Search Our Scientific Publications & Authors

Medicine (Baltimore) 2019 Feb;98(7):e14557

Department of Nephrology.

Rationale: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and anti-glomerular basement membrane (GBM) antibody disease are both rare autoimmune diseases. Monoclonal gammopathy of undetermined significance (MGUS) is one of the most common causes of plasma cell dyscrasias (PCD). The three entities can cause renal lesions via different mechanisms and, however, they have not been reported in a single patient with renal lesion. Read More

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

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

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

Am J Physiol Renal Physiol 2019 Jan 16. Epub 2019 Jan 16.

Institute of Immunology, University Medical Center Hamburg-Eppendorf, Germany.

Anti-glomerular basement membrane (anti-GBM) disease is characterized by antibodies and T cells directed against the Goodpasture antigen α3(IV)NC1 of the GBM. Consequences are the deposition of autoantibodies along the GBM and the development of crescentic glomerulonephritis (GN) with rapid loss of renal function. Foxp3 regulatory T cells (Treg cells) are crucial for the maintenance of peripheral tolerance to self-antigens and the prevention of immunopathology. Read More

BMC Nephrol 2019 Jan 8;20(1). Epub 2019 Jan 8.

Department of Nephrology, CHU Rennes, Rennes, France.

Background: Goodpasture Syndrome (GS) is an autoimmune disease caused by the development of auto-antibodies against the Glomerular Basement Membrane (GBM). Linear deposit of immunoglobulins G on the GBM detected by immunofluorescence analysis of renal biopsies is a GS pathognomonic finding. GS is commonly monophasic and its incidence is 1. Read More

Kidney Int Rep 2019 Jan 22;4(1):163-167. Epub 2018 Aug 22.

University College London Centre for Nephrology, Royal Free Hospital, London, UK.

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

Kidney Int 2019 Feb 3;95(2):310-320. Epub 2018 Dec 3.

Transplantation Research Center, Brigham and Women's Hospital, Boston, Massachusetts, USA; Division of Renal Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA. Electronic address:

Crescentic glomerulonephritis is an inflammatory condition characterized by rapid deterioration of kidney function. Previous studies of crescentic glomerulonephritis have focused on immune activation in the kidney. However, the role of fibroblastic reticular cells, which reside in the stromal compartment of the kidney lymph node, has not been studied in this condition. Read More

Am J Kidney Dis 2018 Dec 7. Epub 2018 Dec 7.

Department of Internal Medicine, Wake Forest School of Medicine, Winston-Salem, NC. Electronic address:

Alemtuzumab, a humanized monoclonal antibody that targets CD52 antigens on lymphocytes and monocytes, has shown efficacy in preventing relapse in relapsing-remitting multiple sclerosis. Despite known severe (yet rare) renal side effects such as anti-glomerular basement membrane disease and membranous glomerulopathy, to our knowledge, alemtuzumab has never been documented to cause drug-induced thrombotic microangiopathy. We describe a 39-year-old woman with relapsing-remitting multiple sclerosis who developed acute kidney injury requiring renal replacement therapy after 1 dose of alemtuzumab, as well as microangiopathic hemolytic anemia and thrombocytopenia. Read More

Best Pract Res Clin Rheumatol 2018 02 16;32(1):52-62. Epub 2018 Aug 16.

Department of Microbiology and Immunology, KU Leuven, and Department of Laboratory Medicine, University Hospitals Leuven, Leuven, Belgium. Electronic address:

The diagnosis of systemic vasculitis is challenging. Laboratory testing may provide useful information. Routine laboratory tests include erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), blood count, serum creatinine, urinalysis, specific autoantibodies, complement, immunoglobulin, cryoglobulin, and Hepatitis B and C serology. Read More

Kidney Int 2018 Dec;94(6):1087-1098

Division of Nephrology, Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, LMU München, Germany. Electronic address:

During the past decade the formation of neutrophil extracellular traps (NETs) has been recognized as a unique modality of pathogen fixation (sticky extracellular chromatin) and pathogen killing (cytotoxic histones and proteases) during host defense, as well as collateral tissue damage. Numerous other triggers induce NET formation in multiple forms of sterile inflammation, including thrombosis, gout, obstruction of draining ducts, and trauma. Whether neutrophils always die along with NET release, and if they do die, how, remains under study and is most likely context dependent. Read More

Clin Nephrol 2019 Feb;91(2):101-106

Background: Antiglomerular basement membrane (anti-GBM) antibody disease is a rare condition causing pulmonary hemorrhage and necrotizing glomerulonephritis (pulmonary renal syndrome).

