1,813 results match your criteria Glomerulonephritis Rapidly Progressive


Neurologic manifestations associated with cryoglobulinemia: A single center experience.

J Neurol Sci 2019 Jan 25;398:121-127. Epub 2019 Jan 25.

Department of Neurology, Cleveland Clinic Foundation, Cleveland, OH 44195, United States. Electronic address:

Introduction: Limited information is available describing the spectrum of neurological complications of cryoglobulinemia.

Methods: Single center retrospective review of patients with neurologic symptoms and elevated serum cryoglobulins, with their potential association being classified as definite, possible, or unlikely using defined criteria.

Results: Among 492 patients, 131 (87 classified as definite and 44 as possible) had neurologic symptoms associated with cryoglobulinemia. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S0022510X193005
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http://dx.doi.org/10.1016/j.jns.2019.01.041DOI Listing
January 2019
3 Reads

A case of aggressive atypical anti-GBM disease complicated by CMV pneumonitis.

BMC Nephrol 2019 Jan 31;20(1):29. Epub 2019 Jan 31.

Department of Pathology and Laboratory Medicine, Cumming School of Medicine, University of Calgary, Foothills Medical Center, 1403 29 St NW, Calgary, AB, T2N 2T9, Canada.

Background: Anti-glomerular basement membrane (anti-GBM) disease is characterized by circulating IgG glomerular basement membrane antibodies and is clinically expressed as a rapidly progressive crescentic glomerulonephritis (GN), with 30-60% of patients also developing pulmonary hemorrhage. Classically, the renal biopsy shows glomerular crescent formation, bright linear staining of glomerular basement membranes (GBM) for IgG on direct immunofluorescence (IF), and the serologic presence of circulating anti-GBM antibodies. Recently, patients with linear IgG IF staining, undetectable circulating anti-GBM antibodies and glomerular changes atypical for anti-GBM disease have been described as "atypical anti-GBM disease", with a distinctly more benign clinical course than typical anti-GBM disease. Read More

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http://dx.doi.org/10.1186/s12882-019-1227-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6357502PMC
January 2019
2 Reads

Atypical Goodpasture's disease: a clinical case report and literature review.

Ter Arkh 2018 Jun;90(6):130-136

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Moscow, Russia.

Goodpasture's disease (anti-GBM disease) is a rare small vessels vasculitis characterized by the presence of autoantibodies directed against the glomerular basement membrane (GBM) and alveolar basement membrane. Common feature of anti-GBM disease is a combination of rapidly progressive glomerulonephritis and alveolar hemorrhage (pulmonary-renal syndrome). We present a case of atypical disease course in a young male patient who developed alveolar hemorrhage without renal failure. Read More

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http://dx.doi.org/10.26442/terarkh2018906130-136DOI Listing
June 2018
1 Read

Clinical features of kidney involvement in microscopic microscopic polyangiitis.

Ter Arkh 2018 Jun;90(6):55-58

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Moscow, Russia.

Aim: To evaluate clinical features and outcomes of renal involvement in patients with microscopic polyangiitis (MPA).

Materials And Methods: We enrolled 99 patients with MPA, diagnosed in accordance with the algorithm of the European Medicines Evaluation Agency (EMEA) and the Chapel Hill consensus conference definition (2012). Serum creatinine (sCr), estimated glomerular filtration rate (eGFR), hematuria and proteinuria were estimated. Read More

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http://dx.doi.org/10.26442/terarkh201890655-58DOI Listing
June 2018
1 Read

Relationship between serologic profile (ANCA type) and clinical features of renal involvement in ANCA-associated vasculitides.

Ter Arkh 2018 Jun;90(6):15-21

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Moscow, Russia.

Aim: To compare the frequency, clinical features and outcomes of renal involvement in ANCA-associated vasculitides (AAV) in patients with antibodies against proteinase-3 (pr3-ANCA) and myeloperoxidase (MPO-ANCA).

Materials And Methods: In our retrospective study we enrolled 264 patients, 94 males and 170 females, median age 53 [36; 62] years. Among them 157 were pr3-ANCA positive and 107 were MPO-ANCA positive. Read More

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http://dx.doi.org/10.26442/terarkh201890615-21DOI Listing
June 2018
1 Read

Diagnostic algorithm for antineutrophil cytoplasmic antibody-associated systemic vasculitis.

Authors:
T V Beketova

Ter Arkh 2018 May;90(5):13-22

V.A. Nasonova Scientific Research Institute of Rheumatology, Moscow, Russia.

Antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) are rare autoimmune disorders and characterized by severe multiple organ lesions with a potential fatal outcome. AAV comprises granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Early diagnosis and treatment can significantly improve the AAV prognosis, but there are can be difficult, largely due to the lack of criteria for the classification MPA, whose main difference is the absence of granulomatous inflammation. Read More

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http://dx.doi.org/10.26442/terarkh201890513-22DOI Listing
May 2018
1 Read

Development of anti-glomerular basement membrane glomerulonephritis during the course of IgA nephropathy: a case report.

BMC Nephrol 2019 Jan 25;20(1):25. Epub 2019 Jan 25.

