Glomerulonephritis Flashcards by Mina Sourial

hypo-complementemic GNs

lupus GN
PIGN
cryoglobulinemic GN
MPGN

How well did you know this?

Not at all

Perfectly

GNs with normal complements

IgA nephropathy
ANCA-associated GN
anti-GBM nephritis

How well did you know this?

Not at all

Perfectly

GNs with granular pattern on IF

lupus GN
PIGN
cryoglobulinemic GN
MPGN
membranous nephropathy
IgA nephropathy

How well did you know this?

Not at all

Perfectly

ONLY GN with LINEAR pattern on IF

anti-GBM nephritis

How well did you know this?

Not at all

Perfectly

ONLY GN with pauci-immune pattern on IF

ANCA-associated GN

How well did you know this?

Not at all

Perfectly

IgA nephropathy presentation

variable clinical presentation
asymptomatic hematuria with mild proteinuria
gross hematuria (sometimes with flank pain) within a FEW days of URI

How well did you know this?

Not at all

Perfectly

mechanism of AKI in IgA nephropathy

crescentic IgA nephropathy

- tubular obstruction from erythrocytes

How well did you know this?

Not at all

Perfectly

treatment of IgA nephropathy if proteinuria < 1 g/day

conservative therapy: ACEI/ARB, fish oil, vitamin d, and statin

How well did you know this?

Not at all

Perfectly

treatment of IgA nephropathy if proteinuria > 1 g/day

conservative therapy: ACEI/ARB, fish oil, vitamin d, and statin
IN ADDITION TO
steroids x 6 months
if crescentic (RPGN), ADD cyclophosphamide

How well did you know this?

Not at all

Perfectly

LN classification

normal glomeruli on LM
mesangial immune deposits on IF

class 1; minimal mesangial LN

How well did you know this?

Not at all

Perfectly

LN classification

purely mesangial hypercellularity of any degree or mesangial matrix expansion on LM
mesangial immune deposits

class 2; mesangial proliferative LN

How well did you know this?

Not at all

Perfectly

LN classification

active or inactive focal, segmental or global
endo- or extracapillary GN involving < 50% of all glomeruli
typically with focal subendothelial immune deposits, with or without mesangial alterations

class 3; focal LN

How well did you know this?

Not at all

Perfectly

LN classification

active or inactive focal, segmental or global
endo- or extracapillary GN involving > 50% of all glomeruli
typically with diffuse subendothelial immune deposits, with or without mesangial alterations
this class is subdivided into S (segmental) when 50% of involved glomeruli have segmental lesions, and G (global) when 50% of involved glomeruli have global lesions

class 4; diffuse LN

How well did you know this?

Not at all

Perfectly

LN classification
- global or segmental subepithelial immune deposits or their morphologic sequelae on LM and on IF or EM, with or without mesangial alterations

class 5; membranous LN

How well did you know this?

Not at all

Perfectly

histological prognostic features in LN

class 4 (diffuse proliferative LN) 
high activity and chronicity on biopsy
crescents
interstitial fibrosis
segmental necrotizing lesions

How well did you know this?

Not at all

Perfectly

clinical prognostic features in LN

HTN
anemia
high baseline Cr
high baseline proteinuria
delay in treatment

How well did you know this?

Not at all

Perfectly

epidemiologic prognostic features in LN

black race

- low socioeconomic status

How well did you know this?

Not at all

Perfectly

induction therapy of class 3 or class 4 LN with or without class 5 LN

steroids and cyclophosphamide (standard NIH protocol)
OR
steroids and MMF (noninferior to CP)

How well did you know this?

Not at all

Perfectly

maintenance therapy of class 3 or class 4 LN with or without class 5 LN

MMF

How well did you know this?

Not at all

Perfectly

ANCA GN clinical presentation

- kidneys

brown, tea-colored urine

How well did you know this?

Not at all

Perfectly

ANCA GN clinical presentation

- joints

pain and swelling

How well did you know this?

