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Vincenzo L'Imperio, Elena Ajello, Federico Pieruzzi, Manuela Nebuloni, Antonella Tosoni, Franco Ferrario, Fabio Pagni
Anti-glomerular basement membrane (GBM) antibody disease is a rare pathological condition that mainly involves renal and/or pulmonary parenchyma. It is characterized by the presence of circulating anti-GBM antibodies accompanied by a linear deposition of immunoglobulins (Ig) detected through immunofluorescence (IF) technique and typical signs and symptoms of organ dysfunction, such as rapidly progressive glomerulonephritis (RPGN) and pulmonary hemorrhage (PH). However, recently atypical forms of anti-GBM disease have been described and the presence of overlapping diseases contributed to make its diagnosis challenging...
April 5, 2017: Journal of Nephrology
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
Mi Hwa Heo, Hee Kyung Kim, Hansang Lee, Myung-Ju Ahn
No abstract text is available yet for this article.
March 22, 2017: Journal of Thoracic Oncology
Kimihiko Goto, Kentaro Nakai, Hideki Fujii, Shinichi Nishi
Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a life-threatening disease characterized by rapidly progressive glomerulonephritis (RPGN) and diffuse alveolar hemorrhage (DAH). Glucocorticoids and immunosuppressants are commonly used to treat this disease but may induce irreversible side effects, particularly in elderly patients. We herein report the case of a 76-year-old woman with RPGN. After methylprednisolone pulse therapy, DAH occurred, and she required ventilatory support. After plasma exchange, her serum creatinine level improved, and she was discharged with home oxygen therapy...
2017: Internal Medicine
Erasmia Sabani, Pantelis A Sarafidis, Antonios Lazaridis, Theodora Kouloukourgiotou, Konstantinos Stylianou, Afroditi Pantzaki, Aikaterini Papagianni, Georgios Efstratiadis
We report a case of a 51-year-old Caucasian man referred at our department due to acute renal failure (ARF) complicating respiratory failure during hospitalization in a regional hospital. The patient was previously started on steroids due to the suspicion of rapidly progressive glomerulonephritis (RPGN) in the context of Goodpasture syndrome. However, clinical and laboratory findings did not support this diagnosis; instead a careful evaluation limited differential diagnosis of the renal insult to acute tubular necrosis or acute interstitial nephritis (AIN) following respiratory infection...
2016: Case Reports in Nephrology
Shilpa Gadde, Belinda Lee, Laura Kidd, Rubin Zhang
Antineutrophil cytoplasmic antibodies (ANCA) are well known to be associated with several types of vasculitis, including pauci-immune crescentic glomerulonephritis, a form of rapid progressive glomerular nephritis (RPGN). ANCA vasculitis has also been reported after administration of propylthiouracil, hydralazine, cocaine (adulterated with levimasole), allopurinol, penicillamine and few other drugs. All previously reported cases of drug-associated ANCA glomerulonephritis were in native kidneys. Sofosbuvir is a new and effective drug for hepatitis C virus infection...
November 6, 2016: World Journal of Nephrology
Mandy Sowa, Barbara Trezzi, Rico Hiemann, Peter Schierack, Kai Grossmann, Juliane Scholz, Valentina Somma, Renato Alberto Sinico, Dirk Roggenbuck, Antonella Radice
Rapidly progressive glomerulonephritis (RPGN) is mainly caused by anti-glomerular basement membrane (GBM) antibody-mediated glomerulonephritis, immune-complex or anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides and leads to rapid loss of renal function. Detection of ANCA and autoantibodies (autoAbs) to GBM and dsDNA enables early diagnosis and appropriate treatment of RPGN aiding in preventing end-stage renal disease.Determination of ANCA on neutrophils (ANCA) as well as autoAbs to myeloperoxidase (MPO-ANCA), proteinase 3 (PR3-ANCA), GBM, and dsDNA was performed by the novel multiplex CytoBead technology combining cell- and microbead-based autoAb analyses by automated indirect immunofluorescence (IIF)...
