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92 Cards in this Set
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Acute Nephritic Syndrome |
Usually presents with hypertension, hematuria, red blood cell casts, pyuria, protenuria |
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Acute Nephritic Syndrome |
Fall in GFR produces uremic symptoms with salt and water retention, leading to edema and hypertension |
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Post-Strep Glomerulonephritis |
prototypical for acute endocapillary proliferative glomerulonephritis |
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Post-Strep Glomerulonephritis |
usually affects children between 2 and 14 years, common in males |
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Post-Strep Glomerulonephritis |
skin and throat infections wiith particular M types of streptococci antedate this glomerular disease |
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Post-Strep Glomerulonephritis |
2-6 weeks after skin infection |
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Post-Strep Glomerulonephritis |
1-3 weeks after pharyngitis |
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Post-Strep Glomerulonephritis |
It is an immune-mediated disease invoving putative streptococcal antigens, immune complexes, compliment activation, cell mediated injury |
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Streptococcal pyrogenic exotoxin B |
generated by proteolysis of a zymogen precursor |
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Post-Strep Glomerulonephritis |
classic presentations are hematuria, pyuria, RBC casts, edema, hypertenion, oiguric renal failure |
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Post-Strep Glomerulonephritis |
Systemic symptoms are headache, malaise, anorexia, flank pain |
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Post-Strep Glomerulonephritis |
hypercelluarity of mesangial and endothelial cells |
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Post-Strep Glomerulonephritis |
glmerular infiltrates of polymorphonuclear leukocytes |
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Post-Strep Glomerulonephritis |
granular subenothelial immune deposits of IgG, IgM, C3, C4, and C5-9 |
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Subacute Bacterial Endocarditis |
subcapsular hemorrhages with a "flea-bitten" appearance |
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Subacute Bacterial Endocarditis |
focal proliferation around foci of necrosis |
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Subacute Bacterial Endocarditis |
mesangial, subendothelial, and subepithelial immune deposits of IgG, IgM and C3 |
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Subacute Bacterial Endocarditis |
Patients present with gross or microscopic hematuria, pyuria, mild proteinuria, less commonly, RPGN with rapid loss of renal function |
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Subacute Bacterial Endocarditis |
normocytic anemia, elevated erythrocyte sedimentation raete, hypocomplementemia, high titers of rheumatoid factor type III cryoglobulins, immune complexes |
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eradication of infection for 4-6 weeks |
primary treatment for Subacute Bacterial Endocarditis |
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Minimal Mesangial |
Class I Lupus Nephritis |
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Mesangial Proliferation |
Class II Lupus Nephritis |
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Proteinuria |
most common clinical sign of renal disease |
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Focal Nephritis |
Class III Lupus Nephritis |
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Diffuse nephritis |
Class IV Lupus Nephritis |
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Membranous nephritis |
Class V Lupus Nephritis |
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Sclerotic nephritis |
Class VI Lupus Nephritis |
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Mesangial Lesions |
mild to minimal clinical renal findings, may have active lupus serology, inactive urinary sediment, HTN infrequent |
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Focal Proliferative Lupus |
Active lupus serology, active urine sediment, hypertension |
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DIffuse Proliferative Lupus |
most active & severe clinical features, hypocomplementemia, high anti-DNA titers, very active urine sediment, impaired renal function |
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Membraous Lupus |
proteinuria, edema, mild serologic activity, hypertension, renal dysfunction acive urine sediment, high risk thromotic compplictions |
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End stage Lupus Nephritis |
result of years of lupus flares alterating with periods of inactivity, much of the renal histologic damage represents non-immunologic progression of diseas in remaining glomeruli, microhematuria, low level proteinuria, hypertension low GFR |
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High-steroids |
Induction of remission for Lupus Nephritis |
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Cyclophosphamide or mycophenolate mofetil |
Maintenance treatment in Lupus Nephritis |
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Goodpsture's syndrome |
pulmonary-renal syndrome, present with lung hemorrhage and glomerulonephritis |
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Anti-Glomerular Basement Membrane Disease |
explosive, with hemoptysis, a sudden fall in hemoglobin, fever, dyspnea, hematuria |
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Hemoptysis |
confined to smokers those who present with lung hemorrhage as a group do better than older |
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Anti-Glomerular Basement Membrane Disease |
focal or segmental necrosis, the later, with aggressive destruction of the capillaaries by cellular proliferation, leads to crescent formation in Bowman's space |
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Anti-Glomerular Basement Membrane Disease |
Non nephritic antibodies against the a1 NC1 domain are seen in paraneolastic syndromes and canot be discerned from assays that use whole BM fragments as the binding target |
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IgA Nephropathy |
clinical and laboratory evidence suggests close similarities between Henoch-Schonlen purpura |
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Henoch-Schonlein purpura |
distinguished clinically from IgA nephropathy prominent systemic symptoms, younger age, preceding infection, abdominal complaints |
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IgA Nephropathy |
deposits of IgA are also found in the glomerular mesangium in a variety of systemic disease |
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IgA Nephropathy |
recurrent episodes of macroscopic hematuria durig or immediately following an upper respiratory infection often accompanied by proteinuria |
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IgA Nephropathy |
persistent asymptomatic microscopic hematuria |
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ACE inhibitors |
treatment used in patients with proteinuria or declining renal function maybe useful in IgA Nephropathy |
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ANCA Small Vessel Vasculitis |
Granulomatosis with polyangiitis |
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ANCA Small Vessel Vasculitis |
Production of abnormal ANCA Abs that damage small veseels |
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Anti-proteinase 3 |