Not at all

Perfectly

ANCA GN clinical presentation

- trachea and lungs

cough (often mistaken for PNA)
hemoptysis
dyspnea

How well did you know this?

Not at all

Perfectly

ANCA GN clinical presentation

- skin

purpura
pruritus
hives
rash

How well did you know this?

Not at all

Perfectly

ANCA GN clinical presentation

- sinus/nose

rhinorrhea
nose pain
nasal congestion
epistaxis
crusting of nares (poor prognostic factor indicative of relapse)

How well did you know this?

Not at all

Perfectly

ANCA GN clinical presentation | - GIT

- abdominal pain | - hematochezia/melena

ANCA GN clinical presentation | - eyes

- eye pain - blurry vision - headache

ANCA GN clinical presentation | - ears

- hearing loss

ANCA GN clinical presentation | - neuro

foot drop; mononeuritis multiplex

MC presenting symptom of ANCA

"I just don't feel right"

vasculitis symptoms

flu-like symptoms including; - fever - body aches - poor appetite - weight loss

ANCA classification

- renal limited vasculitis - microscopic polyangiitis (MPA) - granulomatosis with polyangiitis (GPA) - eosinophilic granulomatosis with polyangiitis (EGPA)

MCC of RPGN, especially as patients get older

ANCA-associated GN

better way to classify ANCA

- renal-limited MPO-ANCA vasculitis - PR3-ANCA granulomatous vasculitis with lung  and renal involvement   - MPO-ANCA necrotizing vasculitis with  multi-organ involvement   - pauci-immune, necrotizing glomerulonephritis  in setting of positive MPO-ANCA

induction therapy for ANCA GN

- pulse steroids for everyone (taper at 8 weeks) - plasmapheresis (everyone with pulmonary hemorrhage or "severe" renal failure) - cyclophosphamide or rituximab

maintenance therapy for ANCA GN

- azathioprine OR - rituximab

goal to come off steroids in treatment of ANCA vasculitis by what time frame?

6 months

treatment for ANCA GN relapse

rituximab

"DOUBLE positive" ANCA serologies with very high titers for anti-MPO should raise suspicion for

drug-induced ANCA vasculitis

drugs that can cause lupus

- procainamide - hydralazine - minocycline - diltiazem - penicillamine - isoniazid - quinidine - anti-TNF alpha therapy - methyldopa

drugs that can cause ANCA

- hydralazine - levamisole (cocaine contaminant) - propylthiouracil (PTU) - minocycline

MCC of chronic GN worldwide

IgA nephropathy

other common cause of acute and chronic GN

crescentic GN on LM, and linear pattern on IF

anti-GBM nephritis

anti-GBM nephritis with lung involvement

Goodpasture's disease (pulmonary-renal involvement)

epidemiology of anti-GBM nephritis

- bimodal age and gender - young MEN in 2nd and 3rd decade - OLDER WOMEN in 6th and 7th decade

up to 1/3 of patients with anti-GBM nephritis have concurrent

ANCA, usually MPO

prognosis of anti-GBM

- very poor | - must be diagnosed and treated early

anti-GBM treatment

- plasmapheresis daily until anti-GBM Ab cleared - pulse steroids then taper over 6 months - cyclophosphamide (PO is better) x 3 months

does anti-GBM relapse?

very rarely; considered to be "one and done"

should you f/u anti-GBM after treatment and how?

- yes! | - anti-GBM titers

PIGN key clinical features

- occurs after acute infection - classic nephritic picture with microscopic or gross hematuria - AKI - proteinuria - HTN - edema - VERY LOW C3 - low/normal C4

PIGN is more common in

children

causative agents of PIGN

- Staphylococcus becoming more common | - Streptococcus

PIGN key LM findings

- diffuse ENDOcapillary proliferative GN | - crescents in severe cases

PIGN key IF findings

"STARRY SKY" pattern; GRANULAR deposits in capillary walls and mesangium with C3 staining +/- IgG

PIGN key EM findings

SUBepithelial hump-shaped deposits

classical complement pathway consumption is triggered by

immune complexes (Ag and Ab)

if classical complement pathway is triggered what is expected on IF?

