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Clin Med II Unit III > 2 Glomerulonephritis & Cystic Diseases > Flashcards

2 Glomerulonephritis & Cystic Diseases Flashcards

1
Q

The basic filtering unit of the kidney

A

Glomerulus

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2
Q

Glomerular disease is any damage to the major components of the glomerulus, with includes…

A

Podocytes

Glomerular Basement Membrane (GBM)

Capillary endothelium

Mesangium

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3
Q

What are the two classifications of glomerular disease?

A

Nephritic (ie glomerulonephritis)

Nephrotic

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4
Q

Glomerular disease is considered to be focal when…

A

<50% of glomeruli are involved

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5
Q

Glomerular injury that is limited to the kidney

A

Primary glomerular disease

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6
Q

Renal abnormalities resulting from a systemic disease

A

Secondary glomerular disease

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7
Q

Diseases that present in the nephritic spectrum and usually signify an inflammatory process —> renal dysfunction

A

Glomerulonephritis (GN)

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8
Q

What is the etiology of glomerulonephritis?

A

Most often, the cause is related to the deposition of IMMUNE COMPLEXES in the glomerulus

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9
Q

In the US, glomerulonephritis comprises ______% of all cases of ESRD

A

25-30%

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10
Q

What are the three diagnoses on the Nephritic spectrum (from least to most severe)?

A

Asymptomatic glomerular hematuria

Nephritic syndrome

Rapidly progressive glomerulonephritis

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11
Q

Microscopic or macroscopic hematuria with or without proteinuria (<1 g/d)

A

Asymptomatic glomerular hematuria

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12
Q

Acute kidney injury with proteinuria of 1-3 g/d, hematuria, RBC CASTS, edema, and hypertension

A

Nephritic syndrome

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13
Q

Acute kidney injury with proteinuria of 1-3 g/d, hematuria, RBC casts, and systemic symptoms

A

Rapidly progressive glomerulonephritis

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14
Q

All of the conditions on the nephritic spectrum can lead to …

A

Chronic glomerular disease (chronic kidney disease w/ or w/o hematuria, proteinuria, HTN, late-stage GN)

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15
Q

What is the hallmark urine appearance for someone with Nephritic syndrome?

A

Smokey or cola-colored urine

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16
Q

What are the hallmark “glomerular hematuria” findings

A

Dysmorphic RBCs (MICKEY MOUSE RBCs)

RBC casts

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17
Q

________ on urine microscopy is pathognomic for nephritic syndrome

A

RBC casts

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18
Q

Proteinuria in nephritic syndrome is usually

A

Subnephrotic (<3g/day)

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19
Q

What will the patient’s creatinine look like in nephritic syndrome?

A

Elevated (acute decrease in GFR)

B/c decreased kidney function

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20
Q

Besides hematuria, mild proteinuria, and elevated creatinine, what other clinical findings suggest nephritic syndrome

A

Oliguria (decreased urine output)

Edema (periorbital, peripheral - less prominent than in nephrotic syndrome)

HTN

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21
Q

Most severe and clinically urgent end of the nephritic spectrum

A

Rapidly Progressive Glomerulonephritis

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

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22
Q

Characteristic morphologic finding that you can appreciate on biopsy in cases of Rapidly Progressive Glomerulonephritis

A

Crescent formation

Nonspecific response to severe injury to the glomerular capillary wall

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23
Q

Major causes of hematuria in younger patients

A

Transient/unexplained

UTI****

Stones

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24
Q

Major causes of hematuria in older patients

A

CANCER***
(Bladder, kidney, prostate)

BPH

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25
Q

Distinguishing extraglomerular from glomerular hematuria:

Color

A

Extra: Red or pink

Glomerular: “cola-colored”

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26
Q

Distinguishing extraglomerular from glomerular hematuria:

Clots

A

Extra: May be present

Glomerular: Absent

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27
Q

Distinguishing extraglomerular from glomerular hematuria:

Proteinuria

A

Extra: Usually absent

Glomerular: May be present (<3g/day)

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28
Q

Distinguishing extraglomerular from glomerular hematuria:

RBC morphology

A

Extra: Normal

Glomerular: Dysmorphic (mickey mouse)

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29
Q

Distinguishing extraglomerular from glomerular hematuria:

RBC casts

A

Extra: Absent

Glomerular: May be present

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30
Q

How do you workup a suspected case of glomerulonephritis

A

Urine dipstick: looking for protein and blood

Urine microscopy: looking for RBCs, WBCs, RBC casts

Serologic testing: 
• Creatinine (GFR)
• ANA and anti-DS DNA Ab
• Complement
• ANCA (C-ANCA and P-ANCA)
• Anti-GBM antibodies
• Antistreptolysin O titer

Renal biopsy

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31
Q

Management of glomerulonephritis is dependent upon…

A

The underlying cause

Management of complications (HTN, fluid overload)

ACE/ARB - consider for antiproteinuric therapy

Nephrology referral or consult
• Immunosuppressive meds
• Possibly dialysis if profound AKI

Acute nephritic syndrome or RPGN warrants immediate hospitalization

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32
Q

What are the possible types of GN that will show a positive ANCA?

A

If no extra-renal disease —> ANCA-associated crescentic GN

If systemic necrotizing vasculitis —> Microscopic polyangitis

If respiratory necrotizing granulomas —> Granulomatosis with polyangitis

If asthma and eosinophilia —> Eosinophilic granulomatosis with polyangitis

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33
Q

What are the different types of GN that can occur with anti-glomerular basement membrane (GBM) antibodies?

A

If there is lung hemorrhage —> Goodpasture syndrome

If not —> Anti-GBM GN

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34
Q

Glomerulonephritis with (+) antinuclear antibodies

A

Lupus GN

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35
Q

Glomerulonephritis with (+) Antipathogen antibodies

A

Postinfectious or peri-infectious GN

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36
Q

Glomerulonephritis with (+) IgA

A

IgA nephropathy

37
Q

Glomerulonephritis with (+) Cryoglobulins

A

Cryoglobulinemic GN

38
Q

Glomerulonephritis with (+) complement activation

A

MPGN

39
Q

The most common cause of primary Glomerulonephritis in the world

A

IgA Nephropathy

Primary is most common but can also be secondary (cirrhosis, celiac, infectious)

Inciting cause is unknown but susceptibility to IgA nephropathy seems to be inheritable

40
Q

Greatest frequency of IgA nephropathy is in what populations?

A

Asians and Caucasians

Peak occurrence in 2nd and 3rd decades of life

Males > Females

41
Q

What is the pathogenesis of IgA Nephropathy?

A

Immune complex-mediated GN

IgA deposition in the glomerular mesangium —> inflammatory response

42
Q

An episode of gross hematuria, usually following a UTI (symptoms often 1-2 days after illness onset)

A

IgA Nephropathy

Can present anywhere along the nephritic spectrum (rarely nephrotic)

43
Q

How is IgA nephropathy diagnosed?

A

Clinical suspicious and laboratory data

Confirmed by kidney biopsy (usually performed for more severe or progressive disease)

44
Q

What is the spectrum of disease course that IgA Nephropathy might take?

A

Spontaneous clinical remission in ~ 1/3 of patients

Progression to ESRD occurs in 20-40% of patients

Remaining may have chronic microscopic hematuria and stable creatinine

45
Q

Which IgA Nephropathy patients should be given an ACE or ARB?

A

Those wit Proteinuria > 1/day, decreased GFR, or HTN

Higher Risk

46
Q

Besides ACE/ARB, why other treatments have been tried in IgA Nephropathy?

A

Glucocorticoids +/- immunosuppressive agents

47
Q

What is the cause of Poststreptococcal GN?

A

Infection with nephritogenic strains of group A beta-hemolytic strep - either Pharyngitis or impetigo

More common in children

Twice as common in males than females

48
Q

What is the pathophysiology of poststreptococcal GN?

A

Immune-mediated

An immune complex containing a streptococcal antigen is deposited in the affected glomeruli

Triggers COMPLEMENT activation and INFLAMMATION

49
Q

What are the clinical findings in post-strep GN?

A

Presentation varies across the nephritic spectrum, with symptoms 1-3 WEEKS AFTER INFECTION

Elevated ASO TITERS

Serum complement levels are LOW

50
Q

How do you diagnose PSGN?