Granulomatosis with polyangiitis type of ANCA |
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Anti-myeloperoxidase |
microscopic polyangiitis & Churg-Strauss syndrome type of ANCA |
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Granulomatosis with Polyangiitis |
Fever, purulent rhinorrhea, nasal ulcers, sinus pain, polyarthralgia, cough, hemoptysis, shortness of breath, microscopic hematuria |
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Granulomatosis with Polyangiitis |
ches X-ray often reveals nodules and persistent infiltrates, sometimes with cavities |
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Granulomatosis with Polyangiitis |
Biopsy of involved tissue will show a small-vessel vasculitis and adjacent noncaseating granuomatosis |
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Granulomatosis with Polyangiitis |
Renal biopsies during active disease demonstrate segmental necrotizing glomerulonephritis without immune deposits |
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Microscopic Polyangiitis |
Present similar those with granulomatosis with polyangiitis except they rarely have significant lung disease or destrucitve sinusitis |
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Churg-Strauss Syndrome |
Associated with peripheral eosinophiliaa, cutaneous purpura, mononeuritis, asthma, allergic rhinitis |
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Churg-Strauss Syndrome |
with hypergammaglobulinemia, elevated levels of serum IgE, presence f rheumatoid factor sometimes accompanies allergic state |
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Churg-Strauss Syndrome |
Lung inflammation, fleeing cough, pulmonary infiltrates, precedes systemic mnifestations of disease by years |
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Churg-Strauss Syndrome |
small-vessel vasculitis and focal segmental necrotizing glomerulonephritis can be seen with usually absent eosinophils or granulomas |
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Membranoproliferative Glomerulonephritis |
immune-mediated glomerulonephritis characterized by thickening of the GBM with mesangioproliferative changes |
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Type I MPGN |
persistent hepatitis C infections, autoimmune diseases like lupus or cryoglobulinemia or neoplastic diseases |
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Type I MPGN |
most proliferative MGN |
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Type I MPGN |
shows mesangial proliferation with lobular segmenttion on renal biopsy and mesangial interposition between capillary basement membrane and endothelial cells |
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Type I MPGN |
producing a double countour sometimes called trans-tracking |
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Nephrotic Syndrome |
presents with heavy proteinuria, minimal hematuria, hypoalbuminemia, hypercholesterolemia, edema, hypertension |
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Minimal Change Disease |
sometimes known as nil lesion |
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Minimal Change Disease |
usually presents as a primary renal disease but can be associated with several other conditions |
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Minimal Change Disease |
there is an abrupt onset of edema and nephrotic syndrome accomplished by acellular urinary sediment |
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Minimal Change Disease |
shows no obvious glomerular lesion by light microscopy, negative for deposits by immunofluorescent microscopy |
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Minimal Change Disease |
electron microscopy shows an effacement of the foot process supporting the epithelial podocytes with weakening of slit-pore membranes |
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Primary responders |
are patients who have a complete remision after a single course of prednison |
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Steroid-dependent patients |
relapse as thheir steroid dose is tapered |
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Frequent relapsers |
have two or ore relapses in the 6 months following taper |
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Focal Segmental Glomeruloslerosis |
refers to a pattern of renal injury characterized by segmental glomerular scars that involve some but ot all glomeruli |
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Proteinuria |
clinical findings of FSGS largely manifest as |
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Focal Segmental Glomeruloslerosis |
can present with hematuria, hypertension, any level of proteinuria, or renal insufficiency |
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Membranous Glomerulonephritis |
it is the most common cause of nephrootic syndrome in the elderly |
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Membranous Glomerulonephritis |
Immunfluorescence demonstrates diffuse granular deposits of IgG and C3 |
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Membranous Glomerulonephritis |
Electron microscopy typically reveals electron-dense subepithelial deposits |
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Membranous Glomerulonephritis |
presence of subendothelial deposits or the presence of tubuloreticular inclusions strongly points to diagnosis |
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Diabetic Nephropathy |
is the single most commo cause of chronic renal failure |
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Diabetic Nephropathy |
Eosinophils PAS+ nodules |
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Diabetic Nephropathy |
with nodular glomerulosclerosis or Kimmelsteil-Wilson nodules |
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Diabetic Nephropathy |
immunofluorescence microscopy often reveals the the nonspecific deposition of IgG or compliment staining without immune deposits on elctron microscopy |
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Alport Syndrome |
Presence of hematuria, thinning and splitting of the GBMs, mild proteinuria |
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Alport Syndrome |
chronic glomerulosclerosis leading to renal failure in associatio with sensorineural deafness |
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Alport Syndrome |
some patients develop lenticonus of the anterior lens capsule, "dot and fleck" retinopathy |
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Alport Syndrome |
have thin basement membranes on renal biopsy, which thicken over time into multilamellations surrounding luscent areas |
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Alport Syndrome |
Tubules drop out, glomeruli scar, and the kidney succumbs to interstitial fibrosis |
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ACE inhibitors |
Primary treatment to control systemic hypertension and slow renal progression in Alport Syndrome |
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Hypertensive Nephrosclerosis |
Uncontrolled systemic hypetension causes permanent damage to the kidneys |
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Hypertensive Nephrosclerosis |
hemodynaamic stress of malignant hypertension leads to fibrinoid necrosis of small blood vessels, thrombotic microangiopathy, a nephritic urinalysis and acute renal failure |
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Hypertensive Nephrosclerosis |
Kidney biopsies with hypertensiion, microhematuria and moderate proteinuria demonstrate atherosclerosis, chronic nephrosclerosis and interstitial fibrosis without immune deposits |