Case: We report a 30-year-old woman who presented with life-threatening pulmonary hemorrhage and an active urinary sediment, with normal glomerular filtration rate in the 13 week of pregnancy. Anti-GBM antibodies in serum were negative, but perinuclear antineutrophil cytoplasmatic antibodies (p-ANCA) were detected. Read More

J Thorac Dis 2018 Sep;10(9):5560-5575

Interdisciplinary Research Group in Pneumology, Institute of Sanitary Research of Santiago de Compostela (IDIS), Santiago de Compostela, Spain.

Systemic vasculitides frequently affect the pulmonary vasculature. As the signs and symptoms of pulmonary vasculitis are variable and nonspecific, diagnosis and treatment represent a real challenge. Vasculitides should be given consideration, as these diseases present severe manifestations of rapidly progressing pulmonary disease. Read More

Int J Nephrol Renovasc Dis 2018 16;11:267-270. Epub 2018 Oct 16.

Division of Nephrology, Department of Pediatrics, University of Minnesota Medical School, Minneapolis, MN 55454, USA,

Alport syndrome is an inherited disorder of basement membrane collagen IV that frequently results in end-stage renal disease. Patients with Alport syndrome who undergo renal transplantation have generally excellent outcomes. Posttransplant antiglomerular basement membrane nephritis is a rare complication of renal transplantation for Alport syndrome. Read More

Semin Respir Crit Care Med 2018 08 7;39(4):504-510. Epub 2018 Nov 7.

Department of Internal Medicine, Hôpital Cochin, Université Paris, Paris, France.

Pulmonary vasculitis can be heterogeneous and have some pathophysiological, clinical, and biological specificities that might influence treatment choices. Main vasculitides associated with pulmonary vasculitis include antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, immune complex small vessel vasculitis, and antiglomerular basement membrane disease. Patient characteristics and association of lung involvement with other manifestations affecting disease severity may also influence treatment strategy. Read More

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

Semin Respir Crit Care Med 2018 08 7;39(4):482-493. Epub 2018 Nov 7.

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

Defined by the accumulation of red blood cells into the alveolar space, diffuse alveolar hemorrhage (DAH) is a severe and potentially fatal medical condition requiring careful attention. In contrast to simple extravasation of erythrocytes facilitated by impaired hemostasis or hemodynamic causes, DAH in vasculitis is due to capillaritis, that is, inflammation of capillaries. Dyspnea, hemoptysis, chest infiltrates, and abrupt fall of blood hemoglobin level represent the cardinal features of DAH; yet, hemoptysis is lacking in one-third of cases. Read More

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

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

BMC Nephrol 2018 Oct 22;19(1):286. Epub 2018 Oct 22.

Ziekenhuisgroep Twente, Department of Nephrology, Almelo, The Netherlands.

Background: Hantavirus infection is an uncommon cause of acute renal failure with massive proteinuria. Serology tests to support a presumptive diagnosis usually take a few days. During the initial work-up, autoimmune causes including anti-glomerular basement membrane (GBM) glomerulonephritis need to be excluded, because these require urgent therapy. Read More

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

Kidney Int 2018 Dec 12;94(6):1141-1150. Epub 2018 Oct 12.

