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Renal > SM_205a: Renal Syndromes > Flashcards

SM_205a: Renal Syndromes Flashcards

1
Q

Describe the classification of renal syndromes

A
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2
Q

Dysmorphic RBCs indicate ______

A

Dysmorphic RBCs indicate glomerular bleeding

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3
Q

In a urine workup, _____, _____, and _____ are abnormal

A

In a urine workup, hematuria, proteinuria, and casts are abnormal

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4
Q

RBC cell casts indicate _______

A

RBC cell casts indicate glomerular hematuria (nephritic syndrome)

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5
Q

Lipid casts / oval body indicate ______

A

Lipid casts / oval body indicate nephrotic syndrome

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6
Q

If asymptomatic hematuria without proteinuria or other renal abnormalities, patient needs _____

A

If asymptomatic hematuria without proteinuria or other renal abnormalities, patient needs urological workup

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7
Q

If proteinuria is alone, first ______. If there is no obvious cause and significant proteinuria, patient needs ______

A

If proteinuria is alone, first quanitfy. If there is no obvious cause and significant proteinuria, patient needs renal workup

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8
Q

If hematuria + proteinuria, casts, or elevated serum creatinine, patient needs ______

A

If hematuria + proteinuria, casts, or elevated serum creatinine, patient needs renal workup

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9
Q

Purpose of a kidney biopsy is to ______ and ______

A

Purpose of a kidney biopsy is to confirm diagnosis and dictate management

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10
Q

Patients with _____, _____, _____, and _____ for which the cause is not obvious require a kidney biopsy

A

Patients with nephrotic syndrome, nephritic syndrome, AKI and kidney transplant for which the cause is not obvious require a kidney biopsy

  • Nephrotic syndrome: lot of proteinuria
  • Nephritic syndrome: results from glomerular inflammation and presents with hematuria
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11
Q

Describe the different ways of analyzing kidney biopsies

A

Analyzing kidney biopsies

  • Light microscopy: cell proliferation, GBM abnormalities
  • Immunofluorescence microscopy: immunocomplement / Ig staining
  • Electron microscopy: immune complex deposits, thickness of GBM, foot process effacement of podocytes
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12
Q

This is a _____ glomerulus

A

This is a normal glomerulus

  • No proliferation, crescent, or segmental lesions
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13
Q

Describe nephrotic syndrome

A

Nephrotic syndrome

  • Constellation of findings: proteinuria >3g/day, hypoalbuminemia, HLD, lipiduria, edema
  • Primary causes: minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy
  • Secondary causes: diabetes, amyloidosis
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14
Q

Describe minimal change disease

A

Minimal change disease

  • Most common cause of nephrotic syndrome in children
  • Due to secondary cause in adults (hematologic malignancy, medications)
  • Presents with sudden onset edema + other features of nephrotic syndrome sometimes following infection
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15
Q

Most common cause of nephrotic syndrome in kids is ______

A

Most common cause of nephrotic syndrome in kids is minimal change disease

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16
Q

Kidney biopsy of minimal change disease reveals _____, _____, and _____

A

Kidney biopsy of minimal change disease reveals normal-appearing glomeruli on LM, negative IF, and diffuse podocyte foot process effacement on EM

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17
Q

Minimal change disease is often responsive to treatment with _____

A

Minimal change disease is often responsive to treatment with steroids

18
Q

Describe focal segmental glomerulosclerosis

A

Focal segmental glomerulosclerosis

  • Most common cause of primary nephrotic syndrome in African Americans
  • Recently linked to APOL1 mutation
  • Often primary or idiopathic but can be secondary to other conditions
  • HTN, edema
  • Proteinuria ± hematuria
  • Serum creatinine normal or elevated
19
Q

_____ is most common cause of primary nephrotic syndrome in African Americans

A

Focal segmental glomerulosclerosis is most common cause of primary nephrotic syndrome in African Americans

