Glomerulonephritis And PCKD 🥤 Flashcards by Annie Gerlicher

Can nephritic and NephrOtic diseases overlap?

Yes

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What is the difference between focal and diffuse glomerular diease?

Focal: typically <50% of glomeruli are involved

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What is the difference between primary and secondary glomerular disease?

Primary: glomerular injury limited to kidney

Secondary: renal abnormalities result from a systemic disease

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What is the definition of glomerulonephritis

A term given to those diseaseS that present in the nephritic spectrum and usually signifies and inflammatory process causeing renal dysfunction

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What is the most common cause of glomerulonephritis?

Deposition of immune complexes in the glomerulus

antibody+antigen= immune complex

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What are the clinical findings of nephritic syndrome?

“Glomerular” hematuria- dysmorphic RBCs, RBC casts, cola/smoke colored

Proteinuria- less than <3.0g/day

Elevated creatinine

Oliguria

Edema

HTN

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What would you expect to see in the urine of someone with nephritic syndrome

Dysmorphic RBCs**** (Mickey mouse shaped)

RBC casts***

Cola/smoke colored

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What condition is the most severe and clinically urgent end of the nephritic spectrum?

Rapidly Progressive Glomerulonephritis

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What is Rapidly Progressive Glomerulonephritis?

Progressive loss of renal function over a comparatively short period of time

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What is the characteristic sign you will see when you look at the glomeruli in someone with Rapidly Progressive Glomerulonephritis?

Crescent Formation

🐝🐝

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What is a crescent formation?

Nonspecific response to severe injury to the glomerular capillary wall

Seen in Rapidly Progressive Glomerulonephritis

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Which one has hematuria: nephritic or nephrotic

Nephritic

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What are the 3 diseases that are listed under the nephritic spectrum on the chart she put in the slides a bunch of times

asymptomatic glomerular nephritis

Nephritic syndrome

Rapidly progressive glomerulonephritis

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So your patient comes in and says he’s pissing blood. What kind of findings would you expect if it was an extraglomerular source (aka NOT glomerular hematuria)

Red or Pink

Clots maybe

No proteinuria

Normal RBC morphology

No RBC casts

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Your patient is pissing blood. What findings would make you suspect a glomerular source?

Cola colored

No clots

Proteinuria maybe

RBCs are dysmorphic **🐝

RBC casts may be present

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What medication should you consider giving your patients with glomerulonephritis for antiproteinuric therapy

ACE/ARB

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Should we immediately hospitalize patients with glomerulonephritis

Yes for acute nephritic syndrome and RPGN

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What are the characteristic of nephritic syndrome that ms Herrick listed on her summary slide

Glomerular hematuria- RBC casts, dysmorphic RBCs

Proteinuria

Increased creatinine or decreased GFR

HTN

Edema

Cola colored urine

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What is the other name for IgA nephropathy that might not be on the test but might help you find YouTube videos

Berger Disease

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What is the most common cause of ~primary~ GN in the world

IgA nephropathy

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What age usually gets IgA nephropathy?

2nd and 3rd decades of life (young adults)

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What is the pathogenesis of IgA nephropathy

IgA complex deposits in the glomerular mesangium -> inflammatory response***

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Why will a patient come to see their PA if they have IgA Nephropathy

They will have an episode of Gross Hematuria a few days after a URI

(Remember the kidney damage is from the deposition of immune complexes in the mesangium)

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What end of the nephritic spectrum are patients with IgA Nephropathy going to be at

Can present anywhere along the spectrum

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How do you confirm the diagnosis of IgA Neprhopathy

Kidney biopsy (only done for severe disease)

What is the prognosis for IgA nephropathy

Spontaneous remission in 1/3 of pts Progression to ESRD in 20-40% Remaining may have chronic microscopic hematurua and stable creatinine

Do you treat every patient with IgA Nephropathy

Only the ones who have: Proteinuria >1g *** Decreased GFR HTN (The ones at higher risk of profressing to ESRD)

How do you treat IgA Nephropathy? (If you decided to treat)

ACE/ARB | Maybe glucocorticoids/immunosuppressive

What kind of infection leads to poststreptococcal GN?

group A beta-hemolytic strep lol Specifically pharyngitis or impetigo (strep skin infection)

Who is more likely to get post streptococcal GN?

males *twice as common

What is the pathophysiology of poststreptococcal GN?