Leiden University Medical Center, Department of Nephrology, Leiden, The Netherlands. Electronic address:

Properdin is the only known positive regulator of complement activation by stabilizing the alternative pathway convertase through C3 binding, thus prolonging its half-life. Recent in vitro studies suggest that properdin may act as a specific pattern recognition molecule. To better understand the role of properdin in vivo, we used an experimental model of acute anti-glomerular basement membrane disease with wild-type, C3- and properdin knockout mice. Read More

J Clin Pathol 2019 Jan 12;72(1):31-37. Epub 2018 Oct 12.

National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China

Aim: To explore the clinicopathological characteristics of patients with anti-GBM antibody-negative anti-GBM disease.

Methods: The clinical and renal pathological findings were retrospectively studied in 19 patients. All patients met the following inclusion criteria: linear GBM IgG deposition on immunofluorescence(IF); and lack of serum anti-GBM antibodies by ELISA and indirect immunofluorescence assay. Read More

Ann Hematol 2018 Oct 9. Epub 2018 Oct 9.

Pathology Department, Facultad de Medicina, Pontificia Universidad Católica, Santiago de Chile, Chile.

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

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

BMC Nephrol 2018 Sep 20;19(1):241. Epub 2018 Sep 20.

Service de Néphrologie, Hémodialyse, Aphérèses et Transplantation, CHU Grenoble-Alpes, Avenue Maquis du Grésivaudan, 38700, La Tronche, France.

Background: Anti-glomerular basement-membrane (anti-GBM) disease (or Goodpasture disease) is characterized by severe kidney and lung involvement. Prognoses have improved with treatments that combine plasma exchange and immunosuppressive drugs. However, patients with severe renal involvement can have poor renal outcomes and cyclophosphamide can cause significant complications. Read More

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

Clin Rheumatol 2018 Nov 10;37(11):3151-3155. Epub 2018 Sep 10.

Division of Rheumatology, Louisiana State University, 1542 Tulane Ave., Box T4M-2, New Orleans, LA, 70112, USA.

RPGN can be subdivided into three categories on an immunopathologic basis: pauci-immune glomerulonephritis (PIGN), anti-glomerular basement membrane glomerulonephritis (anti-GBM disease), or immune complex-mediated glomerulonephritis (GN). PIGN is the most common cause of RPGN (80% of cases). The most common etiology of PIGN is anti-neutrophil cytoplasmic antibody (ANCA)-associated GN, which accounts for up to 90% of PIGN. Read More

Kidney Int Rep 2018 Sep 8;3(5):1229-1232. Epub 2018 May 8.

Division of Nephrology, University Hospitals, UZGasthuisberg, Leuven, Belgium.

Respir Med Case Rep 2018 24;25:181-183. Epub 2018 Aug 24.

Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Gainesville, USA.

A 26 year old female presented for recurrent blood tinged sputum during the previous year with development of frank hemoptysis three days prior to admission. Diffuse alveolar hemorrhage (DAH) was confirmed with serial lavages. The patient had no history of autoimmune disease, vascular thrombosis or pregnancy morbidity including miscarriages or pre-eclampsia. Read More

Clin Exp Rheumatol 2018 Aug 29. Epub 2018 Aug 29.

Division of Nephrology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.

Objectives: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder and is characterised by elevated serum IgG4 concentrations and dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells. IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most common manifestation of IgG4-related kidney disease (IgG4-RKD). We report four cases of kidney injury with concurrent IgG4-TIN and crescentic glomerulonephritis confirmed by renal pathology. Read More

Clin Case Rep 2018 Aug 1;6(8):1647-1648. Epub 2018 Jul 1.

Division of Pathology, Immunology and Laboratory Medicine University of Florida Gainesville FL USA.

Antiglomerular basement membrane (GBM) antibody disease is a vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of autoantibodies. It can be both life- and organ-threatening and delayed diagnosis may be detrimental. Alveolar hemorrhage and microscopic hematuria should be excluded in anybody who is suspected of this disease. Read More

Nephrol Dial Transplant 2019 Feb;34(2):193-199

Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA.