Department of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 193-0998, Japan.

Background: Anti-glomerular basement membrane (GBM) glomerulonephritis does not usually coexist with another glomerulonephritis such as IgA nephropathy. We present a rare case having a combination of these two diseases, and furthermore, histological evaluation could be performed before and after the development of anti-GBM glomerulonephritis over a period of only10 months.

Case Presentation: A 66-year-old woman was admitted with complaints of microscopic hematuria and mild proteinuria for the past 3 years. Read More

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http://dx.doi.org/10.1186/s12882-019-1207-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6347754PMC
January 2019
2 Reads

Leukocytoclastic vasculitis with late-onset Henoch-Schönlein purpura after trifluridine/tipiracil treatment.

Dermatol Online J 2018 Oct 15;24(10). Epub 2018 Oct 15.

Department of Dermatology, The University of Texas McGovern Medical School, Houston, Texas.

Trifluridine/tipiracil has been approved for the treatment of refractory metastatic colorectal cancer. Adverse effects of this drug combination include leukopenia, neutropenia, fatigue, diarrhea, and vomiting. We present a case of trifluridine/tipiracil-induced leukocytoclastic vasculitis (LCV) with late-onset Henoch-Schönlein purpura (HSP) in a 42-year-old man with metastatic appendiceal cancer. Read More

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October 2018
2 Reads

Acute renal failure in a patient with PR3-ANCA and monoclonal immunoglobulin deposition disease: Case report.

Medicine (Baltimore) 2018 Dec;97(52):e13799

Institute of Health Policy, Management, and Evaluation, University of Toronto, Canada.

Rationale: Acute renal failure developing over a short period of time with evidence of glomerular disease by urine sediment microscopy characterizes the clinical syndrome of rapidly progressive glomerulonephritis (RPGN), of which the most common causes are ANCA-associated glomerulonephritis (GN), immune-complex mediated GN and anti-GBM disease.

Patient Concerns: This was a middle-aged gentleman who presented with acute renal failure and a positive PR3-ANCA.

Diagnosis: Renal biopsy showed an unusual combination of PR3-ANCA GN with focal crescents, monoclonal immunoglobulin deposition disease (MIDD) and mesangial IgA deposition on renal biopsy. Read More

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http://Insights.ovid.com/crossref?an=00005792-201812280-0004
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http://dx.doi.org/10.1097/MD.0000000000013799DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314724PMC
December 2018
3 Reads

Cerebral venous sinus thrombosis as a warning signal for double positive crescentic glomerulonephritis in a young male with complete renal recovery.

Saudi J Kidney Dis Transpl 2018 Nov-Dec;29(6):1488-1493

Department of Renal Pathology, Lal Path Lab, New Delhi, India.

Double positive crescentic glomerulonephritis is relatively rare in young population and has variable outcomes. Although increased incidence of deep venous thrombosis in antineutrophil cytoplasmic antibody-associated vasculitis has been reported, cerebral venous sinus thrombosis (CVT) is very rare. We present a young male who presented with CVT followed by rapidly progressive crescentic glomerulonephritis and with appropriate therapeutic modalities he had complete renal and partial neurological recovery. Read More

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http://dx.doi.org/10.4103/1319-2442.248291DOI Listing
December 2018
126 Reads

ANCA-Associated Necrotizing Glomerulonephritis Overlapping with Mesangial Proliferative Lupus Nephritis Refractory to Plasmapheresis, Steroid Pulse Therapy, and a Combination of Mycophenolate Mofetil and Rituximab.

Case Rep Rheumatol 2018 19;2018:3076806. Epub 2018 Nov 19.

Division of Rheumatology, SUNY Upstate Medical University, Syracuse, NY 13210, USA.

Necrotizing glomerulonephritis (GN) associated with antineutrophil cytoplasmic antibody (ANCA) has been increasingly recognized in the context of class III or IV lupus nephritis (LN), hereafter designated as . While this subset of GN appears to portend an unfavorable renal outcome, it is not clear whether it represents a distinct entity and benefits from a more aggressive therapy. We report a 78-year-old woman who presented with rapidly progressive GN and was found to have a double-stranded DNA (dsDNA) antibody, hypocomplementemia, antiphospholipid antibody, and strongly positive myeloperoxidase antibody. Read More

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http://dx.doi.org/10.1155/2018/3076806DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6276453PMC
November 2018
1 Read

Rare heparin induced thrombocytopenia type I reaction in a hemodialysis patient: Case report.

Medicine (Baltimore) 2018 Dec;97(50):e13609

Department of Pharmacy.

Rationale: Heparin-induced thrombocytopenia (HIT) is a common antibody-mediated adverse reaction that occurs after heparin exposure. However, few case reports exist regarding nonantibody-mediated HIT.

Patient Concerns And Diagnoses: An 81-year-old female diagnosed with rapidly progressive glomerulonephritis (RPGN) presented with atypical presentation of non antibody-meditated HIT after using heparin during hemodialysis. Read More

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http://dx.doi.org/10.1097/MD.0000000000013609DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320049PMC
December 2018
1 Read
5.723 Impact Factor

Correlation between Pathological Findings and the Usefulness of Clinical Guidelines for the Treatment of ANCA-Positive RPGN: A Retrospective Analysis.

