Glomerular Disease 1 (Regner) Flashcards
What are the most common etiologies of glomerular diseases?
By far, **idiopathic **or immune. This may be deposition of immune complexes, direct antibody action against GBM/glomerular antigens, or chronic inflammation.
What is meant by a “subepithelial” deposition?
Which glomerular disease is this associated with?
In the context of “subepithelial” glomerular deposition, the podocytes act as the “epithelium”, deposition is just beneath them.
Subepithelial deposition is typical of membranous nephropathy (“Spike & Dome”)
Distinguish between the descriptors of diffuse, focal, global and segmental in the context of glomerular disease.
Diffuse/focal refer to the ennumerative glomerular involvement (all or some).
Global/segmental refer to how much of a glomerulus is involved (all or some).
Describe the major and minor signs of nephrotic syndrome.
Major: Proteinuria (>3.5g/day), hypoalbuminemia, edema, hyperlipidemia.
Minor: Lipiduria, hypercoagulability.
Why are edema, hyperlipidemia, and hypercoagulability seen in nephrotic syndromes?
Edema: Loss of albumin & other proteins that contribute to plasma π.
Hyperlipidemia: Response by the liver to the “thinning” of blood.
Hypercoagulability: Loss of proteins C, S; anti-thrombin 3.
Describe the features seen in nephritic syndromes.
Mild proteinuria (<3.5g/day)
Hematuria (often with RBC casts and/or dysmorphic RBCs)
Hypertension
Edema
Which glomerular diseases tend to blur the lines between nephrotic and nephritic pathophysiologies?
Membranoproliferative GN, IgA nephropathy.
IgA Nephropathy
Describe its epidemiology.
What are its clinical features?
What is revealed on histology?
IgA Nephropathy
Most common GN worldwide, usually younger patients.
Prominent hematuria, with dysuria and loin pain, often synpharyngitic. Sometimes subclinical. HTN in severe cases.
Mesangial hypercellularity, granular mesangial IgA deposits.
IgA Nephropathy
How is it treated?
Describe its prognosis.
IgA Nephropathy
Fish oil to slow progression, ACE-I for HTN, steroids/immunosuppressants.
Prognosis is mixed, may slowly develop kidney disease/failure.
What is the pathophysiology of Henoch-Schonlein Purpura?
Describe its clinical presentation.
IgA deposition in multiple organs.
Skin: Non-blanching purpura in lower body.
Joint arthralgias.
GI tract: Pain, vomiting, melena/hematochezia
IgA nephropathy.
Post-Strep Glomerulonephritis
Describe its epidemiology.
What are its clinical features?
What is revealed on histology?
Post-Strep Glomerulonephritis
Typically affects young patients following pharyngeal or skin GAS infection.
Sudden onset hematuria, HTN, oliguria, azotemia and periorbital edema. Labs reveal low C3 and high ASO titers.
Mesangial/endocapillary proliferation with neutrophils. Granular IF deposits in mesangium & subepithelial (“hump-like”)
Post-Strep Glomerulonephritis
How is it treated?
Describe its prognosis.
Post-Strep Glomerulonephritis
Supportive treatment only.
95% of children recover; adults don’t do as well. Small progression to renal failure or RPGN.
What are the classic signs of a Rapidly Progressive Glomerulonephritis?
Renal failure occurring within weeks. Biopsy shows crescents comprised of fibrin and macrophages.
Goodpasture’s Syndrome
Describe its epidemiology.
What are its clinical features?
What is revealed on histology?
Goodpasture’s Syndrome
Mostly affects younger adult men.
Rapidly Progressive GNitis with pulmonary involvement (hemoptysis, pulmonary infiltrates)
Linear IgG/C3 on kidney biopsy IF.
Goodpasture’s Syndrome
Describe its etiology.
Is there always pulmonary involvement?
How is it treated?
Goodpasture’s Syndrome
Antibodies directed against GBM, probably a3chain of Type IV collagen.
Goodpasture’s implies pulmonary involvement. If not present, this is termed “Anti-GBM disease”
Plasmapheresis, prednisone/cytoxan.
