June 28, 2014: World Journal of Gastroenterology: WJG
Sanjeev Sethi, Richard J Glassock, Fernando C Fervenza
Focal and segmental glomerulosclerosis (FSGS) is a common histopathological lesion that can represent a primary podocytopathy, or occur as an adaptive phenomenon consequent to nephron mass reduction, a scar from a healing vasculitic lesion, direct drug toxicity or viral infection among other secondary causes. Thus, the presence of an FSGS lesion in a renal biopsy does not confer a disease diagnosis, but rather represents the beginning of an exploratory process, hopefully leading ultimately to identification of a specific etiology and its appropriate treatment...
March 2015: Nephrology, Dialysis, Transplantation
Sarah Moran, Mark A Little
PURPOSE OF REVIEW: This review aims to provide a state-of-the-art perspective on the role of kidney transplantation in cases of end-stage kidney disease due to antineutrophil cytoplasmic antibody (ANCA) vasculitis. We focus on patient and graft survival in recent years, timing of transplant, impact of ANCA status, and relapse of vasculitis in the allograft. RECENT FINDINGS: Graft and patient outcome compare very favorably with other causes of kidney failure and several recent studies have indicated that these outcomes have improved further in recent years...
January 2014: Current Opinion in Rheumatology
W Chebbi, B Zantour, W Alaya, H Belhadjali, M H Sfar
INTRODUCTION: Vasculitis associated to antineutrophil cytoplasmic antibodies is a rare complication of therapy with antithyroid medication. They were mainly reported in patients treated with propylthiouracil and rarely with benzylthiouracil. CASE REPORT: We report a 22-year-old woman treated with benzylthiouracil for Graves' disease, who developed a vasculitic skin involvement. The presence of antineutrophil cytoplasmic antibodies with anti-myeloperoxidase specificity was documented...
September 2013: La Revue de Médecine Interne
Amy G Rapsang, Prithwis Bhattacharyya
Scrub typhus is an acute febrile illness caused by orientia tsutsugamushi, transmitted to humans by the bite of the larva of trombiculid mites. It causes a disseminated vasculitic and perivascular inflammatory lesions resulting in significant vascular leakage and end-organ injury. It affects people of all ages and even though scrub typhus in pregnancy is uncommon, it is associated with increased foetal loss, preterm delivery, and small for gestational age infants. After an incubation period of 6-21 days, onset is characterized by fever, headache, myalgia, cough, and gastrointestinal symptoms...
March 2013: Indian Journal of Anaesthesia
Michelle A Petri, Ronald F van Vollenhoven, Jill Buyon, Roger A Levy, Sandra V Navarra, Ricard Cervera, Z John Zhong, William W Freimuth
OBJECTIVE: To identify predictors of moderate-to-severe systemic lupus erythematosus (SLE) flare in 562 patients treated with standard therapy alone in phase III belimumab trials, and to evaluate the impact of standard therapies on preventing flares. METHODS: Post hoc analysis assessed baseline demographics, disease activity, and biomarkers in patients with and those without flare at treatment weeks 24 and 52. Severe flare was defined by the modified SLE Flare Index (SFI) and the development of any new British Isles Lupus Assessment Group (BILAG) A domain score...
August 2013: Arthritis and Rheumatism
Justin Keasberry, Jeremy Frazier, Nicole M Isbel, Carolyn L Van Eps, Kimberley Oliver, David W Mudge
UNLABELLED: HASH(0x473d520) INTRODUCTION: Anti-neutrophil cytoplasmic antibody-associated vasculitis has been associated with many drugs and it is a relatively rare side effect of the antihypertensive drug hydralazine. The diagnosis and management of patients who have anti-neutrophil cytoplasmic antibody-associated vasculitis may be challenging because of its relative infrequency, variability of clinical expression and changing nomenclature. The spectrum of anti-neutrophil cytoplasmic antibody-associated vasculitis is wide and can be fatal...
January 14, 2013: Journal of Medical Case Reports
Manmohan Mehndiratta, Sanjay Pandey, Rajeev Nayak, Ravindra K Saran
Hepatitis B can have varied extrahepatic manifestations involving the skin, renal, haematological and nervous systems. Neurological manifestations in hepatitis B may take the form of Guillain-Barré syndrome and secondary systemic vasculitis-related mononeuritis multiplex. The clinical course of hepatitis B-related, vasculitis-related neuropathy is usually subacute to chronic and clinical evolution is relatively benign. To our knowledge, acute hepatitis B-associated vasculitis manifesting as acute distal symmetric polyneuropathy has not been reported...
February 2013: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
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