A kidney biopsy is done to determine the etiology of the glomerulonephritis (GN) and the severity of the lesion, to identify whether other lesions, related to or not related to the GN, are present on the kidney biopsy and finally to ascertain the extent of chronicity of the GN. The etiology of GN is based on the classification of GN into five groups: immune complex-mediated GN, antineutrophil cytoplasmic antibody (ANCA)-associated GN, anti-glomerular basement membrane (GBM) GN, monoclonal immunoglobulin-mediated GN and C3 glomerulopathy. Immune complex GN includes multiple specific diseases such as lupus nephritis, IgA nephropathy, infection-related GN and fibrillary GN. Read More

N Engl J Med 2018 Aug;379(6):568-578

From the Department of Medicine, University of North Carolina School of Medicine, Chapel Hill (R.J.F.); and the Departments of Radiology (J.S.H.), Medicine (A.Z.F.), and Pathology (I.A.R.), Massachusetts General Hospital, and the Departments of Radiology (J.S.H.), Medicine (A.Z.F.), and Pathology (I.A.R.), Harvard Medical School - both in Boston.

BMJ Case Rep 2018 Jul 24;2018. Epub 2018 Jul 24.

Nephrology, Johns Hopkins University, Baltimore, Maryland, USA.

Approximately one in four patients (23.3%) with inflammatory myositis including dermatomyositis can require evaluation for acute kidney injury. The main cause of kidney injury is acute tubular necrosis from medications or myoglobinuria, though clinicians should be aware of a wide variety of possible aetiologies. Read More

Lung Cancer 2018 Aug 19;122:22-24. Epub 2018 May 19.

Department of Internal Medicine, Kochi Red Cross Hospital, Kochi, 2-13-51 Shin-honmachi, Kochi, 780-8562, Japan.

Nivolumab, an anti-programmed death-1 immune checkpoint inhibitor (ICI), is now widely used to treat numerous cancers. Although most adverse effects related to ICIs are controllable, fulminant immune-related adverse events can occur. A 74-year-old patient with non-small-cell lung cancer was treated with nivolumab as a second-line treatment. Read More

PeerJ 2018 9;6:e5178. Epub 2018 Jul 9.

Department of Nephrology, Chinese PLA General Hospital, Chinese PLA Institute of Nephrology, State Key Laboratory of Kidney Diseases, National Clinical Research Center for Kidney Diseases, Beijing Key Laboratory of Kidney Disease, Beijing, China.

Background: The importance of circulating antibodies as biomarkers of kidney disease has recently been recognized. However, no study has systematically described the methodology of sample preparation and storage regarding antibodies as biomarkers of kidney disease. It remains unknown whether repetitive freeze-thaw cycles, physical disturbances, storage at different temperatures or for different periods of time, or haemolytic or turbid serum samples affect antibody measurements. Read More

Kidney Int Rep 2018 Jul 23;3(4):991-996. Epub 2018 Mar 23.

Department of Pathology, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada.

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

J Coll Physicians Surg Pak 2018 Jul;28(7):518-522

Department of Immunology, University of Health Sciences (UHS), Lahore.

Objective: To assess activation of immune system in rheumatic heart disease (RHD) patients in the form of AECA, ACL and anti GBM antibodies.

Study Design: Descriptive, observational study.

Place And Duration Of Study: Department of Immunology, University of Health Sciences (UHS), Lahore, and Outpatient Department, Punjab Institute of Cardiology, from February 2015 to January 2016. Read More

Clin Nephrol 2018 Oct;90(4):291-295

Membranous nephropathy (MN) is a common cause of nephrotic syndrome. Rarely, it can present with rapidly-progressive renal failure and hematuria. While this may be due to lupus nephritis, superimposed anti-glomerular basement membrane (GBM) disease, or antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, there have been rare reports of anti-GBM - and ANCA-negative crescentic glomerulonephritis presenting in primary membranous nephropathy (pMN). Read More

Georgian Med News 2018 May(278):107-114

1Higher state educational establishment of Ukraine "Ukrainian medical stomatological academy", Poltava; 2State scientific establishment "Scientific-practical centre of prophylactic and clinical medicine" of State affairs management, Kyiv.