November 2016: Medicine (Baltimore)
Shamsa Shariatpanahi, Shahryar Pourfarzam, Mohammadhosein Gheini
Macrophage Activating Syndrome (MAS) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, Still's disease, polyarteritis nodosa. It is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. A 27 yr. old female patient was admitted in shahid Mostafa Khomeini Hospital (Tehran-Iran) in May 2011 because of lower extremities edema and ascites and fever from 1.5 month ago. In physical examinations she had generalized lymphadenopathy, splenomegaly and pleural effusion...
2016: Iranian Journal of Pathology
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
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
M O Faroque, K M Hadiuzzaman, S F Islam, R M Hossain, M N Islam, A H Ahmed, P I Ahmed, M R Alam
Over a period of 3 years (January 2011 to December 2013) 100 cases of Lupus nephritis patients admitted in nephrology department of Bangabandhu Sheikh Mujib Medical University (BSMMU) were evaluated. Their clinical characteristics, biochemical parameters, renal histology according to WHO classification were categorized and their treatment modalities and outcome was observed. Among 100 patients, 84 were female and 16 were male, with F:M ratio 5:1. Mean age of female were 23±4 years and male were 29±4 years, mean BP in male was systolic 135±8 mmHg, diastolic 80±9mmHg and in female systolic was 130±7mmHg, diastolic 75±6 mmHg, mean Serum Creatinine for male was 180±12μmol/L and mean serum creatinine in female was 170±20μmol/L...
April 2016: Mymensingh Medical Journal: MMJ
Pui Shan Julia Chan, Moon Ho Leung
We report a case of 63-year-old Chinese man, having a history of anti-myeloperoxidase (MPO) antibody anti-neutrophil cytoplasmic antibody (ANCA)-associated pulmonary-renal syndrome 9 years ago, presented with second episode of rapidly progressive glomerulonephritis (RPGN) and alveolar haemorrhage compatible with anti-glomerular basement membrane (GBM) disease. In first presentation, his anti-GBM antibody was negative. This time, anti-MPO antibody was negative, but anti-GBM antibody was positive. The long interval of sequential development of anti-GBM disease after ANCA-associated vasculitis in this patient may provide clues to the potential immunological links between these two distinct conditions...
April 2016: Oxford Medical Case Reports
Ken-Ei Sada, Masayoshi Harigai, Koichi Amano, Tatsuya Atsumi, Shouichi Fujimoto, Yukio Yuzawa, Yoshinari Takasaki, Shogo Banno, Takahiko Sugihara, Masaki Kobayashi, Joichi Usui, Kunihiro Yamagata, Sakae Homma, Hiroaki Dobashi, Naotake Tsuboi, Akihiro Ishizu, Hitoshi Sugiyama, Yasunori Okada, Yoshihiro Arimura, Seiichi Matsuo, Hirofumi Makino
OBJECTIVE: To compare disease severity classification systems for six-month outcome prediction in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: Patients with newly diagnosed AAV from 53 tertiary institutions were enrolled. Six-month remission, overall survival, and end-stage renal disease (ESRD)-free survival were evaluated. RESULTS: According to the European Vasculitis Study Group (EUVAS)-defined disease severity, the 321 enrolled patients were classified as follows: 14, localized; 71, early systemic; 170, generalized; and 66, severe disease...
September 2016: Modern Rheumatology
Wisit Cheungpasitporn, Claudia C Zacharek, Fernando C Fervenza, Lynn D Cornell, Sanjeev Sethi, Loren P Herrera Hernandez, Samih H Nasr, Mariam P Alexander
Anti-glomerular basement membrane (anti-GBM) disease is a major cause of rapidly progressive glomerulonephritis (RPGN). On the other hand, fibrillary glomerulonephritis (GN) typically presents as proteinuria, hematuria and renal insufficiency, but rarely as RPGN. Without electron microscopy, the diagnosis of fibrillary GN can be missed. We report a 68-year-old white woman who presented with RPGN with kidney biopsy demonstrating diffuse crescentic GN on light microscopy. By immunofluorescence, there was bright linear staining of the GBMs and smudgy mesangial staining for immunoglobulin G, C3, and kappa and lambda light chain...