J Nippon Med Sch 2018 ;85(5):259-264

Department of Nephrology, Nippon Medical School.

Background: The physical condition of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated rapidly progressive glomerulonephritis (RPGN) is at times too critical for physicians to undertake a renal biopsy. In such cases, physicians need to start treatments without determining the pathological features of the disease. However, the prevalent clinical practice guidelines for ANCA-associated RPGN in Japan do not necessitate the pathological findings in a renal biopsy for determining first-line treatments. Read More

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http://dx.doi.org/10.1272/jnms.JNMS.2018_85-41DOI Listing
February 2019
10 Reads

Infection-Related Glomerulonephritis.

Pediatr Clin North Am 2019 02;66(1):59-72

Division of Nephrology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.

Acute postinfectious glomerulonephritis or infection-related glomerulonephritis has been associated with several viral or bacterial infections. Group A beta-hemolytic streptococcal infection is the prototypical cause of postinfectious glomerulonephritis and the main focus of this discussion. The clinical spectrum can vary widely, from asymptomatic microscopic hematuria incidentally detected on routine urinalysis to rapidly progressive glomerulonephritis with acute kidney injury requiring emergent dialysis. Read More

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http://dx.doi.org/10.1016/j.pcl.2018.08.005DOI Listing
February 2019
9 Reads

Hydralazine-associated antineutrophil cytoplasmic antibody vasculitis with pulmonary-renal syndrome.

BMJ Case Rep 2018 Nov 8;2018. Epub 2018 Nov 8.

Nephrology, Deaconess Health System, Evansville, Indiana, USA.

Hydralazine, a vasodilator, is commonly used as an adjunctive treatment for moderate to severe hypertension, heart failure and hypertensive emergencies in pregnancy. Hydralazine-induced lupus was first described in 1953. Clinical presentation ranges from arthralgia, myalgia, petechiae, or rash to single or multiorgan involvement. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22716
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http://dx.doi.org/10.1136/bcr-2018-227161DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6229221PMC
November 2018
7 Reads

A case report of granulomatous polyangiitis complicated by tuberculous lymphadenitis.

Medicine (Baltimore) 2018 Oct;97(43):e12430

Lung Cancer and Respiratory Disease Center, Yamanashi Central Hospital.

Rational: Granulomatous polyangiitis (GPA) is a type of vasculitis involving medium and small arteries, typically affecting the upper and lower respiratory tract with coexisting glomerulonephritis. GPA is also characterized by necrotizing granulomatous inflammation and the presence of antineutrophil cytoplasm antibodies (ANCA). So far, various infections have lead to elevation of titers of serum ANCA, making it difficult to diagnose. Read More

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http://Insights.ovid.com/crossref?an=00005792-201810260-0000
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http://dx.doi.org/10.1097/MD.0000000000012430DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6221664PMC
October 2018
12 Reads

Antiglomerular Basement Membrane Disease.

Semin Respir Crit Care Med 2018 08 7;39(4):494-503. Epub 2018 Nov 7.

Department of Medicine, Centre for Inflammatory Disease, Imperial College London, London, United Kingdom.

Antiglomerular basement membrane (anti-GBM) disease is a rare but life-threatening autoimmune vasculitis that is characterized by the development of pathogenic autoantibodies to type IV collagen antigens expressed in the glomerular and alveolar basement membranes. Once deposited in tissue, these autoantibodies incite a local capillaritis which manifests as rapidly progressive glomerulonephritis (GN) in 80 to 90% of patients, and with concurrent alveolar hemorrhage in ∼50%. A small proportion of cases may present with pulmonary disease in isolation. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0038-1669413
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http://dx.doi.org/10.1055/s-0038-1669413DOI Listing
August 2018
10 Reads

Microscopic Polyangiitis: New Insights into Pathogenesis, Clinical Features and Therapy.

Authors:
Alexandre Karras

Semin Respir Crit Care Med 2018 08 7;39(4):459-464. Epub 2018 Nov 7.

Department of Nephrology, Hôpital Européen Georges Pompidou, Assistance Publique- Hôpitaux de Paris, Paris France.

Microscopic polyangiitis (MPA) is one of the main clinical presentations of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although the disease is defined by clinical and pathological criteria, the anti-myeloperoxidase (MPO) specificity of ANCAs is observed in almost 80% of MPA patients. The direct pathogenic role of anti-MPO antibodies has been proven in animal models, in which the disease was transmitted by transfer of anti-MPO antibodies or anti-MPO-specific splenocytes. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0038-1673387
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http://dx.doi.org/10.1055/s-0038-1673387DOI Listing
August 2018
7 Reads

Temporal change in life and renal prognosis of rapidly progressive glomerulonephritis in Japan via nationwide questionnaire survey.

Clin Exp Nephrol 2018 Oct 31. Epub 2018 Oct 31.

Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.

