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Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): A consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis
Criteria that centers can now use to define organ involvement and uniform response criteria for reporting outcomes in patients with light‐chain AL are reported.
Risk HLA-DQA1 and PLA(2)R1 alleles in idiopathic membranous nephropathy.
An HLA-DQA1 allele on chromosome 6p21 is most closely associated with idiopathic membranous nephropathy in persons of white ancestry and may facilitate an autoimmune response against targets such as variants of PLA2R1.
INF2 mutations in Charcot-Marie-Tooth disease with glomerulopathy.
INF2 mutations appear to cause many cases of FSGS-associated Charcot-Marie-Tooth neuropathy, showing that INF2 is involved in a disease affecting both the kidney glomerulus and the peripheral nervous system.
Immunoglobulin light (heavy)-chain deposition disease: from molecular medicine to pathophysiology-driven therapy.
Because these diseases represent a model of glomerular and interstitial fibrosis that is induced by a single molecule species, a better understanding of their pathomechanisms may help to unravel the pathophysiology of kidney fibrosis and renal disease progression.
Antiphospholipase A2 receptor antibody titer and subclass in idiopathic membranous nephropathy.
High agreement between IIFT and ELISA assessments of aPLA(2)R antibody titer and subclass is shown and the pathogenetic role of these antibodies, especially the IgG4 subclass, is highlighted, given the observed relationships between a PLA( 2)R titer, baseline proteinuria, and outcome.
Adult Faconi Syndrome Secondary to Light Chain Gammopathy: Clinicopathologic Heterogeneity and Unnsual Features in 11 Patients
Genetic and biochemical analyses of the light chains showed a striking homogeneity, which indicates that FS light chains are related by the sequence of their variable regions and Resistance of V kappa to proteolysis in FS patients can explain the accumulation of a light chain in the endocytotic compartment of the proximaltubule cells, leading to impairment of proximal tubule functions.
Rituximab for Severe Membranous Nephropathy: A 6-Month Trial with Extended Follow-Up.
The data suggest that PLA2R-Ab levels are early markers of rituximab effect and that addition of r ituximabs to NIAT does not affect safety.
PLA2R autoantibodies and PLA2R glomerular deposits in membranous nephropathy.
  • H. Debiec, P. Ronco
  • Medicine, Biology
    The New England journal of medicine
  • 16 February 2011
To the Editor: Membranous nephropathy is a common cause of the nephrotic syndrome in adults. Treatment is controversial and challenging because of the heterogeneity of the disease and a lack of
Rituximab treatment for severe steroid- or cyclosporine-dependent nephrotic syndrome: a multicentric series of 22 cases
RTX was effective in all patients when administered during a proteinuria-free period in association with other IS agents, and when relapses occurred, they were always associated with an increase in CD19 cell count.