Immune mediated: An immune complex containing a streptococcal antigen is deposited in glomerulus➡️complement activation and inflammation

How long after a GAS infection will post streptococcal GN present?

1-3 WEEKS after infection *** | IgA nephropathy was only 1-2 days after onset of URI sx

What will you find in the serum in post streptococcal GN?

Elevated streptococcal antibodies (ASO titers) Low complement

How do you treat post streptococcal GN?

Supportive care | Kids do better, and recurrence is rare

What is Anti-GBM disease?

Antibodies attack the glomerular basement membrane (GBM) (The target antigen is also found in the *alveolar basement membrane**** when antibodies attack those too it’s called GoodPasture)

What is the difference between Anti-GBM disease and Goodpasture Syndrome?

Anti-GBM: anti-GBM antibodies plus glomerulonephritis (KIDNEY ONLY) Goodpasture: glomerulonephritis AND Pulmonary hemorrhage (LUNG AND KIDNEY)

_____________accounts for 10-20% of patients with acute rapidly progressive GN

Anti-GBM disease

What age and genders get anti-GBM/Goodpasture

First peak: 30yo, males, more lung involvement Second peak: 60yo, females

What causes anti-GBM/Goodpasture?

Idiopathic usually

What pulmonary sx will you see in Goodpasture syndrome

Overt hemoptysis **pulmonary hemorrhage** Cough Dyspnea

How do you diagnose anti-GBM/Goodpasture syndrome

anti-GBM antibodies in serum/kidney biopsy ANCA will be positive in 40%*** (If they have anti-GBM ~and~ ANCA, it will help confirm your diagnosis)

What is the other name for Systemic Lupus Erythematosis (SLE) Nephritis

Lupus Nephritis

What kind of antibodies will be present in Lupus Nephritis?

Anti-ds DNA antibodies

What is the treatment for anti-GBM/Goodpasture

Urologist will do plasmapheresis and give immunosuppressants

What will initially make you suspect that your patient has Lupus Nephritis

Abnormal urinalysis High creatinine Confirmed with renal biopsy

What is the other name for IgA Vasculitis

Henoch-Schonlein Purpura (HSP)

What is the characteristic pathology of IgA Vasculitis (HSP)

Tissue deposition of IgA-containing immune complexes

Who is most likely to get IgA Vasculitis (HSP)

Children post URI

What is the CLASSIC TETRAD of IgA Vasculitis (HSP)

Rash (Palpable* purpura) Abdominal Pain Arthralgias Renal disease 🐝🐝🐝

Which kidney problems show up after other illnesses?

Poststreptococcal GN: 1-3 weeks post GAS IgA nephropathy: 1-2 days post URI IgA Vasculitis: post URI

How do you treat IgA Vasculitis (HSP)

Supportive care

Name that dx: 8 year old boy Red-brown urine this morning Sore throat resolved last week

Postinfectious GN

Name that dx: 20year old male Gross hematuria 2 days after URI

IgA Nephropathy | Due to age and 2 days post URI

What is Pauci-Immune Glomerulonephritis?

Systemic ANCA-associated Vasculitis (GPA, MPA, or EGPA) cause vascular and tissue damage. BUT there is an Absence or *paucity* of immune deposits on kidney biopsy

What are the systemic ANCA-associated vasculitides

Granulomatosis with polyangitis (GPA) Microscopic polyangitis (MPA) Eosinophilic GPA (EGPA)

What are the hallmark features (not symptoms) of GPA

Necrotizing granulomatous inflammation** Pauci-immune Vasculitis of small-medium vessels

What parts of the body are affected by GPA

Upper AND Lower respiratory tracts Kidneys

What are the *pulmonary* symptoms of GPA

Chronic sinusitis Saddle nose deformity Otitis media Ocular involvement Cough, dyspnea, hemoptysis

What are the *RENAL* manifestations of GPA

Glomerulonephritis- crecentic necrotizing glomerulonephritis** (RPGN)

What is the difference between MPA and GPA

MPA: Absence of granuloma formation UPPER respiratory tract is SPARED (no chronic sinusitis in MPA****)

When does GPA typically present vs MPA

GPA: 35-55yo MPA: 50yo

What is Eosinophilic GPA?

