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"Crescentic glomerulonephritis"

Oleg Palygin, Daria V Ilatovskaya, Alexander Staruschenko
Protease-activated receptors (PARs) are a well-known family of transmembrane G-protein-coupled receptors (GPCRs). To date, four PARs have been identified and PAR1 and PAR2 are the most abundant receptors, which were shown to be expressed in the kidney vascular and tubular cells. PAR signaling is mediated by an N-terminus tethered ligands that can be unmasked by serine protease cleavage. PARs are activated by endogenous serine proteases, such as thrombin (acts on PARs 1, 3 and 4) and trypsin (PAR2). PARs can be involved in glomerular, microvascular and inflammatory regulation of renal function in both normal and pathological conditions...
October 12, 2016: American Journal of Physiology. Renal Physiology
Turki Al Hussain, Hadeel Al Mana, Maged H Hussein, Mohammed Akhtar
The glomerulus has 3 resident cells namely mesangial cells that produce the mesangial matrix, endothelial cells that line the glomerular capillaries, and podocytes that cover the outer surface of the glomerular basement membrane. Parietal epithelial cells (PrECs), which line the Bowman's capsule are not part of the glomerular tuft but may have an important role in the normal function of the glomerulus. A significant progress has been made in recent years regarding our understanding of the role and function of these cells in normal kidney and in kidneys with various types of glomerulopathy...
September 26, 2016: Advances in Anatomic Pathology
Huifang Liu, Jiachuan Xiong, Jun Zhang, Ying Zhang, Ling Nie, Yiqin Wang, Jinghong Zhao
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a life-threatening condition that causes renal failure. Multiple myeloma (MM) is a malignant proliferation of monoclonal plasma cells in the blood that can also cause renal failure. The two diseases have high morbidity and mortality rates in the elderly, with a poor prognosis. A 64-year-old female presented to Xinqiao Hospital (Chonqing, China) with fatigue and a poor appetite that had been apparent for 6 weeks. Laboratory tests revealed a serum creatinine level of 10...
September 2016: Oncology Letters
Shuichi Asano, Shige Mizuno, Shotaro Okachi, Hiromichi Aso, Keiko Wakahara, Naozumi Hashimoto, Satoru Ito, Yohei Kozaki, Takayuki Katsuno, Shoichi Maruyama, Yoshinori Hasegawa
A 73-year-old woman was diagnosed with pulmonary Mycobacterium avium complex (MAC) infection and received no treatment. Disease progression was evident one year later with the development of myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA) titers and systemic symptoms of a fever, polyarthritis, purpura, and rapidly progressive glomerulonephritis. Her symptoms did not improve with antibiotic treatment. A renal biopsy revealed crescentic glomerulonephritis with immunodeposition. According to these findings, she was diagnosed with ANCA-associated vasculitis (AAV) superimposed on infection-related glomerulonephritis (IRGN)...
2016: Internal Medicine
Nuntawan Piyaphanee, Chompoonut Ananboontarick, Suroj Supavekin, Achra Sumboonnanonda
BACKGROUND: Rapidly progressive glomerulonephritis (RPGN), a clinical diagnosis defined as acute nephritic syndrome with dramatic loss of renal function within few months, is associated with crescentic glomerulonephritis (CresGN), which requires ≥50% crescents in pathology. The percentage of crescents in children with RPGN may vary, however, determining disease characteristics and renal outcome. METHODS: To evaluate the renal outcomes and factors associated to end-stage renal disease (ESRD), this retrospective cohort study assessed children aged ≤15 years with RPGN at a tertiary medical center...
August 20, 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Hong Xiao, Peiqi Hu, Ronald J Falk, J Charles Jennette
BACKGROUND: Antineutrophil cytoplasmic autoantibodies (ANCA) are associated with a spectrum of necrotizing vasculitis including granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited necrotizing and crescentic glomerulonephritis. Clinical observations and in vitro and in vivo experimental evidence strongly indicate that ANCA are pathogenic. SUMMARY: The etiology and pathogenesis of ANCA-associated vasculitis (AAV) are multifactorial, with contributions from genetic factors, environmental exposures, infections, characteristics of the innate and adaptive immune system, and the intensity and duration of the injury...