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http://dx.doi.org/10.1007/s10157-018-1663-2DOI Listing
October 2018
2 Reads

Immune complex-mediated rapidly progressive glomerulonephritis in a patient with family history of systemic lupus erythematosus.

Saudi J Kidney Dis Transpl 2018 Sep-Oct;29(5):1227-1231

Department of Pathology, King Abdulaziz Medical City, Riyadh, Saudi Arabia.

We describe here a case of a 53-year-old Saudi female who presented to the emergency room with shortness of breath progressive in nature for the previous one month, associated with a cough and occasional greenish sputum. She turned out to be a case of immunocomplex mediated glomerulonephritis presenting as rapidly progressive glomerulonephritis in a female patient with a history of systemic lupus erythematosus (SLE) in her daughter. Read More

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http://dx.doi.org/10.4103/1319-2442.243949DOI Listing
November 2018
2 Reads

Analysis of native kidney biopsy: Data from a single center from Bihar, India.

Saudi J Kidney Dis Transpl 2018 Sep-Oct;29(5):1174-1180

Department of Nephrology, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India.

This is a retrospective study of all native kidney biopsies performed at our center between October 1, 2012 and March 31, 2015. Relevant clinical and laboratory variables were recorded. Biopsy samples were processed for light microscopy and immunofluorescence in all cases. Read More

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http://www.sjkdt.org/text.asp?2018/29/5/1174/243975
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http://dx.doi.org/10.4103/1319-2442.243975DOI Listing
November 2018
4 Reads

Anti-glomerular basement membrane disease: an update on subgroups, pathogenesis and therapies.

Nephrol Dial Transplant 2018 Oct 29. Epub 2018 Oct 29.

Lund University, Skane University Hospital, Department of Clinical Sciences Lund, Nephrology, Lund, Sweden.

Most patients with anti-glomerular basement membrane (anti-GBM) disease present with rapidly progressive glomerulonephritis with or without pulmonary haemorrhage; however, there are several variants and vigilance is necessary to make a correct diagnosis. Such variants include overlap with anti-neutrophil cytoplasm antibodies-associated vasculitis and membranous nephropathy as well as anti-GBM occurring de novo after renal transplantation. Moreover, patients can present with isolated pulmonary haemorrhage as well as with negative tests for circulating anti-GBM. Read More

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http://dx.doi.org/10.1093/ndt/gfy327DOI Listing
October 2018
9 Reads

Bacterial endocarditis manifesting as autoimmune pulmonary renal syndrome: ANCA-associated lung hemorrhage and pauci-immune crescentic glomerulonephritis
.

Clin Nephrol 2018 Dec;90(6):431-433

The etiology of pulmonary renal syndrome can be broadly divided into infectious and autoimmune (predominantly ANCA vasculitis). The importance of timely differentiating between them stems from the deleterious effects of their respective treatment if misdiagnosed. Serology and tissue evaluation by pathology are employed to aid in this, however, in rare cases, this can be difficult. Read More

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http://dx.doi.org/10.5414/CN109495DOI Listing
December 2018
14 Reads

Membranous nephropathy followed by anti-glomerular basement disease: A case report and review of clinical presentation and treatment.

SAGE Open Med Case Rep 2018 19;6:2050313X18807621. Epub 2018 Oct 19.

Department of Nephrology, Heidelberg University Hospital, Heidelberg, Germany.

Membranous nephropathy is a common cause of nephrotic syndrome in adults and can be primary or secondary through autoimmune disease, medication, infection, or malignancy. Rapidly progressive glomerulonephritis with crescent formation is rare in patients with membranous nephropathy. Thus, in cases with rapid decline in renal function, after excluding complications such as malignant hypertension, acute hypersensitivity interstitial nephritis, and bilateral renal vein thrombosis, the simultaneous occurrence of a superimposed glomerulonephritis should be considered. Read More

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http://journals.sagepub.com/doi/10.1177/2050313X18807621
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http://dx.doi.org/10.1177/2050313X18807621DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6196619PMC
October 2018
11 Reads

A unique case of rapidly progressive glomerulonephritis following dexamethasone/bortezomib/thalidomide treatment for myeloma.

Nephrology (Carlton) 2018 11;23(11):1065-1067

Department of Medicine, Nephrology, University of Padova, Padova, Italy.

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http://dx.doi.org/10.1111/nep.13249DOI Listing
November 2018
3 Reads

Histopathological Classification-A Prognostic Tool for Rapidly Progressive Glomerulonephritis.

Medicina (Kaunas) 2018 Apr 17;54(2). Epub 2018 Apr 17.

Centre of Nephrology, Vilnius University Hospital Santaros Klinikos, LT⁻08661 Vilnius, Lithuania.

Recently proposed histopathological classification may predict patient outcome in pauci-immune glomerulonephritis. This study sought to prove that the prognostic effect could be extended to all types of rapidly progressive glomerulonephritis. Retrospective analysis of patients diagnosed with rapidly progressive glomerulonephritis between April 1999 and August 2015 was performed. Read More

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http://www.mdpi.com/1648-9144/54/2/17
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http://dx.doi.org/10.3390/medicina54020017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6037248PMC
April 2018
9 Reads

Histopathological classification of anti-neutrophil cytoplasmic antibody-associated glomerulonephritis in a nationwide Japanese prospective 2-year follow-up cohort study.

