Myeloproliferative disorders (MPDs) are clonal disorders of the haematopoietic stem cells. These are classified as polycythaemia vera (PV), essential thrombocythaemia (ET), chronic myeloid leukaemia (CML) and myelofibrosis (MF) depending on the main haematopoietic lineage involved. Glomerulonephritis (GN) refers to a pattern of glomerular injury based on characteristic histopathology findings. ...

We report the first case of familial C3 glomerulonephritis (C3GN) associated with mutations in the gene for complement factor B (CFB). A 12-year-old girl was diagnosed with biopsy-proven C3GN. Her mother had a history of treatment for membranoproliferative glomerulonephritis, and her brother had hypocomplementemia without urinary abnormalities. DNA analysis revealed heterozygosity for CFB p.S36...

BACKGROUND IgG4-related disease is a novel disease entity characterized by elevated serum IgG4 and tissue infiltration by IgG4-positive plasma cells. Typical renal pathology is tubulointerstitial nephritis with storiform fibrosis, although the co-existence of various glomerular lesions has been described. Here, we present the first report of a case of IgG4-related kidney disease and membranopro...

Distinct patterns of glomerular lesions, including membranoproliferative glomerulonephritis and focal segmental glomerulosclerosis, are associated with infection by Schistosoma mansoni or Schistosoma japonicum. Evidence suggests that immune complex deposition is the main mechanism underlying the different forms of schistosomal glomerulonephritis and that immune complex deposition may be intensi...

We report two cases of Lyme disease-associated glomerulonephritis. A 57-year-old female presented with rash, volume overload, hypertension and rapidly progressive glomerulonephritis. Biopsy confirmed an immune complex-mediated, membranoproliferative lesion. She was treated successfully with steroids and antibiotics. In a second case, a 40-year-old male, with a previously known microscopic hemat...

A major shift in our understanding of the membranoproliferative glomerulonephritis (MPGN) lesion is the focus on which components of the complement pathway are involved in mediating renal injury. Hence, MPGN is no longer classified solely by ultrastructural findings on biopsy but instead divided into immune complex-mediated lesions versus complement-mediated lesions. This emphasis on complement...