20
Q

Describe kidney biopsy findings of focal segmental glomerulosclerosis

A

Kidney biopsy findings of focal segmental glomerulosclerosis

  • Focal: not all glomeruli involved
  • Segmental: only a portion of glomerulus involved
  • Need ≥ 1 lesions of segmental sclerosis on LM
  • Scant or non-specific staining of IgM, C3, or C1q on IF
  • Podocyte foot process effacement (focal or diffuse) on EM
21
Q

Describe treatment of focal segmental glomerulosclerosis

A

Treatment of focal segmental glomerulosclerosis (FSGS)

  • Challenging
  • Treat secondary cause (HIV, obesity, etc) if possible
  • If primary/idiopathic FSGS, start with steroids -> most do not respond -> try Calcineurin inhibitor or Cellcept
22
Q

Describe membranous nephropathy

A

Membranous nephropathy

  • Most common cause of primary nephrotic syndrome in Caucasian adults
  • Can be associated with solid tumor cancers, medication exposures (NSAIDs, Gold), infections (Hepatitis B or C), or autoimmune disease (SLE)
  • If no secondary cause, called primary
  • Onset occurs over days to weeks
23
Q

Describe kidney biopsy findings of membranous nephropathy

A

Kidney biopsy findings of membranous nephropathy

  • Thickened GBM on LM
  • “Spikes and holes” on silver stain
  • Granular IgG on IF
  • Subepithelial immune deposits on EM
    • PLA2R in primary disease
24
Q

Describe treatment of membranous nephropathy

A

Treatment of membranous nephropathy

  • Prompt treatment needed to avoid complications of the disease
  • Treatment of an existing secondary cause may cause resolution of membranous nephropathy
  • If primary depends on degree of proteinuria: low level treated with RAAS blockade, salt restriction, and diuresis; high level treated with immune modulation (cyclophosphamide, calcineurin inhibitor)
25
Q

Describe nephritic syndrome

A

Nephritic syndrome

  • Clinical sequelae of glomerular inflammation
  • Hematuria
  • Dysmorphic RBCs and/or RBC casts
  • Proteinuria (usually < 3g/day)
  • Varying degrees of renal insufficiency, HTN, and edema
  • Rapidly progressive glomerulonephritis (RPGN): glomerular inflammation that causes renal failure in weeks to months, crescentic glomerulonephritis on biopsy
26
Q

Rapidly progressive glomerulonephritis manifests as _____ on biopsy

A

Rapidly progressive glomerulonephritis manifests as crescentic glomerulonephritis on biopsy

27
Q

Three broad classifications of nephritic syndrome are _____, _____, and _____

A

Three broad classifications of nephritic syndrome are immune-complex mediated, pauci-immune, and anti-glomerular basement membrane disease

  • Immune-complex mediated: post-infectious GN, IgA nephropathy, lupus nephritis, membranoproliferative GN
  • Pauci-immune (ANCA positive): granulomatosis with polyangiitis, microscopic polyangiitis
  • Ant-glomerular basement membrane disease
28
Q

Describe anti-GBM disease

A

Anti-GBM disease

  • Pulmonary-renal syndrome
  • Auto-antibodies that bind to type IV collagen in the GBM
  • Goodpasture’s when lung involvement
  • Symptoms: malaise, weight loss, hemoptysis
  • Labs: hematuria, proteinuria, often rising Cr, + anti-GBM anitbody
  • Biopsy: diffuse crescentic glomerulonephritis on LM, classic linear IgG staining on IF
  • Treatment: steroids + cyclophosphamide + plasmapheresis (aggressive)
29
Q

Anti-GBM characteristic lab finding is ______ and characteristic biopsy finding is ______

A

Anti-GBM characteristic lab finding is + anti-GBM antibody and characteristic biopsy finding is linear IgG staining on IF

30
Q

Describe post-infectious glomerulonephritis

A

Post-infectious glomerulonephritis

  • Onset of nephritic syndrome 10-14 days after Strep pharyngitis or skin infection
  • Symptoms: gross hematuria, fever, malaise, HTN
  • Labs: elevated Cr, hematuria, proteinuria, low C3/C4, possibly +ASO titer
  • Biopsy: hypercellularity on LM, “starry sky” on IF, subepithelial humps on EM
  • Treatment: supportive (antibiotics for infection), steroids if severe to reduce inflammation
31
Q