Clin Exp Nephrol 2018 Oct 10. Epub 2018 Oct 10.

Department of Nephrology, Internal Medicine, Nagoya University Graduate School of Medicine, Aichi, Japan.

Background: The prognostic value of the EUVAS-proposed histopathological classification of anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis has been evaluated throughout the world. Here, we performed a Japanese nationwide biopsy survey to assess the association between this histopathological classification and renal prognosis after 2-year follow-up in ANCA-associated glomerulonephritis.

Methods: We collected 67 renal biopsy materials of the 321 entries in the RemIT-JAV-RPGN cohort study, and assessed their histologies. Read More

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http://dx.doi.org/10.1007/s10157-018-1656-1DOI Listing
October 2018
3 Reads

Successful treatment of steroid-refractory double-positive ANCA and anti-GBM disease with a combination of plasma exchange and immunosuppression: A case report and literature review.

Respir Med Case Rep 2018 1;25:242-246. Epub 2018 Oct 1.

Division of Neurology, Respirology, Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Kiyotake, Miyazaki, 889-1692, Japan.

The concurrence of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and anti-glomerular basement membrane (GBM) disease, known as double-positive disease, is rare, but it occurs at a much higher frequency than expected by chance. Double-positive disease has an aggressive clinical course, with no optimal treatment strategy. Here we describe a patient with steroid-refractory double-positive disease who was treated successfully with the addition of plasma exchange (PE) and cyclophosphamide (CPA). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22130071183027
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http://dx.doi.org/10.1016/j.rmcr.2018.09.016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6174835PMC
October 2018
10 Reads

Anti-Glomerular Basement Membrane Disease.

Rheum Dis Clin North Am 2018 11 7;44(4):651-673. Epub 2018 Sep 7.

Renal and Vascular Inflammation Section, Department of Medicine, Imperial College London, Commonwealth Building, Hammersmith Hospital Campus, Du Cane Road, London W12 ONN, UK; Vasculitis Clinic, Imperial College Healthcare NHS Trust, Hammersmith Hospital, Du Cane Road, London W12 0HS, UK. Electronic address:

Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune small vessel vasculitis characterized by autoreactivity to antigens in type IV collagen chains expressed in glomerular and alveolar basement membrane. The detection of circulating anti-GBM antibodies, which are shown to be directly pathogenic, is central to disease diagnosis. Clinically, anti-GBM disease usually presents with rapidly progressive glomerulonephritis with or without alveolar hemorrhage. Read More

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http://dx.doi.org/10.1016/j.rdc.2018.06.011DOI Listing
November 2018
3 Reads

Renal Involvement in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Rheum Dis Clin North Am 2018 11 7;44(4):525-543. Epub 2018 Sep 7.

Division of Nephrology, Vasculitis and Glomerulonephritis Center, Massachusetts General Hospital, 101 Merrimac Street, Boston, MA 02114, USA. Electronic address:

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is the most common cause of rapidly progressive glomerulonephritis. ANCAs play an important role in the pathogenesis and diagnosis of AAV. The classic renal lesion in AAV is a pauci-immune necrotizing and crescentic glomerulonephritis. Read More

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http://dx.doi.org/10.1016/j.rdc.2018.06.001DOI Listing
November 2018
13 Reads

[Rapidly progressive proliferative glomerulonephritis with monoclonal immunoglobulin G deposits despite the mild histological changes. Case report].

Orv Hetil 2018 Sep;159(38):1567-1572

Patológiai Intézet, Szegedi Tudományegyetem, Általános Orvostudományi Kar Szeged.

Proliferative glomerulonephritis with monoclonal immunoglobulin G (IgG) deposits is characterized by granular deposits of monoclonal IgG; histologically it has typically a membranoproliferative or endocapillary pattern, and seen electronmicroscopically there are dense deposits without substructure. Here, we present the case of a 62-year-old Caucasian woman who was admitted with rapidly progressive kidney failure. The patient's status, the laboratory and imaging examinations did not support prerenal, postrenal and - among the intrinsic causes - vascular and tubulointerstitial origin. Read More

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http://dx.doi.org/10.1556/650.2018.31072DOI Listing
September 2018
5 Reads

Crescentic glomerulonephritis with anti-GBM antibody but no glomerular deposition.

BMC Nephrol 2018 Sep 12;19(1):228. Epub 2018 Sep 12.

UCL Centre for Nephrology, Royal Free Hospital, London, NW3 2PF, UK.

