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ABIM > Nephron-Glomerular Disease > Flashcards

Nephron-Glomerular Disease Flashcards

1
Q

What are the two ways that glomerular disease presents?

A

1-Nephrotic syndrome

2-Nephritic syndrome

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2
Q

What is nephrotic syndrome?

A

Urine protein excretion >3500mg/24h or a urine protein-creatinine ratio of >3500 mg/g that may be accompanied by hypoalbuminemia, edema, and HLD

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3
Q

What is the most common cause of nephrotic syndrome? What is the most common cause of IDIOPATHIC nephrotic syndrome in adults?

A

Diabetes mellitus

Idiopathic: Membranous glomerulopathy and FSGS

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4
Q

What is nephritic syndrome?

A

glomerular inflammation with evidence of hematuria, variable proteinuria, and sometimes leukocytes in the urine sediment–edema, hypertension, kidney failure

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5
Q

What is the role of serum complement with glomerulonephritis?

A

It helps differentiate the underlying etiology of GN. Levels are typically normal in anti-glomerular basement membranes disease and pauci-immune GN but are LOW in immune complex GN (with the exception of IgA nephropathy)

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6
Q

What race is FSGS most common in?

A

black persons

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7
Q

What is treatment and prognosis for FSGS?

A

Spontaneous remission only occurs in a small number of patients. Therefore treatment is indicated in most patients.

  • Treat with glucocorticoids or calcineurin inhibitors at time of presentation or for relapsing disease.
  • If FSGS is due to a drug or disease, then treat disease or remove drug
  • Remission on treatment suggests a good clinical course. Some may need a transplant…
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8
Q

What diseases is FSGS associated with?

A

HIV and heroin use

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9
Q

In patients with membranous glomerulopathy, what are they at increased risk for?

A

They are at increased risk for thromboembolic events–particularly renal vein thrombosis. Malignancy can precipitate MG as well!

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10
Q

What is the treatment and prognosis for MG?

A
  • Idiopathic MG that does not subside after 6-12 months or someone has a thromboembolic event, treat with steroids, calcineurin inhibitors, cyclophosphamide
  • If refractory to this… give MMF, rituximab
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11
Q

What are the calcineurin inhibitors

A

Cyclosporine and tacrolimus

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12
Q

What are some secondary causes of MG?

A

SLE, hep B and C, NSAIDS, TNF-a inhibitors
Malignancies
Thyroid disease

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13
Q

Which of the nephrotic syndromes has a higher risk for renal vein thrombosis?

A

membranous glomeruopathy

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14
Q

How are these three diagnosed on renal biopsy and EM:

-

A

FSGS: Segmental scars in some glomeruli, on EM you see visceral epithelial foot process effacement but NO IMMUNE DEPOSITS

MG: Glomerular capillary loops that appear thickened. EM and IMMUNOFLUORESCENCE show immune deposits and PLA2R antibodies

MCD: normal glomeruli on light and immunofluorescence, on EM the GBM is normal with extensive effacement of epithelial foot processes

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15
Q

How is nephrotic syndrome from DM diagnosed?

A

proteinuria with diagnosis of diabetes

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16
Q

How should MCD be treated

A

Patients usually respond to glucocorticoids, but if relapsing or refractory–cyclophosphamide, calcineurin inhibitors, MMF, rituximab

17
Q

How should diabetic nephropathy be treated?

A

ACE inhibitors or ARBs

18
Q

What are the most common causes of glomerulonephritis?

A

Pauci-immune and IgA nephropathy (immune complex mediated)

19
Q

Describe the clinical course and epi of RPGN

A

–associated with anti-GBM antibodies and pauci-immune small vessel vasculitis

*goes to advanced kidney failure very quickly

20
Q

Diagnosis of RPGN

A

DEFINITIVE:
*kidney biopsy with glomerular crescents associated with inflammation of glomerular capillaries–specific diagnosis is Ade with immunofluorescence microscopy (immune complexes, linear anti-GBM, pauci-immune)

SUPPORTIVE:
*serology: +ANCA, systemic vasculitis or anti-GBM antibodies

*chest imaging may show diffuse infiltrates in a pt with pulmonary hemorrhage or nodules in the presence of granulomatosis with polyangiitis (Wegeners)

21
Q

Kidney biopsy findings

  • RPGN
  • Infectious GN
  • Anti-GBM
  • Pauci Immune
  • IgA Nephropathy
A
  • RPGN: glomerular crescents associated with inflammation of glomerular capillaries–specific diagnosis is Ade with immunofluorescence microscopy (immune complexes, linear anti-GBM, pauci-immune)
  • Infectious: Kidney biopsy: diffuse endocapillary proliferative and exudative GN, and rarely crescents on light microscopy, immunofluorescence microscopy reveals C3-dominant or C3 codominant glomerular straining
  • Anti-GBM: proliferative GN often with many crescents, EM with immunofluoresence for anti-GBM
  • Pauci-immune; Kidney biopsy: absent or minimal staining with immunoglobulin (hence pauci-immune)
  • *Spectrum of abnormalities range from mild focal and segmental GN to a diffuse necrotizing GN