Post-infectious glomerulonephritis manifests as _____ on IF and ______ on EM

A

Post-infectious glomerulonephritis manifests as starry sky on IF and epithelial humps on EM

32
Q

Describe lupus nephritis

A

Lupus nephritis

  • Prototypical immune-complex GN
  • Usually younger woman with known lupus
  • Symptoms: malaise, joint pain, rash, oral ulcers, serositis, edema
  • Labs: + ANA, + anti-dsDNA, low C3/C4, anemia
  • Urine: hematuria and/or proteinuria
  • Kidney biopsy required for diagnosis
  • Treatment: supportive for minimal inflammation, high dose steroids + immune-modulating drugs (cyclophosphamide + Cellcept) for active inflammation
33
Q

____ is required for diagnosis of lupus nephritis

A

Kidney biopsy is required for diagnosis of lupus nephritis

(6 classes)

34
Q

Describe IgA nephropathy

A

IgA nephropathy

  • Most common GN worldwide
  • Can be secondary to inflammatory bowel disease or cirrhosis
  • Symptoms: hematuria at the same time or 1-2 days after upper respiratory infection (or diarrheal illness)
  • Labs: Cr normal or elevated, C3/C4 normal, variable proteinuria
  • Biopsy: mesangial proliferation on LM and + IgA on IF
  • Treatment: ACE-i/ARB if mild, steroids or immunosuppressive therapy if high risk
35
Q

Kidney biopsy in IgA nephropathy shows _____ on LM and _____ on IF

A

Kidney biopsy in IgA nephropathy shows mesangial proliferation on LM and +IgA on IF

36
Q

Describe membranoproliferative glomerulonephritis

A

Membranoproliferative glomerulonephritis

  • Often associated with underlying infection (Hepatitis C)
  • Can also be related to genetic mutation (C3 nephritic factor) or be idiopathic
  • Presentation: nephritic or nephrotic features
  • Labs: Cr usually elevated, C3 may be low
  • Biopsy: lobular glomeruli on LM, tram-track appearance on Jones/silver stain, IF often shows C3 and IgG staining, various types of immune complex deposits on EM
  • Treatment: for infection of Hep-C associated, otherwise difficult
37
Q

Membranoproliferative glomerulonephritis manifests as _____ on LM and has a _____ appearance on Jones/silver stain

A

Membranoproliferative glomerulonephritis manifests as lobular glomeruli on LM and has a tram-track appearance on Jones/silver stain

(IF often shows C3 and IgG staining, various types of immune complex deposits on EM)

38
Q

Describe granulomatosis with polyangiitis (Wegener’s granulomatosis)

A

Granulomatosis with polyangiitis (Wegener’s granulomatosis)

  • Pulmonary-renal syndrome
  • Middle age or older individuals
  • Symptoms: constitutional, nasal or sinus (saddle nose deformity), palpable purpura
  • Labs: hematuria, proteinuria, elevated Cr, + c-ANCA, PR-3
  • Biopsy: necrotizing crescentic GN
  • Treatment depends on level of organ involvement/damage, often high-dose steroids + cyclophosphamide
39
Q

Describe microscopic polyangiitis

A

Microscopic polyangiitis

  • Pulmonary-renal syndrome
  • Middle-aged individuals
  • Symptoms: constitutional, lower respiratory tract (hemoptysis), palpable purpura
  • Labs: hematuria, proteinuria, elevated CR, + p-ANCA, MPO
  • Biopsy: necrotizing crescentic GN
  • Treatment: depends on level of organ involvement/damage, often high-dose steroids + cyclophosphamide
40
Q

Nephritic syndrome is the result of ______

A

Nephritic syndrome is the result of glomerular inflammation

Renal (31 decks)

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