March 2016: Kidney Diseases
Duriye Deren Oygar, Yonca Morris
Behçet's disease (BD) is a multisystem disorder of unknown etiology. Renal involvement in Behçet's disease is not frequent. Pauciimmune necrotizing and crescentic glomerulonephritis is associated with antineutrophil cytoplasmic antibodies (ANCA) which are believed to activate neutrophils and cause vasculitis, especially if there is a concurrent synergistic inflammatory process. In this case report, we describe a patient diagnosed with BD who also developed pauciimmune necrotizing and crescentic glomerulonephritis...
2016: Internal Medicine
S K Rampelli, N G Rajesh, B H Srinivas, K T Harichandra Kumar, R P Swaminathan, P S Priyamvada
There is limited data on the etiology, clinical and histopathological spectrum and outcomes of crescentic glomerulonephritis (CrGN) in adult Indian population. This prospective study was done to evaluate the etiology, clinicohistological patterns and predictors of outcome of CrGN in South Indian population. All the patients received standard protocol based immunosuppression in addition to supportive care. Immune-complex glomerulonephritis (ICGN) was the most common etiology (n = 31; 77.5%) followed by pauci-immune glomerulonephritis (PauciGN; n = 8; 20%) and anti-glomerular basement membrane disease (n = 1; 2...
July 2016: Indian Journal of Nephrology
M G Chen, X H Ye, H Y Liang, Q Yang
OBJECTIVE: To investigate the incidence, etiology, clinico- pathological characteristics and prognosis in primary IgA nephropathy (IgAN) children with acute kidney injury (AKI). METHOD: Retrospective analysis of the clinical and pathological manifestations and follow-up results of 19 Chlidren, who were associated with AKI in 196 cases of children with IgA nephropathy treated in our department from January, 1996 to Jun, 2012 was performed. RESULT: (1) The 19 cases associated with AKI accounted for 9...
August 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Lauren E Neidig, Michael A Owston, Erin Ball, Edward J Dick
BACKGROUND: Crescentic glomeruli are the hallmark finding in rapidly progressive glomerulonephritis (RPGN) and are characterized by disruption and proliferation of the glomerular capsule and an influx of cells into Bowman's space. Pauci-immune-type RPGN is identified by a lack of immunoglobulins and immune complexes in the glomerular basement membrane. METHODS: Complete necropsy and histology were performed on the affected chimpanzee. Electron microscopy was performed on kidney sections...
August 10, 2016: Journal of Medical Primatology
Zeyuan Lu, Jianyong Yin, Hongda Bao, Qiong Jiao, Huijuan Wu, Rui Wu, Qin Xue, Niansong Wang, Zhigang Zhang, Feng Wang
INTRODUCTION: IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder that may involve almost each organ or system. IgG4-related kidney disease (IgG4-RKD) refers to renal lesions associated with IgG4-RD. The most frequent morphological type of renal lesions is IgG4-related tubulointerstitial nephritis (IgG4-TIN) which is associated with increased IgG4-positive plasma cell infiltration and interstitial fibrosis. CASE REPORT: Herein, we present a rare case with coexisting IgG4-RKD and acute crescent glomerulonephritis with concomitant severe tubulointerstitial lesions instead of classic IgG4-TIN...
May 2016: Case Reports in Nephrology and Dialysis
Supraja Yeturi, Mary Cronin, Adam Robin, Campbell Lorna, Ann K Rosenthal
Pauci-immune crescentic glomerulonephritis is commonly seen in ANCA-associated vasculitis but it is rarely seen during the course of other connective tissue diseases like lupus or Sjogren's syndrome or MCTD. We report 3 cases of pauci-immune crescentic glomerulonephritis in patients with connective tissue disease other than vasculitis. We reviewed literature and made summary of previously reported cases of this rare entity. Clinical and laboratory features of these patients varied widely, but most of patients have met criteria for lupus...
2016: Case Reports in Rheumatology
Rui Fernandes, Sara Freitas, Pedro Cunha, Gloria Alves, Jorge Cotter
BACKGROUND: Goodpasture's syndrome, a rare disease, is an organ-specific autoimmune disease mediated by anti-glomerular basement membrane antibodies. Its pathology is characterized by crescentic glomerulonephritis with linear immunofluorescent staining for immunoglobulin G on the glomerular basement membrane. Although rare, a few cases with absence of circulating anti-glomerular membrane antibodies have been described. CASE PRESENTATION: The objective of this clinical case report is to describe and discuss a case of a 27-year-old white man who was hospitalized with a 1-year history of weight loss and a 1-month history of hemoptysis, with aggravation  the day before, having developed dyspnea and cough in the previous 24 hours...