The article presents a clinical case with intravital diagnosis of Goodpascher's syndrome in a 22 years old patient. In this work we analyze clinical, laboratory-instrumental, pathologoanatomic and pathomorphological aspects of the disease, which was accompanied by glomerulonephritis with rapidly progressive renal insufficiency, anemia, arterial hypertension and symptoms of lung injury. Article emphasizes on the necessity of timely diagnosis of Goodpascher's syndrome and following administration of immunosuppressive therapy. Read More

Curr Rheumatol Rev 2018 05 16. Epub 2018 May 16.

Division of Nephrology, University of Washington School of Medicine, Seattle, Washington. United States.

Background: It is unclear whether patients with anti-glomerular basement membrane (GBM) disease and anti-neutrophil cytoplasmic antibodies (ANCA), so called "double-positive" (DP), have a different clinical presentation and outcome compared to patients with anti-GBM antibody disease alone. This study describes the clinical and histologic characteristics as well as the patient and renal outcomes of DP patients at the University of Washington compared to patients with anti-GBM antibody disease alone.

Methods: Adults admitted to the University of Washington and Harborview Medical Centers from 2000 to 2016 who had a kidney biopsy showing anti-GBM disease characterized by crescentic glomerulonephritis with strong linear staining of glomerular basement membranes for IgG by immunofluorescence were included. Read More

J Nippon Med Sch 2018 ;85(2):138-144

Department of Nephrology, Nippon Medical School.

We report here a case of systemic lupus erythematosus (SLE) with pulmonary hemorrhage and anti-glomerular basement membrane (anti-GBM) antibodies. A 42-year-old woman was admitted to our hospital with complaints of exanthema, arthralgia, shortness of breath, and hemoptysis. Plain chest computed tomography (CT) scan revealed pericardial effusion, bilateral pleural effusions, and pulmonary hemorrhage. Read More

BMJ Case Rep 2018 May 2;2018. Epub 2018 May 2.

Pathology Department, Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal.

The antiglomerular basement membrane (anti-GBM) antibody disease is marked by the presence of specific antibodies against the non-collagenous domain of the type IV collagen's α3 chain. We describe a case of a 24-year-old Caucasian man, who may have had an atypical presentation of anti-GBM (slow progressive renal insufficiency, massive proteinuria and no detectable circulating anti-GBM antibody). The patient was treated with steroids and cyclophosphamide. Read More

Am J Physiol Renal Physiol 2018 Oct 2;315(4):F759-F768. Epub 2018 May 2.

Department of Medicine, Division of Renal Diseases and Hypertension.

Osteopontin (OPN) is a pro-and anti-inflammatory molecule that simultaneously attenuates oxidative stress. Both inflammation and oxidative stress play a role in the pathogenesis of glomerulonephritis and in the progression of kidney injury. Importantly, OPN is highly induced in nephritic kidneys. Read More

Eur J Immunol 2018 06 2;48(6):1082-1084. Epub 2018 May 2.

Laboratoire d'Immunologie, Pôle de Biologie, Centre Hospitalier Universitaire Grenoble Alpes, Grenoble, Cedex 9, France.

Detection of circulating anti-GBM antibodies has a key role for the diagnosis of Goodpasture syndrome but immunoassays using purified or recombinant alpha3(IV)NC1 as antigen do not recognize all anti-GBM antibodies. We show that anti-GBM antibodies directed against epitopes in their native conformation or cryptic epitopes are detected by indirect immunofluorescence. Read More

Zhonghua Nei Ke Za Zhi 2018 Mar;57(3):229-232

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.

A 56-year-old female was admitted to the Department of Rheumatology, Peking Union Medical College Hospital with complaint of recurrent fever and acute lumbar pain. Fever was complicated with malaise, cough and occasional blood-streaked sputum. Lab tests showed elevated white blood cell count, increased serum creatinine, erythrocyte sedimentation rate and C-reactive protein. Read More