A

Clinical findings of acute nephritis with documentation of recent GAS infection

51
Q

How do you treat PSGN?

A

Supportive care - resolution often begins within the first two weeks

Prognosis: depends on severity and age of patient

CHILDREN are more likely to fully recover (>90%)

Recurrent episodes are rare

52
Q

Acute RPGN in which circulating antibodies directed against the GBM

A

Anti-GBM Disease

Accounts for 10-20% of patients with acute RPGN

53
Q

Why does anti-GBM disease affect both the kidney and the lung?

A

Target antigen is also found in the ALVEOLAR BASEMENT MEMBRANE

54
Q

What does the case distribution of anti-GBM disease look like?

A

Bimodal distribution

First peak ~age 30 (slight male predominance), more lung involvement

Second peak ~age 60 (female predominance)

55
Q

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

A

Anti-GBM Disease is the antibodies +GN

Goodpasture is GN + pulmonary hemorrhage

56
Q

Clinical manifestations of anti-GBM disease?

A

Often IDIOPATHIC but some genetic predisposition (HLA)

Associations with PULMONARY INFECTION, TOBACCO USE, HYDROCARBON EXPOSURE

Renal involvement = Nephritic spectrum, including RPGN

Pulmonary involvement = Cough, dyspnea, sometimes overt hemoptysis

57
Q

How is anti-GBM disease diagnosed?

A

Demonstration of anti-GBM antibodies in serum or kidney biopsy

Test for antineutrophil cytoplasmic antibodies (ANCA)
• Up to 40% will also test positive for ANCA (having both can alter course of treatment/prognosis)

58
Q

How is anti-GBM disease treated?

A

Plasmapheresis
Immunosuppressive agents

SEND TO NEPHROLOGY

59
Q

Immune complex mediated glomerular disease with anti-ds DNA antibodies

A

Lupus Nephritis

Many possible patterns of injury, most cases present within the nephritic spectrum

Incidence higher in nonwhites

60
Q

How is Lupus Nephritis diagnosed?

A

Often suspected by an abnormal UA and/or elevation of serum creatinine

Dx confirmed with renal biopsy

Treatment varies with each class —> nephrology/rheum consult

61
Q

Henoch-Schoenlein Purpura is another name for …

A

IgA Vasculitis

Immune-mediated vasculitis characterized by tissue deposition of IgA-containing immune complexes

62
Q

When might you see IgA Vasculitis?

A

Most common in children, M>F

Cause is unknown but often post-URI (strep)

63
Q

Classic tetrad for IgA Vasculitis/Henoch-Schoenlein Purpura

A

Rash (palpable purpura - but normal platelets)

Arthralgias

Abdominal pain

Renal disease

64
Q

IgA vasculitis is more common in kids but _______ is more common in adults

A

Progressive renal disease

Signs of renal involvement typicall appear AFTER the rash —> may present with nephritic or nephrotic syndrome

65
Q

What is the treatment for IgA Vasculitis?

A

Often self-limited

Supportive care, symptomatic therapy, and targeted therapy to decrease complications

66
Q

Pauci-Immune Glomerulonephritis is a ___________ vasculitis

A

ANCA-associated vasculitis

67
Q

Pauci-Immune GN is caused for what systemic ANCA-associated vasculitides?

A

Granulomatosis with polyangiitis (GPA)

Microscopic polyangiitis (MPA)

Eosinophilic granulomatosis with polyangiitis (EGPA)

All have similar features on renal histology (absence or paucity of immune deposits on kidney biopsy)

68
Q

What are the two versions of ANCA?

A

P-ANCA and C-ANCA

These antibodies produce vascular and tissue damage

69
Q

What is granulomatosis with polyangiitis (GPA)?

A

ANCA-associated vasculitis

Rare, complex, multisystem autoimmune disease of unknown etiology

70
Q

Hallmark features of Granulomatosis with Polyangiitis

A

NECROTIZING GRANULOMATOUS INFLAMMATION mostly in lungs and kidneys

Pauci-immune vasculitis of small-medium vessels

Upper Respiratory System: chronic sinusitis, SADDLE NOSE DEFORMITY, otitis media, ocular involvement, cough, dyspnea, hemoptysis

Renal manifestations: CRESCENTIC NECROTIZING GLOMERULONEPHRITIS

May also present with fever, malaise, weight lost, arthritis, skin manifestations, polyneuropathy

Typically presents between 35-55

71
Q

How is Microscopic Polyangiitis (MPA) different from GPA?