Background: Anti-glomerular basement membrane (GBM) antibodies are highly specific for Goodpasture's or anti-GBM disease, in which they are generally directed against the non-collagenous (NC1) domain of the alpha 3 chain of type IV collagen(α3(IV)), and less commonly, toward the α 4(IV) or α 5(IV) chains, which form a triple helical structure in GBM and alveolar basement membrane (ABM). Alterations in the hexameric structure of the NC1 (α3 (IV)), allows novel epitopes to be exposed and an immune response to develop, with subsequent linear antibody deposition along the GBM, leading to a crescentic glomerulonephritis. Positive anti-GBM antibodies are assumed to be pathogenic and capable of binding GBM in vivo, especially in the context of rapidly progressive glomerulonephritis. Read More

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http://dx.doi.org/10.1186/s12882-018-1027-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6136232PMC
September 2018
2 Reads

Pauci-immune Glomerulonephritis in Systemic Lupus Erythematosus (SLE).

Cureus 2018 Jul 9;10(7):e2949. Epub 2018 Jul 9.

Medicine, The Johns Hopkins University School of Medicine, Essex, USA.

Glomerulonephritis (GN) in lupus is generally an immune complex glomerulonephritis from the deposition of immunoglobulin and complements. Pauci-immune GN is the most common cause of rapidly progressive GN and is frequently associated with an anti-nuclear cytoplasmic antibody (ANCA). We report a patient with a history of systemic lupus erythematosus who presented with worsening proteinuria and was subsequently diagnosed with pauci-immune GN on renal biopsy, in the absence of ANCA. Read More

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http://dx.doi.org/10.7759/cureus.2949DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6128587PMC
July 2018
2 Reads

PYCARD Gene Plays a Key Role in Rapidly Progressive Glomerulonephritis: Results of a Weighted Gene Co-Expression Network Analysis.

Am J Nephrol 2018 6;48(3):193-204. Epub 2018 Sep 6.

Background: Rapidly progressive glomerulonephritis (RPGN) is caused by various diseases process, thereby resulting in extensive crescent formation, which could lead to a rapid loss of kidney function. The molecular pathogenesis of RPGN remains largely unknown and requires clarification. The weighted gene co-expression network analysis (WGCNA) is a powerful bioinformatics tool to identify meaningful molecules in diseases. Read More

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http://dx.doi.org/10.1159/000492725DOI Listing
September 2018
8 Reads

Have We Made "Rapid Progress" Understanding the Pathogenesis in Rapidly Progressive Glomerulonephritis?

Am J Nephrol 2018 5;48(3):190-192. Epub 2018 Sep 5.

Department of Internal Medicine, Section on Nephrology, Wake Forest School of Medicine, Winston-Salem, North Carolina, USA.

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http://dx.doi.org/10.1159/000492726DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6177289PMC
September 2018
5 Reads

Systemic lupus erythematosus: a case-based presentation of renal, neurologic, and hematologic emergencies.

Expert Rev Clin Immunol 2018 10 20;14(10):803-816. Epub 2018 Sep 20.

c Solovy Arthritis Research Society Professor of Medicine, Department of Medicine/Rheumatology , Northwestern University Feinberg School of Medicine , Chicago , IL , USA.

Introduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder with systemic manifestations and multiorgan involvement. Although primarily diagnosed, and managed in the outpatient setting, it can occasionally present with life-threatening complications that require rapid assessment and urgent aggressive therapy. Areas covered: In our review, we explore three organ systems that are often affected in SLE, but have the potential to present as medical emergencies; these are the kidney, the central nervous system, and the hematologic system. Read More

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http://dx.doi.org/10.1080/1744666X.2018.1518132DOI Listing
October 2018
12 Reads

Acute renal failure by rapidly progressive glomerulonephritis with IgA deposition in a patient concomitantly diagnosed with multibacillary Hansen's disease: a case report.

J Bras Nefrol 2018 Aug 23. Epub 2018 Aug 23.

Programa de Pós-Graduação em Ciências Médicas, Departamento de Clínica Médica, Universidade Federal do Ceará, Fortaleza, CE, Brasil.

Rapidly progressive glomerulonephritis (RPGN) is a renal disease with an extensive differential diagnosis. This paper reports the case of a 55-year-old female patient diagnosed with Hansen's disease with acute progressive renal impairment after developing lower limb pyoderma. The association between Hansen's and kidney disease has been well documented, with glomerulonephritis (GN) ranked as the most common form of renal involvement. Read More

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http://dx.doi.org/10.1590/2175-8239-jbn-2018-0056DOI Listing
August 2018
14 Reads

Pediatric Nephrology and Rheumatology Practice Patterns in Granulomatosis with Polyangiitis: A Midwest Pediatric Nephrology Consortium Study.

Int J Nephrol 2018 5;2018:8413096. Epub 2018 Aug 5.

Virginia Commonwealth University, Internal Medicine Nephrology, Virginia, USA.

Objective: To assess practice pattern similarities and differences amongst pediatric rheumatologists and nephrologists in the management of pediatric Granulomatosis with Polyangiitis (GPA).

Methods: A voluntary survey was distributed to the Midwest Pediatric Nephrology Consortium Group (MWPNC) and an international pediatric rheumatology email listserv in 2016-2017. Data were collected on general practice characteristics and preferences for induction management under three clinical scenarios (A-C): newly diagnosed GPA with glomerulonephritis, GPA with rapidly progressive glomerulonephritis, and GPA with pulmonary hemorrhage. Read More

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https://www.hindawi.com/journals/ijn/2018/8413096/
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http://dx.doi.org/10.1155/2018/8413096DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6098870PMC
August 2018
7 Reads

An uncommon cause of rapidly progressive renal failure in a lupus patient: Pauci-immune crescentic glomerulonephritis.

