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pauci-immune crescentic glomerulonephritis

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
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
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
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
Yinghua Chen, Yuemei Ding, Zhengzhao Liu, Haitao Zhang, Zhihong Liu, Weixin Hu
BACKGROUND: The clinic-pathological features and outcomes of Chinese patients with antineutrophil cytoplasmic autoantibody (ANCA)-positive eosinophilic granulomatosis with polyangiitis (EGPA) and renal involvement have not been studied. METHODS: Fourteen EGPA patients with renal involvement were included. All patients underwent renal biopsy. Clinic-pathological features and outcomes were retrospectively analyzed. RESULTS: The most common initial symptom of EGPA was asthma (57...
July 26, 2016: BMC Nephrology
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
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
Rohit Tewari, Sonia Badwal, Arun Kumar, Shankar Subramaniam, V S Nijhawan, V Srinivas
Renal Involvement in scleroderma is a known problem and the manifestations are well described. Renal involvement in systemic lupus erythematosus (SLE) is also well known. However, in scleroderma and SLE overlap syndrome, the renal findings may vary being a combination of features of immune complex mediated glomerulonephritis as well as thrombotic microangiopathy. We report a case in which the renal manifestation in such a situation was of a focal necrotising pauci-immune glomerulonephritis with crescents, anti-neutrophil cytoplasmic antibody negative...
May 2016: Saudi Journal of Kidney Diseases and Transplantation
R Nada, A Kumar, V G Kumar, K L Gupta, K Joshi
Renal biopsy interpretation requires histopathology, direct immunofluorescence (DIF) and electron microscopy. Formalin-fixed, paraffin-embedded tissue (FFPE) sent for light microscopy can be used for DIF after antigen retrieval. However, complement staining has not been satisfactory. We standardized DIF using proteinase-K for antigen retrieval in FFPE renal biopsies. A pilot study was conducted on known cases of membranous glomerulonephritis (MGN), membranoproliferative type-1 (MPGN-1), immunoglobulin A nephropathy (IgAN), and anti-glomerular basement disease (anti-GBM)...
May 2016: Indian Journal of Nephrology
Tamer Rezk, James Penton, Anna Stevenson, Mared Owen-Casey, Mark Little, John Cunningham, Alan D Salama
BACKGROUND: Silver is a transition metal, toxic when ingested in significant amounts, causing argyria (skin deposition) and argyrosis (eye deposition). It is excreted mainly via the gastrointestinal tract with only small amounts eliminated by the kidneys, and rarely have cases of nephrotoxicity due to silver been reported. Here we present the case of a woman who used colloidal silver as an alternative remedy for a T cell lymphoma, who subsequently developed argyria and a pauci-immune crescentic glomerulonephritis with evidence of extensive glomerular basement membrane silver deposition...
2016: BMC Nephrology
In Hee Lee, Gun Woo Kang, Kyung Chan Kim
Granulomatosis with polyangiitis (GPA), an autoimmune disease characterized by inflammatory granulomas and necrotizing small-vessel vasculitis, primarily affects the respiratory tract and kidneys. Azathioprine (AZA) is a purine analog that is commonly used for maintaining GPA remission after induction therapy with cyclophosphamide. While the dose-dependent side effects of AZA are common and well known, hypersensitivity reactions such as pulmonary toxicity are rare. Here, we describe a case involving a 38-year-old man with GPA-associated pauci-immune crescentic glomerulonephritis who developed subacute hypersensitivity pneumonitis (HP) during AZA maintenance therapy...