ANCA-associated Vasculitis that is associated with ***asthma and eosinophilia****🐝

What are the 3 disease phases of EGPA?

1. Prodrome- allergic disease (asthma/allergic rhinitis) 2. Eosinophilic/tissue infiltrative phase 3. Vasculitis- necrotizing Vasculitis of the small-medium vessels

Which Pauci-Immune GN do you think your patient has if they have asthma, are corticosteroid dependent and have nasal polyps

EGPA

How do you treat Pauci-Immune GN (GPA, MPA, and EGPA)

Immunosuppressants- corticosteroids and cytotoxic agents Referral to nephro/neuro/rheum/pulm etc

Of the 3 Pauci-Immune GNs, which ones have more C-ANCA and which ones have more P-ANCA

C-ANCA: GPA P-ANCA: MPA and EGPA

What kind of glomerulonephritis do patients with Anti-GBM usually present with

RPGN

What kind of glomerulonephritis do patients with Pauci-immune GN typically present with

RPGN

Name that dx: 30 yr old male Presents with acute RPGN Has GN + pulmonary hemorrhage +anti-GBM antibodies

Goodpasture syndrome

Name that dx: 50 year old male Hx of recurrent URIs and chronic sinusitis Presents with acute RPGN + C-ANCA

GPA

When are most simple kidney cysts found

Incidentally on ultrasound

You’re doing an ultrasound and WHOA you found a cyst on their kidney. What do you need to do now?

You need to do an ultrasound and a CT to differentiate it from malignancy, abscess, or polycystic kidney disease

What will you see on CT of a benign, harmless simple cyst

Smooth thin walls that are sharply demarcated Does NOT enhance with contrast*****

Do you get polycystic kidney disease from your parents?

Yes it can be autosomal dominant or autosomal recessive

What are the 2 types of polycystic kidney disease

Autosomal dominant (ADPKD) Autosomal recessive (ARPKD)

What is the most common genetic cause of CKD?

Autosomal Dominant PKD

Which two genes account for most cases of ADPKD

PKD1 PKD2

Does PKD affect one or both kidneys

ALWAYS BOTH

Is a “progressive decline in kidney function” seen in ADPKD, ARPKD, or both

Both

For most patients with ADPKD, renal function remains intact until the ________ decade of life

4th

What causes the decline of renal function in ADPKD

Cysts accumulate fluid ➡️ enlarge*➡️compress neighboring renal parenchyma➡️progressively compromising renal function

What is the mechanism that the kidneys use to try to compensate in ADPKD

“Hyperfiltration.”

What are the RENAL manifestations of ADPKD

HTN ***** might be presenting sx Abdominal/flank pain (big bois squishing) Palpable kidneys Hematuria +/- proteinuria Hx of UTIs and nephtolithisis

What are the EXTRArenal manifestions of ADPKD?

Intracranial aneurysms (“worst headache of my life”) Hepatic Cysts***** Heart valve probs (midsystolic click)

What is the Initial modality used for the screening and diagnosis of ADPKD?

Ultrasound ***

What is the management of ADPKD?

Strict BP control, low salt diet, statins🧂 Avoid caffeine? ☕️ Pain control Tolvaptan ***(vasopressin receptror antagonist) Dialysis/transplant

What is the only disease modifying medication for ADPKD/

Tolvaptan | ADH receptor antagonist- though that ADH may make it worse

Who gets Autosomal recessive PKD?

Infants and children (they are born with big kidneys and liver probs)

What gene is mutated in autosomal recessive PKD

PKHD1

Are the kidneys the only thing affected by ARPKD

No. Hepatobiliary tract is affected big time

What are the characteristic findings of ARPKD

Bilateral markedly enlarged kidneys HTN (in babies!) Congenital hepatic fibrosis Portal HTN (ascites in a baby!)

Which kind of PKD can be detected before birth on ultrasound after 24 weeks

ARPKD

What is the treatment for ARPKD?

One health