Saudi J Kidney Dis Transpl 2018 Jul-Aug;29(4):989-992

Department of Nephrology and Clinical Transplantation, Institute of Kidney Diseases and Research Center, Dr. H. L. Trivedi Institute of Transplantation Sciences, Ahmedabad, Gujarat, India.

We report a case of systemic lupus erythematosus (SLE) who presented with rapidly progressive renal failure (RPRF) with positive antinuclear antibody (ANA) and anti-double-stranded DNA (dsDNA) antibody and active urinary sediment in the form of microscopic hematuria and proteinuria. Provisional clinical diagnosis of lupus nephritis was made. Renal biopsy showed pauci-immune crescentic glomerulonephritis, the diagnosis of which was supported by positive serum anti-MPO antibody. Read More

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http://dx.doi.org/10.4103/1319-2442.239632DOI Listing
August 2018
2 Reads

Short-term outcome of clinical and histopathologic variants of mesangiocapillary glomerulonephritis in children: A retrospective analysis from a tertiary care center.

J Pak Med Assoc 2018 Aug;68(8):1199-1204

Paediatric Nephrology Department, SIUT, Karachi.

Objective: To study the frequency, clinicopathological features and short-term outcome of mesangiocapillary glomerulonephritis (MCGN) in children at a tertiary care kidney center in Pakistan.

Methods: A descriptive, observational study was conducted at the Paediatric Nephrology Department, Sindh Institute of Urology and Transplantation, Karachi, from January 2011 till December 2015. A review of all paediatric (<18 years) renal biopsies during the study period was performed and cases of MCGN were enrolled. Read More

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August 2018
2 Reads

CIDP, myasthenia gravis, and membranous glomerulonephritis - three autoimmune disorders in one patient: a case report.

BMC Neurol 2018 Aug 14;18(1):113. Epub 2018 Aug 14.

Department of Neurology, Essen University Hospital, Hufelandstrasse 55, 45147, Essen, Germany.

Background: We present a patient fulfilling the electrophysiological criteria for definite chronic inflammatory demyelinating polyneuropathy (CIDP), antibody-positive myasthenia gravis (MG), and membranous glomerulonephritis (MGN) confirmed by biopsy. To our knowledge, this is the first case of the concomitant appearance of these three autoimmune diseases in a single patient.

Case Representation: A 42-year-old Caucasian male presented with rapidly progressive gait disturbance, distal weakness of the lower extremities, ascending hypoesthesia, impaired fine motor skills, and beginning cranial nerve palsy showing dysarthrophonia, facial paralysis, and eye movement abnormalities and was diagnosed as rapid onset (atypical) CIDP. Read More

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http://dx.doi.org/10.1186/s12883-018-1120-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6092826PMC
August 2018
7 Reads

Does cyclophosphamide still play a role in glomerular diseases?

Autoimmun Rev 2018 Oct 11;17(10):1022-1027. Epub 2018 Aug 11.

Nephrology Unit, Fondazione Ca' Granda IRCCS Ospdale Maggiore Policlinico Milano, Via della Commenda 15. 20122 Milano, Italy. Electronic address:

Cyclophosphamide is a prodrug that is converted to inactive carboxy-cyclophosphamide, acrolein and phosphoramide mustard, an agent that adds alkyl groups to oxygen and nitrogen atoms of guanine, one of the four nitrogen bases that form the DNA nucleotides, causing DNA cross-links and introducing DNA breaks. These cytotoxic and mutagenic effects mainly occur in proliferating cells. Repair mechanisms may prevent DNA damage in quiescent cells, but they may be insufficient to contrast the side effects of cyclophosphamide if high doses of the drug are used. Read More

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http://dx.doi.org/10.1016/j.autrev.2018.04.007DOI Listing
October 2018
4 Reads

Clinical and Pathological Characteristics of Elderly Japanese Patients with IgA Vasculitis with Nephritis: A Case Series.

Intern Med 2019 Jan 10;58(1):31-38. Epub 2018 Aug 10.

Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, Japan.

Objective This case series aimed to identify the clinical and pathological characteristics of elderly patients (≥60 years) with biopsy-proven IgA vasculitis with nephritis (IgAVN). Methods The clinical and pathological presentation and treatment outcomes were compared between two groups. Patients Patients with IgAVN who were ≥19 years old at the time of their renal biopsy were divided into elderly (≥60 years) and adult (19-59 years) groups. Read More

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http://dx.doi.org/10.2169/internalmedicine.1379-18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367074PMC
January 2019
3 Reads

Apheresis for Kidney Disease.

Contrib Nephrol 2018 24;196:188-193. Epub 2018 Jul 24.