C3 and Ab staining, such as IgG

if you ONLY see C3 staining on IF then you can assume what?

alternative complement pathway was triggered

- LM; mesangial proliferation with mesangial interposition and GBM duplication - IF; C3 with IgG and/or IgM, C1

MPGN type 1

- diverse glomerular histology w/ or w/o MPGN pattern | - C3 alone

C3 glomerulopathy (previously MPGN type 2)

types of C3 glomerulopathy

- C3 GN (MPGN type 1 pattern) - DDD - C3 GN (MPGN type 3 pattern)

- LM; mesangial proliferation with mesangial interposition and GBM duplication, usually with MEMBRANOUS features - IF; C3 with IgG and/or IgM, C1

MPGN type 3

classical complement pathway triggered by what conditions?

- infection - cancer - autoimmune disease - allergic reaction

alternative complement pathway HYPERACTIVITY triggered by

- mutations - auto-Abs - monoclonal gammopathy

possible treatment for C3 glomerulopathy that targets C5

eculizumab

name the 4 possible HCV related GNs

- type 1 MPGN a/w type 2 cryoglobulinemia - MPGN without cryoglobulinemia (probably false negative d/t difficulty checking cryoglobulins) - membranous nephropathy - FSGS, TMA a/w anti-cardiolipin Ab, fibrillary GN, and immunotactoid GN

how many types of cryoglobulins are there?

cryoglobulinemia - type 1 - what IGs and type?

MONOclonal IgG, IgM, or IgA

cryoglobulinemia - type 1 - a/w what diseases?

lymphoproliferative d/o's; MM, WM, CLL, B-cell NHL

cryoglobulinemia - type 2 - what IGs and type?

- MONOclonal IgM, IgM, or IgA with RF activity OR - POLYclonal IgG

cryoglobulinemia - type 2 - a/w what diseases?

- infections, mainly HCV - autoimmune d/o's - lymphoproliferative d/o's - rarely idiopathic

cryoglobulinemia - type 3 - what IGs and type?

POLYclonal mixed IGs (all isotypes) with RF activity from one polyclonal component (usually IgM)

cryoglobulinemia - type 3 - a/w what diseases?

- infections, mainly HCV - autoimmune d/o's - rarely idiopathic

RF is only positive in which types of cryoglobulinemia?

types 2 and 3

hallmark LM and EM findings of cryoglobulinemia

- LM = MPGN, nodular pattern | - EM = precipitating cryoglobulins in glomerulus

hallmark laboratory features of HCV-cryoglobulinemic GN

- variable proteinuria - hematuria - renal insufficiency - LOW C4, sometimes low C3 - RF positive (if type 2 or 3) - elevated transaminases in 70%

treatment of HCV-associated cryoglobulinemic MPGN - IF mild to moderate proteinuria - IF slow but progressive loss of kidney function - IF eGFR > 50

interferon + ribavirin +/- protease inhibitor

treatment of HCV-associated cryoglobulinemic MPGN - IF mild to moderate proteinuria - IF slow but progressive loss of kidney function - IF eGFR < 50

interferon ONLY

treatment of HCV-associated cryoglobulinemic MPGN - IF nephrotic range proteinuria - IF rapidly progressive loss of kidney function

- plasmapheresis x 2-3 weeks - rituximab x 4 weeks or CP x 2-4 months - pulse steroids +/- taper - after controlling acute vasculitis, treat HCV

definition of hemolytic-uremic syndrome

- microangiopathic hemolytic anemia (MAHA) - thrombocytopenia - AKI

expected laboratory findings of microangiopathic hemolytic anemia (MAHA)

- schistocytes on PBS - elevated LDH - low haptoglobin - elevated bilirubin

90% of HUS is typical, which means what?