2016: Journal of Medical Case Reports
Kamel El-Reshaid, Jozsef Varro, John P Madda
Lymph node involvement as a part of systemic vasculitis is rare. We report a case of women with rapidly progressive renal disease associated with recurrent epigastric pain, weight loss, and massive hilar as well as para-aortic lymphadenopathy. Ultrasound-guided biopsy of her scarred kidneys revealed antineutrophil cytoplasmic antibody-negative crescentic glomerulonephritis and that of lymph nodes showed severe necrotizing vasculitis. Biopsy of the lymph nodes and the failing kidney established the diagnosis of this rare presentation and ruled out lymphoma and tuberculosis...
July 2016: Saudi Journal of Kidney Diseases and Transplantation
In Hee Lee, Seong-Kyu Kim, Min-Kyung Kim
Sjögren's syndrome is an autoimmune disease that primarily affects exocrine glands. Renal involvement of Sjögren's syndrome may lead to tubulointerstitial disease, whereas secondary glomerulopathies such as anti-neutrophil cytoplasmic antibody (ANCA)-related pauci-immune crescentic glomerulonephritis are rarely observed. In addition, crescent glomerulonephritis that is simultaneously positive for both myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA has never been reported in Sjögren's syndrome. Here, we report a case of pauci-immune crescentic glomerulonephritis exhibiting positivity for both MPO- and PR3-ANCAs in a patient with primary Sjögren's syndrome...
September 2016: Rheumatology International
Ryan Kunjal, Raafat Makary, Andreea Poenariu
Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest...
2016: Case Reports in Nephrology
Christian F Krebs, Jan-Eric Turner, Hans-Joachim Paust, Sonja Kapffer, Tobias Koyro, Sonja Krohn, Friederike Ufer, Manuel A Friese, Richard A Flavell, Brigitta Stockinger, Oliver M Steinmetz, Rolf A K Stahl, Samuel Huber, Ulf Panzer
The ability of CD4(+) T cells to differentiate into pathogenic Th1 and Th17 or protective T regulatory cells plays a pivotal role in the pathogenesis of autoimmune diseases. Recent data suggest that CD4(+) T cell subsets display a considerable plasticity. This plasticity seems to be a critical factor for their pathogenicity, but also for the potential transition of pathogenic effector T cells toward a more tolerogenic phenotype. The aim of the current study was to analyze the plasticity of Th17 cells in a mouse model of acute crescentic glomerulonephritis and in a mouse chronic model of lupus nephritis...
July 15, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
Javier Villacorta, Francisco Diaz-Crespo, Mercedes Acevedo, Carmen Guerrero, Yolanda Campos-Martin, Eugenio García-Díaz, Manuela Mollejo, Gema Fernandez-Juarez
Pauci-immune necrotizing crescentic glomerulonephritis is the histologic substrate of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Several studies in animal models have demonstrated the crucial role of complement activation in the pathogenesis of ANCA-associated vasculitis, but only small series have analyzed the prognostic implications of complement glomerular deposits. This study aimed to assess the clinical and prognostic implications of C3d- and C4d-positive glomerular staining in renal vasculitis...
October 2016: Human Pathology
Yi-Wei Justin Liu, Sangeeta Mutnuri, Sarah Batool Siddiqui, Geoff Richard Weikle, Olajumoke Oladipo, Niharika Ganti, Robert E Beach, Marjan Afrouzian
OBJECTIVES: The issue of levamisole-adulterated cocaine is emerging as a rapidly growing public health concern due to an increasing number of reports describing its role in cutaneous vasculitis and agranulocytosis. Of note, levamisole is recognized as a contaminant in 69% of the cocaine used within the United States. METHODS: We describe a patient who was a chronic cocaine user and developed systemic vasculitis characterized by polyarthralgia, bullous skin lesions, agranulocytosis, and antineutrophil cytoplasmic antibody-positive rapidly progressive glomerulonephritis...
May 2016: American Journal of Clinical Pathology
Chi Chiu Mok
Kidney involvement is a major determinant for morbidity and mortality in patients with systemic lupus erythematosus. The treatment target of lupus renal disease is to induce and maintain remission and to minimize disease or treatment-related comorbidities. Cyclophosphamide (CYC), in conjunction with glucocorticoids, has conventionally been used for the initial treatment of lupus nephritis. However, the major concerns of CYC are its toxicities, such as infertility, urotoxicity and oncogenicity, which are particularly relevant in women of childbearing age...
July 2016: Nephrology, Dialysis, Transplantation
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