Plasma exchange or double filtration plasmapheresis for rapidly progressive glomerulonephritis, and low-density lipoprotein (LDL) apheresis or leukocytapheresis for nephritic syndrome are two major apheresis therapies for kidney diseases. In addition to these apheresis therapies, plasma exchange for lupus nephritis or LDL apheresis for refractory focal segmental glomerulonephritis is clinically valuable and established. Although several possibilities of apheresis for kidney diseases were speculated in animal experiments or human studies, clinical applications have thus far been limited. Read More

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http://dx.doi.org/10.1159/000485721DOI Listing
July 2018
13 Reads

[Posterior Reversible Encephalopathy Syndrome (PRES) induced by Rituximab in two patients with vasculitis, and treated by hemodialysis].

G Ital Nefrol 2018 Jul;35(4)

U.O. Nefrologia Universitaria, Azienda Ospedaliero-Universitaria Policlinico e Università degli Studi Aldo Moro di Bari.

Small and medium vessel vasculitides, either ANCA-associated or caused by anti-GBM antibodies, are multisystemic diseases with predominantly renal involvement that often require dialysis support; clinical remission can be induced with immunosuppressive therapies including apheretic treatments, high doses of steroids, and immune suppressants. In addition to the complications resulting from the primary pathological process, those associated with the immunosuppressive therapies are not negligible. Reversible Posterior Encephalopathy Syndrome (PRES) is a clinical condition with a hyperacute onset, which can complicate the evolution of vasculitides while treated by immunosuppressive therapy. Read More

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July 2018
1 Read

ASK1 inhibitor treatment suppresses p38/JNK signalling with reduced kidney inflammation and fibrosis in rat crescentic glomerulonephritis.

J Cell Mol Med 2018 Sep 11;22(9):4522-4533. Epub 2018 Jul 11.

Department of Nephrology, Monash Medical Centre, Clayton, Vic., 3168, Australia.

Activation of p38 mitogen-activated protein kinase (MAPK) and c-Jun amino terminal kinase (JNK) is prominent in human crescentic glomerulonephritis. p38 and JNK inhibitors suppress crescentic disease in animal models; however, the upstream mechanisms inducing activation of these kinases in crescentic glomerulonephritis are unknown. We investigated the hypothesis that apoptosis signal-regulating kinase 1 (ASK1/MAP3K5) promote p38/JNK activation and renal injury in models of nephrotoxic serum nephritis (NTN); acute glomerular injury in SD rats, and crescentic disease in WKY rats. Read More

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http://dx.doi.org/10.1111/jcmm.13705DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6111820PMC
September 2018
5 Reads

CD8+ cells and glomerular crescent formation: outside-in as well as inside-out.

J Clin Invest 2018 Aug 9;128(8):3231-3233. Epub 2018 Jul 9.

Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Clayton, Victoria, Australia.

Crescentic glomerulonephritis, a complication of severe immune glomerular injury, is the pathological correlate of rapidly progressive glomerulonephritis, mediated by both humoral and cellular effectors. In the current issue of the JCI, Chen et al. have implicated Bowman's capsule in functionally isolating potentially immune effectors, specifically antigen-specific CD8+ T lymphocytes, from podocytes. Read More

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http://dx.doi.org/10.1172/JCI122045DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6063495PMC
August 2018
5 Reads

Bowman's capsule provides a protective niche for podocytes from cytotoxic CD8+ T cells.

J Clin Invest 2018 Aug 9;128(8):3413-3424. Epub 2018 Jul 9.

Institute of Precision Immunology, Icahn School of Medicine at Mount Sinai, New York New York, USA.

T cells play a key role in immune-mediated glomerulonephritis, but how cytotoxic T cells interact with podocytes remains unclear. To address this, we injected EGFP-specific CD8+ T cells from just EGFP death inducing (Jedi) mice into transgenic mice with podocyte-specific expression of EGFP. In healthy mice, Jedi T cells could not access EGFP+ podocytes. Read More

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http://dx.doi.org/10.1172/JCI97879DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6063505PMC
August 2018
5 Reads

Pulmonary cavitary lesions may be one of the presenting features in Ig A nephropathy.

Reumatismo 2018 Jul 6;70(2):111-114. Epub 2018 Jul 6.

Department of Internal Medicine, University of Health Sciences, Kartal Dr. Lutfi Kirdar Training and Research Hospital, Istanbul.

Immunoglobulin A (Ig A) nephropathy is the most frequent primary glomerulonephritis. Renal limited disease is the most widespread clinical form of the disease. Pulmonary involvement may also be seen concomitantly and capillaritis with pulmonary hemorrhage is the most frequent pulmonary involvement. Read More

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http://dx.doi.org/10.4081/reumatismo.2018.1037DOI Listing
July 2018
1 Read

The Respiratory System in Autoimmune Vascular Diseases.

Respiration 2018;96(1):12-28. Epub 2018 Jul 4.

Hospices Civils de Lyon, Department of Respiratory Medicine, National Reference Center for Rare Pulmonary Diseases, Lyon, France.

The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis, affects the respiratory tract more commonly than other vasculitis types. Granulomatosis with polyangiitis is always associated with upper or lower respiratory tract involvement. Read More

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http://dx.doi.org/10.1159/000486899DOI Listing
December 2018
20 Reads