- caused by Shiga-like toxin | - prodromal episode of diarrhea

10% of HUS is "atypical," which means what?

- heterogeneous d/o - NO diarrhea - NO Shiga toxin-producing E. coli infection

classification of atypical HUS

``` # familial # sporadic: - idiopathic - pregnancy-associated - HELLP syndrome - drug-induced - organ transplantation - HIV # cancer ```

most common protein affected by genetic abnormalities in patients with aHUS

factor H

treatment for aHUS

plasmapheresis then eculizumab

eculizumab works by targeting

Alport syndrome defect in

type 4 collagen α-5 chain gene (COL4A5)

most common GN inherited by X-linked pattern

Alport syndrome

can Alport syndrome be inherited by AR or AD pattern?

yes

clinical presentation of Alport syndrome

- microscopic hematuria - subnephrotic range proteinuria - declining renal function - sensorineural hearing loss - anterior lenticonus of eye

diagnosis of Alport syndrome if NO ESTABLISHED family history

renal biopsy

renal biopsy findings of Alport syndrome

EM required*** - GBM THICKENING - lamellation --> "BASKET-WEAVE" appearance

diagnosis of Alport syndrome if POSITIVE family history

GENETIC TESTING for α3, α4, and α5 chains of type 4 collagen

epidemiology of Alport syndrome

MALE predominance

thin basement membrane nephropathy renal biopsy findings

``` # EM required*** - diffuse GBM ATTENUATION (thinning) ```

epidemiology of thin basement membrane nephropathy

- familial (often), or sporadic | - no gender difference

thin basement membrane nephropathy presentation

- microscopic hematuria | - NORMAL renal function

thin basement membrane nephropathy AKA

benign familial hematuria

thin basement membrane nephropathy caused by

COL4A3 or COL4A4 gene mutation

GNs a/w subENDOthelial deposits

- MPGN type 1 - MPGN type 3 - cryoglobulinemia - LN class 3 - LN class 4

GNs a/w double contours (same list as GNs a/w subENDOthelial deposits + 2 more)

- MPGN type 1 - MPGN type 3 - cryoglobulinemia - LN class 3 - LN class 4 - TMA - chronic transplant glomerulopathy

diffuse thinning of GBM

thin basement membrane disease

irregularity and multilayering or splitting of the GBM

Alport’s syndrome

diffuse thickening of the GBM

- diabetic nephropathy - hypertensive nephrosclerosis - Alport's disease

mainly IgG LINEAR deposition in capillary walls

anti-GBM disease

nonspecific LINEAR IgG or "PSEUDOLINEAR" IgG deposition in capillary walls

diabetic nephropathy

GRANULAR parietal deposition of IGs and COMPLEMENT | - ***subENDOthelial***

MPGN type 1

GRANULAR parietal deposition of IGs and COMPLEMENT | - ***subEPIthelial***

PIGN

GRANULAR parietal deposition of IGs and COMPLEMENT | - ***subENDOthelial AND intramembranous***

membranous GN

what Ab in granulomatosis with polyangiitis ANCA?

anti-PR3 ANCA

what Ab eosinophilic granulomatosis with polyangiitis ANCA?

anti-MPO ANCA

ONLY GN with granular pattern on IF AND NORMAL complements

IgA nephropathy

GRANULAR parietal deposition of IGs and COMPLEMENT | - ***subENDOthelial, subEPIthelial, AND intramembranous***

MPGN type 3

"SAUSAGE-LIKE" (ribbon-like), osmiophilic deposits | - GBM deposits

DDD

GN a/w Drusen bodies in retina

DDD (C3 deposition in retina)

- MPGN pattern LM - negative IF - ***GBM deposits with ribbon-like pattern***

DDD

treatment for DDD

eculizumab

- MPGN pattern LM - negative IF - ***subENDOthelial AND mesangial deposits***

C3 GN

treatment for C3 GN

rituximab and plasmapheresis

Glomerulonephritis Flashcards

(121 cards)