BACKGROUND Mannose-binding lectin (MBL), a member of the collectin family, binds to various oligosaccharides and activates the classical pathway of complement independent from C1q. At present it is unknown whether this so-called lectin pathway of complement activation plays a role in the pathogenesis of human glomerulonephritis. METHODS Direct immunofluorescence of 84 renal biopsies using an ...

BACKGROUND Signal transducer and activator of transcription (STAT) 3 plays an important role in the regulation of cell proliferation. However, the mechanism of STAT3 activation in human glomerulonephritis is unclear. METHODS STAT3 activation was determined using immunohistochemistry for phosphorylated STAT3 (p-STAT3) in normal human kidney and various types of glomerulonephritis. We also iden...

A light, electron and immunofluorescence microscopy study was performed on 102 consecutive patients on whom suitable percutaneous renal biopsies were obtained. In this selected group of patients primary IgA glomerulonephritis was diagnosed in 6 (5.9%) cases. On light microscopy the glomerular lesions were predominantly focal (WHO class III) and diffuse mesangial proliferative glomerulonephritis...

IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis worldwide. Recently, there have been multiple advances in understanding IgAN pathophysiology and therapeutic options. Despite advent new treatment options, individual risk stratification disease course choosing best strategy for patient remains challenging. A multitude clinical trials ongoing, opening opportunities enr...

BACKGROUND Kidney transplantation confers superior outcomes for patients with end stage kidney disease, and live donor kidneys associate with superior outcomes compared to deceased donor kidneys. Modern immunosuppression has improved rejection rates and transplant survival and, as a result, recurrence of glomerulonephritis has emerged as a major cause of allograft loss. However, many glomerulon...

Iptacopan (LNP023) is a first-in-class, oral, low molecular weight, Factor B inhibitor being developed for the treatment of diseases associated with activation alternative complement pathway. Current Phase III studies iptacopan include paroxysmal nocturnal hemoglobinuria, C3 glomerulonephritis, IgA nephropathy, and atypical hemolytic uremic syndrome. Preclinical identified CYP2C8, OATP P-gp as ...

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 granula...

AIM To report a case of a patient with myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA)-negative microscopic polyangiitis (MPA) and IgA nephropathy associated with severe pulmonary haemorrhage. CASE REPORT A 59-year-old man presented with ANCA-negative systemic vasculitis accompanied by purpura, nephritis and pulmonary haemorrhage. A skin biopsy specimen revealed pandermal leucocyto...

Immunoglobulin A (IgA) vasculitis, formerly called Henoch-Schönlein purpura, is an immune complex vasculitis affecting small vessels with dominant IgA deposits. Clinical manifestations mainly involve cutaneous purpura, arthralgias and/or arthritis, acute enteritis and glomerulonephritis. IgA vasculitis is more common among children than adults, with more severe disease in adults. Gastrointestin...

IgA nephropathy is a chronic kidney disease defined by deposition of IgA in the glomeruli. An abnormality in the glycosylation of the hinge region of the IgA1 isotype of IgA is fundamental to the origins of this very common form of glomerulonephritis. In this issue of the JCI, Suzuki and coworkers describe the characteristics of IgG autoantibodies to the abnormally glycosylated IgA1 secreted by...