*IgA Nephropathy: kidney biopsy shows mesangial proliferation with IgA deposits

22
Q

Clinical manifestations of Anti-GBM antibody disease

A

kidney function deteriorates over days to weeks, pts with pulmonary hemorrhage may have life threatening respiratory failure with diffuse alveolar infiltrates on chest radiograph

23
Q

Diagnosis of anti-GBM disease

A

Nephritic syndrome

  • Serology: normal complement and elevated anti-GBM antibody
  • Kidney bx: proliferative GN often with many crescents
  • EM: There are linear deposits along the GBM by immunofluorescence
24
Q

Treatment of RPGN

A

1st: IV pulse steroids followed by oral glucocorticoids
2nd: cyclophosphamide or rituximab may be needed
3rd: Plasmapheresis may be indicated in the presence of pulmonary hemorrhage or severe kidney failure to remove circulating antibody

25
Q

Treatment of anti-GBM disease

A

Steroids, cyclophosphamide and daily plasmapheresis to remove circulating anti-GBM

26
Q

Clinical manifestations of PAUCI-immune GN

A
  • Most people have circulating ANCA
  • Associated with granulomatosis with polyangiitis, granulomas associated with necrotizing vasculitis, microscopic polyangiitis
  • wide range of minimal symptoms with hematuria to RPGN
  • leukocytoclastic vasculitis resulting palpable purpura, as well as pulmonary disease
  • Granulomatous lesions on biopsy, particularly for GPA: saddle nose tissue destruction, tracheal stenosis, hearing loss
  • If eosinophilic granulomatosis wit polyangiitis, then patients have a history of asthma, pulmonary infiltrates, and eosinophilia
27
Q

Diagnosis of Pauci-Immune GN

A
  • most positive for ANCA
  • specifically, GPA is pr3-ANCA
  • specifically, MPA is myeloperoxidase-ANCA
  • *Kidney biopsy: absent or minimal staining with immunoglobulin (hence pauci-immune)
  • *Spectrum of abnormalities range from mild focal and segmental GN to a diffuse necrotizing GN
28
Q

Treatment and prognosis of Pauci-Immune

A

ACUTE: glucocorticoids with cyclophosphamide with or without plasmapheresis

MAINTENANCE: azathioprine, MMF, or MTX

29
Q

Ig A vasculitis–Diagnosis

A

Leukocytoclastic vasculitis or kidney biopsy shows mesangial proliferation with IgA deposits

30
Q

Lupus nephritis–clinical symptoms

A

Extrarenal symptoms of SLE with active lupus serologies (ANA antibodies, ds-DNA antibodies) and low complement levels

31
Q

Lupus nephritis–treatment and prognosis

A

Treatment is primarily directed at treating the class of lupus. Class I and II LN require aggressive combination immunosuppressive therapy

32
Q

Diagnosis of lupus nephritis

A

ANA antibodies, anti-DS DNA antibodies usually positie

C3 and C4 is usually depressed

Kidney biopsy required to make the diagnosis and classify the lesions of LN to guide treatment

33
Q

Classification of Lupus nephritis: I-VI

A

I-no renal findings

II-mild clinical kidney disease, minimally active urine sediment, mild to moderate proteinuria, active serology

III-focal proliferative LN with <50% glomeruli involvement, increased proteinuria, may have hypertension and some active lesions may require treatment

IV- Diffuse proliferative LN >50% glomeruli involvement–active urine sediment, HTN, heavy proteinuria reduced GFR, active serology, active lesions requiring treatment

V-membranous LN GN-significant proteinuria (often nephrotic) with less active serology

VI-Advanced sclerosing LN-more than 90% glomerulosclerosis; no treatment prevents kidney failure

34
Q

Infection-related GN–clinical manifestations

A

Acute nephritis syndrome, occurs after infection/illness after a latent period of 1-6 weeks

Staphylococcus GN usually is associated with ongoing infection at the time of development of nephritis

35
Q

Diagnosis of infectious GN

A

Patients are nephritic and have an ongoing or preceding infection

Search for infection using microbiologic cultures usually shows the inciting organism in nonstreptococcal GN

  • Depressed complement
  • Post-strep GN, depressed complement levels, usually C3, signifying activation of the complement pathway…. in PSGN antibodies to streptococcal antigens (antistreptolysin-O, anti-DNAse B) are detected in almost all cases

*Kidney biopsy: diffuse endocapillary proliferative and exudative GN, and rarely crescents on light microscopy, immunofluorescence microscopy reveals C3-dominant or C3 codominant glomerular straining

36
Q

What are cryoglobulins?

A

Cryoglobulins are immune-related proteins that precipitate at temps below 37C in vitro and may be associated with a systemic inflammatory syndrome involving small to medium vessel vasculitis

37
Q

Describe amyloid

A

Amyloid consists of randomly oriented fibrils composed of various proteins that form organized beta-pleated sheets within tissues

38
Q

How is amyloid seen on biopsy?

A

Stains with apple green or Congo red under a polarizing microscope or EM

39
Q

Describe the overall picture with cryoglobulinemia? What disease processes does it lead to? How diagnosed?

A

Waldenstrom’s macroglobulinemia
Myeloma
Hepatitis C
Connective tissue disease

Range of clinical spectrum from mild proteinuria and hematuria to RPGN, low complement, positive RF

ABIM (58 decks)

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