Anti-glomerular basement membrane (anti-GBM) nephritis is rare in childhood with few published cases. We report a 19-month-old boy with rapidly progressive glomerulonephritis (RPGN) due to anti-GBM nephritis. Treatment was started under 2 weeks after presentation and included plasma exchange, intravenous high-dose methylprednisolone, intravenous cyclophosphamide and mycophenolate as mainstay me...

Glomerular kidney diseases are of major public health importance because of their strong impact on the quality of life of patients and of their costly management. A relatively neglected area of study is the local factors that influence development of glomerular demolition. The involvement of a glomerular factor has been now demonstrated in glomerulonephritis with cell proliferation such as cres...

For over thirty-five years, available data suggested that therapeutic plasma exchange (TPE) was a useful treatment for patients with Rapidly Progressive Glomerulonephritis (RPGN) associated ANCA Associated Vasculitis (AAV) and elevated creatinine levels. The publication of the PEXIVAS study has challenged this conclusion. This perspective will outline history issue present our assessment curren...

Therapeutic plasma exchange (TPE) with hollow fiber modules is used in different severe diseases since more than 35 years. Based on many years of experience with the extracorporeal circulation in end-stage renal disease, the authors try to give an overview of therapeutic apheresis (TA) in renal diseases. The updated information on immunology and molecular biology of different renal diseases are...

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-ol...

Iyoda et al. have provided strong experimental evidence forbeneficial effects of PDGF signalling inhibition in two see-mingly unrelated glomerular diseases: rapidly progressive glo-merulonephritis (RPGN) in the present study and focal andsegmental glomerulosclerosis (FSGS) in a previous study.Novel insights into the pathogenesis of these two diseaseshave unravelled a com...

Goodpasture's syndrome is a rare autoimmune disorder characterized by rapidly progressive glomerulonephritis (RPGN) and alveolar hemorrhage in the presence of antiglomerular basement membrane (anti-GBM) antibodies. Central nervous system involvement is highly unusual in the absence of anti-neutrophil cytoplasmic antibodies. We report the case of a 20-year-old man with RPGN accompanied by bloody...

BACKGROUND Antineutrophil cytoplasmic antibody (ANCA)-associated crescentic glomerulonephritis (CGN) is a major cause of rapidly progressive glomerulonephritis (RPGN). ANCA-associated CGN is generally classified into pauci-immune RPGN, in which there are few or no immune complexes. CASE PRESENTATION A 78-year-old man presented with RPGN after a 7-year course of chronic proteinuria and hematur...

Abstract Background and Aims Rapidly progressive glomerulonephritis/crescentic glomerulonephritis (RPGN/cGN) may compromise renal function in the short or medium term thus evolving into acute kidney injury (AKI) chronic disease. Innate adaptive immune responses are placed at epicentre of cGN. However, molecular pathways involved activation these by far less known. Sting is an innate DNA-sensing...

The relationship between brucellosis and p-antinuclear cytoplasmic antibody (p-ANCA)-associated vasculitis (pAAV) is a rare condition. Herein, we report a 52-year-old man who was diagnosed as rapidly progressive glomerulonephritis (RPGN) and endocarditis due to brucellosis. He was treated with antimicrobial agents, steroids, plasmapheresis, renal replacement therapy and aortic valve replacement...