February 2016: Clinical Kidney Journal
Soumita Bagchi, Parmod Mittal, Geetika Singh, Sanjay Kumar Agarwal, Lavleen Singh, Dipankar Bhowmik, Sandeep Mahajan, Amit Dinda
BACKGROUND: An aging population is an important demographic issue in India. The knowledge base about kidney diseases among the elderly Indians is inadequate. We aim to delineate the clinical profile and spectrum of biopsy-proven kidney disease in elderly patients. METHODS: Records of all elderly patients (≥60 years) who had undergone kidney biopsy in the nephrology department from January 2010 to December 2014 were reviewed. Their clinical details and laboratory investigations at the time of biopsy were noted...
April 2016: International Urology and Nephrology
Dahai Wang, Fang Wang, Jie Ding, Huijie Xiao, Xuhui Zhong, Xiaoyu Liu
OBJECTIVE: IgA nephropathy is the most common type of glomerulonephritis in the world. Its clinical and pathological manifestations vary. A few of the patients with IgA nephropathy present with rapidly progressive glomerulonephritis (RPGN) and/or crescent formation. Their conditions are serious and acute, but there are few reports on their characteristics, treatment and outcome. This study aimed to analyze the clinicalopathological features, treatment and prognosis of primary IgA nephropathy in children, to provide a reference for clinical diagnosis and treatment...
September 2015: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Rafeel Syed, Amina Rehman, Gautam Valecha, Suzanne El-Sayegh
Rapidly progressive glomerulonephritis (RPGN) is a syndrome signified by a precipitous loss of renal function, with features of glomerulonephritis including dysmorphic erythrocyturia and glomerular proteinuria. RPGN is associated with extensive crescent formation, and, thus, the clinical term RPGN is often used interchangeably with the pathologic term crescentic glomerulonephritis (CGN). From an immunopathologic standpoint, primary RPGN is divided into pauci-immune GN (PICG), anti-GBM GN, and immune complex GN...
2015: BioMed Research International
Jimmy A Thomas, Dmitri Vasin, Mercury Lin, Arthur E Anderson, Charles E Alpers
Fibrillary glomerulonephritis (FGN) is a rare cause of rapidly progressive glomerulonephritis (RPGN). We report a case of FGN in which the patient presented with a clinical pulmonary-renal syndrome and whose kidney biopsy showed > 90% crescents on light microscopy. Immunofluorescence microscopy showed pseudo-linear IgG and C3 staining of the glomerular capillary walls resulting in an initial diagnosis of crescentic glomerulonephritis of anti-glomerular basement membrane (anti-GBM) antibody etiology. Electron microscopy showed fibrillary deposits permeating the glomerular capillary walls, characteristic of FGN...
February 2016: Clinical Nephrology
Ya-ting Ge, Jin-lan Liao, Wei Liang, Zu-ying Xiong
BACKGROUND: Anti-glomerular basement membrane disease (anti-GBM disease) is an autoimmune glomerulonephritis disease that is characterized by IgG linear deposition along the non-collagen domain of a3 chains of type IV collagen on the GBM. Although anti-GBM disease accompanied with IgA linear deposition along GBMs was discussed previously in some papers, anti-GBM disease combined with IgA granular deposition in the mesangial area, especially complicated with reversible posterior leukoencephalopathy syndrome (RPLS), was rarely reported...
2015: American Journal of Case Reports
Kiyoki Kitagawa, Kengo Furuichi, Akihiro Sagara, Yasuyuki Shinozaki, Shinji Kitajima, Tadashi Toyama, Akinori Hara, Yasunori Iwata, Norihiko Sakai, Miho Shimizu, Shuichi Kaneko, Takashi Wada
BACKGROUND: The prevention of relapse and infection complications during remission maintenance therapy is required to improve the prognosis of patients with microscopic polyangiitis (MPA) showing rapidly progressive glomerulonephritis (RPGN). The clinicopathological characteristics of patients with ANCA-positive MPA were examined to determine the risk factors for relapse or infectious complications after remission induction therapy. PATIENTS AND METHODS: The study population consisted of 52 patients diagnosed as ANCA-positive MPA showing RPGN from 2002 to 2012, after publication of the Japanese guideline for RPGN...
October 2016: Clinical and Experimental Nephrology
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