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Nephritic Syndromes
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- 1What are the two CLINICAL categories of proliferative glomerular disease?1. Slow progressive glomerulonephritis--> Nephritic syndrome
2. Rapidly progressing glomerulonephritis (RPGN) - 2****Each clinical categorization of proliferative glomerular disease also has a histological categorization. What are the histological categorizations for non-RPGN and RPGN respectively?1. non-RPGN --> mesangial proliferative or focal proliferative diseases
2. RPGN --> DIFFUSE proliferative diseases --> may see crescents OR necrosis - 3Non-RPGN diseases are commonly IMMUNE COMPLEX diseases. What 3 disease processes are classified as non-RPGN?1. IgA Nephropathy
2. Lupus Nephritis
3. Post-Infectious glomerulonephritis (PIGN) - 4RPGN diseases can be split into 2 categories _____ immune - meaning immunofluorescence is negative and Linear Ig__ deposits.PAUCI
linear Ig-G deposits - 5Which RPGN is Pauci-immune?ANCA-associated vasculitis
- 6In ANCA vasculitis, there are circulating antibodies directed against PR3 and MPO. PR3 and MPO are antigens found on which type of blood cell?NEUTROPHILS
- 7ANCA vasculitis and Anti-GBM disease share the SAME presentation on light microscopy. Describe the presentation.Segmental GBM breaks
Necrosis
CRESCENTS
necrosis in small arteries - 8** ON EXAM ** There are 3 ANCA-vasculitis profiles based on the antigen being targetted. They are: - MPA - GPA - eGPA Describe the neutrophil antigen each targets, immunofluroscence pattern (p-ANCA or c-ANCA), renal and/or ENT involvement and whether or not it is granulomatous
- 9ANCA vasculitis is a serious condition that can progress to ESRF if left untreated. Who is most effected by ANCA-vasculitis?middle aged white adults
- 10Which RPGN has linear IgG deposits?Anti-GBM disease
- 11Anti-GBM disease is characterized by having autoantibodies againist the alpha-3 chain of type IV collagen. However, if a patient presents with pulmonary hemorrhage, this is called _____ disease.Goodpasture's disease
- 12Anti-GBM disease causes ____ vessel inflammation and _____ progressive GN.small, rapid
- 13What do you see on immunofluorescence in a patient with anti-GBM?Linear IgG
- 14Anti-GBM disease has a ______ age distribution whereas Goodpasture's is more common in ____ people. If left untreated, nearly all patients will progress to ESRF.BIMODAL (affects really young and really old)
YOUNG --> Goodpasture's - 15Lupus is a ______ autoimmune disease that effects the entire body. There are 6 histopathologic classes of lupus nephritis.SYSTEMIC
- 16***Lupus nephritis is characterized by DISTINCT patterns of injury caused by immune-complex depositions due to anti-_____ antibodiesds-DNA
- 17Unlike nephrotic syndrome, immune complexes are deposited in blood vessel walls and can, therefore, recruit ________.COMPLEMENT
- 18What are some of the signs and symptoms of systemic lupus?- fever
- arthritis
- depression/anxiety
- pain when taking a deep breath
- sun sensitivity
- Reynaud's
- hair loss
- recurrent sores around the mouth - 19Ig A nephropathy is very common in _____ populations. It is categorized by deposition of IgA in the _______.Asian
MESANGIUM - 20**** What is the classic presentation of IgA Nephropathy? When does it present?"synpharyngitic" recurrent gross hematuria
Presentation 1-5 days post-pharyngitis - 21Diabetic nephropathy and IgA nephropathy both have mesangial expansion, but what distinguished IgA?MATRIX EXPANSION and mesangial HYPERcellularity
recall: IgA = proliferative - 22If a patient has a Strep infection, what non-RPGN are they at risk of developing? What is the timeline?Post-infectious glomerulonephritis (PIGN)
1-3 weeks --> Strep pharyngitis infection
3-6 weeks --> strep cellulitis infection
TIMELINE IS WHAT DISTINGUISHES IgA from PIGN!! - 23In PIGN, strep A antigens deposit in the ______. This created ________ humps on light microscopy, as well as proliferation within podocytes.GBM
Subepithelial humps - 24What is membranoproliferative glomerulonephritis?A PATTERN OF INJURY
Includes:
- Mesangial hypercellularity
- endocapillary proliferation
- Double-contouring of the glomerular basement membrane
- lobulation - 25How are Nephritic Syndromes and RPGNs treated?Treatment is unique to each disease!!
But, generally
1. Steroids +/- immunosuppressant
2. Maintenance therapy (for ppl at risk of relapse) --> PROLONGED IMMUNOSUPPRESSION - 26*** What are some of the broad manifestations of vasculitis? Especially ENT conditions that can be seen in some of the ANCA profiles.ENT: nasal crusting, SINUSITIS, persistent rhinorrhea, purulent/bloody discharge, NASAL ulcers
PULM: hoarseness, COUGH, stridor, wheezing
Flu like symptoms - 27*****Since alpha-3 type IV collagen is also found in the ____, pulmonary involvement/pathology can be seen in anti-GBM disease.ALVEOLI
- 28****What is the definition of Nephritic Syndrome?PS: not everything that is hematuria is Nephritic Syndrome.... isolated hematuria is a thing on it's own
Nephritic Syndrome:
- glomerular inflammation that results in hematuria, variable degrees of proteinuria (which can sometimes be in the nephrotic range), and often renal impairment - 29If 10% of cases, IgA nephropathy can cause nephrotic syndrome!!!Just know this...
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- Descripción
- Renal Fichas sobre Nephritic Syndromes, creado por Manisha BHARADIA el 01/05/2019.
- Creado por
- Manisha BHARADIA
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