July 2016: Rheumatology International
F V Veronese, R S O Dode, M Friderichs, G G Thomé, D R da Silva, P G Schaefer, V C Sebben, A R Nicolella, E J G Barros
Levamisole has been increasingly used as an adulterant of cocaine in recent years, emerging as a public health challenge worldwide. Levamisole-associated toxicity manifests clinically as a systemic vasculitis, consisting of cutaneous, hematological, and renal lesions, among others. Purpura retiform, cutaneous necrosis, intravascular thrombosis, neutropenia, and less commonly crescentic nephritis have been described in association with anti-neutrophil cytoplasmic antibodies (ANCAs) and other autoantibodies. Here we report the case of a 49-year-old male who was a chronic cocaine user, and who presented spontaneous weight loss, arthralgia, and 3 weeks before admission purpuric skin lesions in the earlobes and in the anterior thighs...
2016: Brazilian Journal of Medical and Biological Research, Revista Brasileira de Pesquisas Médicas e Biológicas
Irfan Moinuddin, Machaiah Madhrira, Erika Bracamonte, Bijin Thajudeen, Amy Sussman
ANCA-associated vasculitis (AAV) is the most common cause of crescentic rapidly progressive glomerulonephritis (GN). Levamisole used as an adulterant in cocaine is increasingly recognized as a cause of AAV. We report the case of a 50 year old woman with atypical anti-MPO AAV associated with cocaine use and exposure to levamisole. In addition to the clinical and pathologic findings of crescentic GN, the patient also had biopsy evidence of secondary membranous nephropathy (MN). Although AAV and MN have been reported previously in the same patient and both have been induced by drug exposures, this is the first report of MN in a patient with AAV likely induced by levamisole...
July 2016: Pathology, Research and Practice
Camillo Carrara, Stefano Emili, Mercury Lin, Charles E Alpers
Levamisole is an antihelminthic agent widely used as an adulterant of illicit cocaine recently implicated as a cause of antineutrophil cytoplasmic antibody (ANCA)-associated microscopic polyangiitis in cocaine abusers. An isolated case of membranous nephropathy (MN) associated with levamisole exposure has also been reported. We report the first case, to our knowledge, of a patient with both microscopic polyangiitis manifest as a pauci-immune necrotizing and crescentic glomerulonephritis and concurrent MN in the setting of chronic cocaine abuse and presumed levamisole exposure, raising the hypothesis that levamisole was the causative agent in the development of this rare dual glomerulopathy...
April 2016: Clinical Kidney Journal
Francisco Diaz-Crespo, Javier Villacorta, Mercedes Acevedo, Teresa Cavero, Carmen Guerrero, Eugenio García Díaz, Juan Luis Orradre, Miguel Angel Martinez, Manuel Praga, Gema Fernandez-Juarez
The histopathologic classification of antineutrophil cytoplasmic antibody-associated vasculitis has been demonstrated to have prognostic value in small cohorts of patients with pauci-immune extracapillary glomerulonephritis. We aimed to validate this histologic subgrouping system in a large cohort of patients with renal vasculitis from 3 Spanish centers. The additional value of several histologic parameters for predicting renal outcome was investigated. A total of 151 biopsies of patients with renal vasculitis were reviewed and classified as follows: 41% crescentic, 24% mixed, 21% focal, and 14% sclerotic...
June 2016: Human Pathology
A Scemla, C Charlier, L-H Noel, K Amazzough, F T Von Rosen, P Lesavre, O Lortholary
No abstract text is available yet for this article.
May 2016: Médecine et Maladies Infectieuses
Xiang-Yang Li, Ying-Shan Liang, Pearl Pai
OBJECTIVES: The kidneys are frequently involved in antineutrophil cytoplasmic autoantibody (ANCA) associated small-vessel vasculitis (AASVV). The pathological hallmark of ANCA-associated glomerulonephritis (AAGN) is a pauci-immune necrotising crescentic glomerulonephritis. The histopathology of AAGN may change during the course of the disease as a consequence of immunosuppressive therapy. Herein, we report the pathological evolution of a case of AAGN. METHODS: We report a female presented with renal-limited AASVV, hypocomplementemia and nephrotic syndrome...
May 2016: Clinical and Experimental Rheumatology
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