A

ABSENCE OF GRANULOMATOSIS FORMATION and sparing of the UPPER respiratory tract (so no chronic sinusitis, saddle nose)

Age of onset is ~50 (a little older than GPA)

72
Q

ANCA-associated vasculitis associated with asthma and eosinophilia

A

Eosinophilic granulomatosis with polyangiitis (EGPA)

Lung, skin, heart, renal, GI, and neuro symptoms

73
Q

What are the three disease phases for Eosinophilic Granulomatosis with Polyangiitis?

A

Prodrome phase - allergic disease (asthma or allergic rhinitis)

Eosinophilic/tissue infiltrative phase

Necrotizing vasculitis of the small-medium vessels

74
Q

How are Pauci-Immune GNs treated?

A

Consultation/referral to multiple specialists

Immunosuppressants (corticosteroids and cytotoxic agents)

Without treatment, prognosis is poor

75
Q

C-ANCA is more common with ___________ and P-ANCA more common with ____________.

A

C-ANCA = GPA

P-ANCE = MPA, EGPA

76
Q

________ account for 65-70% of all renal masses

A

Simple Cysts

Typically asymptomatic - most found incidentally on ultrasound

77
Q

What is the diagnostic objective for simple cysts?

A

To differentiate them from malignancy, abscess, or polycystic kidney disease

Use U/S and CT

Benign features: smooth, thin walls that are sharply demarcated, does not enhance with contrast

78
Q

Simple cysts do not enhance with contrast. If something DOES enhance with contrast, it’s more likely to be…

A

Renal cell carcinoma

79
Q

Inherited disease that causes an irreversible decline in kidney function

A

Polycystic Kidney Disease (PKD)

Can be either autosomal dominant or recessive

All racial and ethnic groups affected, M=F

80
Q

Majority of individuals with PKD eventually require ___________.

A

Renal replacement therapy (either dialysis or kidney transplant)

81
Q

Most common genetic cause of CKD

A

Autosomal Dominant PKD

Variable penetrate, accounts for 10% of patients on dialysis in the US

(+) FHx in 75% of cases

82
Q

What are the two genes that account for most cases of ADPKD?

A

PKD1 and PKD2

83
Q

ADPKD is characterized by …

A

Massive kidney enlargement due to BILATERAL progressive increase in the number of cysts

Progressive decline in renal function (GFR)

For most patients, renal function remains intact until 4th decade of life

Upt o 50% will have ESRD by age 60 years

84
Q

What are the renal manifestations of ADPKD?

A 
HTN in >50%
Abdominal/flank pain
Palpable kidneys
Hematuria
\+/- proteinuria
Hx of UTIs and nephrolithiasis
85
Q

What are extrarenal manifestations of ADPKD?

A

Intracranial aneurysms

Hepatic cysts (40-50%)

Pancreatic cysts, splenic cysts, etc

CVD (ie MITRAL VALVE PROLAPSE)

86
Q

Initial modality for screening and diagnosis of ADPKD

A

Ultrasound

CT/MRI helpful when U/S unclear

Typical findings - large kidneys and extensive cysts scattered throughout both kidneys

Genetic testing for definitive diagnosis

87
Q

How do you manage a patient with ADPKD?

A

Strict BP control, low-salt diet, statins (to prevent CVD)
Avoid caffeine
Supportive therapy
TOLVAPTAN (vasopressin receptor antagonist - helps slow progression of disease)
Dialysis or kidney transplant

88
Q

Autosomal Recessive PKD primarily affects…

A

Infant’s and children’s kidneys and hepatobiliary tract

Characterized by BILATERAL markedly enlarged kidneys, progressive renal impairment, and CONGENITAL HEPATIC FIBROSIS (portal HTN)

May have feeding difficulties and growth impairment

89
Q

How is autosomal recessive PKD diagnosed and treated?

A

Can be detected by routine antenatal U/S after 24 weeks gestation

Supportive therapy with a multidisciplinary team

Clin Med II Unit III (6 decks)

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