The most common glomerular disease worldwide, often presenting with gross hematuria after URI.
What is IgA nephropathy?
Immunofluorescence shows “full house” pattern.
What is lupus nephritis?
This trial showed benefit of dapagliflozin in CKD patients, including those with IgA nephropathy.
What is DAPA-CKD?
First-line supportive therapy for all proteinuric glomerular diseases.
What is RAAS blockade (ACEi or ARB)?
DOUBLE JEOPARDY!!
These genetic variants are associated with high risk of FSGS in African ancestry.
What are APOL1 risk alleles?
This class of drugs, first-line for proteinuria, can cause hyperkalemia and acute kidney injury—especially when volume-depleted.
What are ACE inhibitors or ARBs?
Severe, diffuse podocyte foot process effacement causes this clinical syndrome.
What is nephrotic syndrome?
This disease presents with linear IgG staining on IF and anti-GBM antibodies in serum.
What is anti-GBM disease (Goodpasture's)?
This glomerular disease is characterized by thickened capillary loops on light microscopy, subepithelial immune complex deposits on EM, and granular IgG and C3 staining on immunofluorescence.
What is membranous nephropathy?
This large RCT found no benefit of plasma exchange in severe ANCA-associated vasculitis.
What is PEXIVAS?
Used for remission maintenance in ANCA vasculitis after induction.
What is rituximab or azathioprine?
Genetic or acquired dysregulation of this innate immune pathway is the key driver of C3 glomerulopathy.
What is the alternative complement pathway?
High cumulative doses of this alkylating agent can increase the risk of infertility and bladder cancer.
What is cyclophosphamide?
DOUBLE JEOPARDY!!
In AL amyloidosis, this light chain type is more commonly deposited, and is typically identified by immunofluorescence or mass spectrometry on kidney biopsy.
What is lambda light chain?
What is collapsing FSGS (or collapsing glomerulopathy)?
DOUBLE JEOPARDY!!
What are tubuloreticular inclusions?
This European trial in lupus nephritis demonstrated that a low-dose, short-course IV cyclophosphamide regimen was as effective as high-dose NIH protocol.
What is the Euro-Lupus Nephritis trial?
This corticosteroid, designed with a targeted-release formulation to act on gut-associated lymphoid tissue in the ileum, has been shown to reduce proteinuria and eGFR decline in patients with IgA nephropathy, as demonstrated in the NefIgArd trial.
What is budesonide?
Recent research has identified autoantibodies against this key slit diaphragm protein in a subset of adults with minimal change disease, suggesting an autoimmune mechanism.
What is nephrin?
This monoclonal antibody used in ANCA vasculitis and membranous nephropathy can cause late-onset neutropenia.
What is rituximab?
On kidney biopsy, this condition shows Congo red–positive deposits with apple-green birefringence under polarized light and randomly arranged non-branching fibrils ~10 nm on EM.
What is renal amyloidosis?
DOUBLE JEOPARDY!!
This is a putative target antigen in membranous nephropathy associated with autoimmune disorders and portends a better prognosis
What is exostosin 1/exostosin 2?
(will also accept neural cell adhesion molecule 1 (NCAM1) or Type III transforming growth factor beta receptor (TGFBR3)
What is Fabry's Disease?
This trial showed rituximab was non-inferior to cyclosporine at 12 months and superior to cyclosporine at 24 months in patients with membranous nephropathy.
What is MENTOR?
This oral C5a receptor antagonist was shown in the ADVOCATE trial to be non-inferior to prednisone for inducing remission in ANCA-associated vasculitis—while reducing glucocorticoid toxicity.
What is avacopan?
These two agents—one a dual ERA/ARB, the other a selective ETA receptor antagonist—target the endothelin-1 pathway to reduce proteinuria in conditions like IgA nephropathy.
What are sparsentan and atrasentan?
Inhibition of this angiogenic factor, often targeted in cancer therapy with agents like bevacizumab, can lead to glomerular diseases such as thrombotic microangiopathy and collapsing FSGS.
What is VEGF (vascular endothelial growth factor)?
Mutations in genes encoding the α3, α4, or α5 chains of type IV of collagen cause this hereditary glomerular disease characterized by thinning and splitting of the glomerular basement membrane and often secondary focal segmental glomerulosclerosis.
What is Alport's syndrome?
Both iptacopan and pegcetacoplan were recently approved by the FDA for this glomerular disorder.
What is C3 glomerulopathy?
This rare glomerular disease is characterized by organized microtubular deposits >30 nm in diameter on electron microscopy, often arranged in parallel arrays—distinguishing it from fibrillary GN.
What is immunotactoid glomerulopathy?
The phase 2 ENVISION trial studied this monoclonal antibody targeting APRIL, showing significantly greater reduction in proteinuria than placebo in patients with IgA nephropathy.
What is sibeprenlimab?
This anti-CD20 monoclonal antibody was shown to be more efficacious than standard therapy alone in active lupus nephritis.
What is obinutuzumab?
These two B-cell activating cytokines are implicated in the pathogenesis of IgA nephropathy by promoting Gd-IgA1 production—and are the targets of drugs like atacicept and telitacicept.
What are BAFF and APRIL?
Exposure to mercury-containing facial whitening cream has been linked to this form of membranous nephropathy, often seronegative for PLA2R antibody.
What is NELL-1-assoicated membranous nephropathy?
DOUBLE JEOPARDY!!
This heat shock protein has emerged as a highly specific immunohistochemical marker for fibrillary glomerulonephritis, helping distinguish it from amyloidosis and other mimickers.
What is DNAJB9?