Acute Nephritic Syndrome Flashcards
Acute nephritic syndromes classically present with _____
hypertension, hematuria, red blood cell casts, pyuria, and mild to moderate proteinuria.
[PSGN] kin and more commonly throat infections with particular ___(nephritogenic strains) antedate glomerular disease.
M types of streptococci
Poststreptococcal glomerulonephritis due to ___ develops 1–3 weeks after infection and 2–6 weeks after ____.
pharyngitis
impetigo
[PSGN] Renal biopsy findings:
The renal biopsy in poststreptococcal glomerulonephritis demonstrates hypercellularity of mesangial and endothelial cells; glomerular infiltrates of polymorphonuclear leukocytes; granular subendothelial immune deposits of IgG, IgM, C 3, C4 , and C5–9 ; and subepithelial deposits (which appear as “humps”)
P Polymorphonuclear
S Subepithelia humps
G granular immediately deposits
N eNdothelial and mesangial hypercellularity
[PSGN]
Complement and Immune Findings:
Depressed CH50 and low C3 (90% cases)
Normal C4 levels.
Rheumatoid factor (30–40%), cryoglobulins, ANCA (10%).
Streptococcal markers:
ASO (30%), anti-DNAse (70%), antihyaluronidase (40%).
What is the most common immunologic finding associated with active lupus nephritis?
A. Elevated C3 and C4
B. Low C3 and C4
C. Positive p-ANCA
D. Positive anti-Ro/SSA
B
Which of the following immune complexes plays a central role in the pathogenesis of lupus nephritis?
A. DNA-anti-DNA complexes
B. C3-C4 complexes
C. Rheumatoid factor complexes
D. Anti-Smith antigen complexes
A
In lupus nephritis, which histologic lesion is characterized by normal glomeruli on light microscopy but mesangial immune deposits on immunofluorescence?
A. Class I
B. Class II
C. Class III
D. Class IV
A
Class I nephritis describes normal glomerular h tology by normal light microscopy with minimal mesangial deposits on immunofluorescent or electron microscopy.
Class II designates mesangial immune complexes with mesangial proliferation. B
A patient with lupus nephritis presents with >50% of glomeruli showing diffuse endocapillary proliferation and numerous crescents. What is the likely classification?
A. Class III
B. Class IV-G
C. Class IV-S
D. Class V
B
What is the most common presenting clinical feature of lupus nephritis?
A. Hypertension
B. Hematuria
C. Proteinuria
D. Edema
C
In which class of lupus nephritis is nephrotic syndrome most likely to be present?
A. Class II
B. Class III
C. Class IV
D. Class V
D
Class 4 nephrotic range proteinuria >50% of patients
Which of the following treatments is first-line for inducing remission in Class IV lupus nephritis?
A. Hydroxychloroquine alone
B. Low-dose prednisone
C. High-dose steroids + Cyclophosphamide/Mycophenolate Mofetil
D. Rituximab + Methotrexate
C
Current evidence suggests that inducing a remission with administration of high-dose steroids and either cyclophosphamide or mycophenolate mofetil for 2–6 months, followed by maintenance therapy with lower doses of steroids and mycophenolate mofetil or azathioprine,
What is the hallmark histologic feature of Class V lupus nephritis?
A. Mesangial proliferation
B. Subepithelial immune deposits
C. Crescent formation
D. Fibrinoid necrosis
B
A. Class II
Which of the following therapies should not be used for a patient of childbearing age with lupus nephritis unless fertility preservation has been addressed?
A. Mycophenolate mofetil
B. Cyclophosphamide
C. Azathioprine
D. Hydroxychloroquine
B
What laboratory marker correlates best with lupus nephritis activity?
A. Anti-Smith antibodies
B. Anti-dsDNA antibodies
C. Anti-RNP antibodies
D. Rheumatoid factor
B
A patient with lupus nephritis has >90% sclerotic glomeruli with interstitial fibrosis. Which histologic class does this indicate?
A. Class IV-G
B. Class V
C. Class VI
D. Class II
C
[Lupus Nephritis] Which class?
normal glomerular histology by normal light microscopy with minimal mesangial deposits on immunofluorescent or electron microscopy.
I
[Lupus Nephritis] Which class?
Mesangial Proliferation
II
[Lupus Nephritis] Which class?
Focal lesions involving <50% of the glomeruli with proliferation or scarring,
III
[Lupus Nephritis] Which class?
Diffuse lesions with >50% of the glomeruli involved and proliferative endocapillary lesions with or without extracapillary lesions that may be segmental (IV-S), involving <50% of the glomerular tuft, or global (IV-G), involving >50%
IV
[Lupus Nephritis] Patients with _____ on biopsy often have a rapidly progressive decline in renal function
crescents
[Lupus Nephritis] Which class?
Nephrotic range proteinuria
Class IV
[Lupus Nephritis] Which class?
Nephrotic Syndrome
Class V
[Lupus Nephritis]
Class IV
Induce remission with administration of?
High-dose steroids and either cyclophosphamide or mycophenolate mofetil for 2–6 months, followed by maintenance therapy with lower doses of steroids and mycophenolate mofetil or azathioprine
[Lupus Nephritis] Which class?
subepithelial immune deposits p ing a membranous pattern
V
[Lupus Nephritis] Which class?
Renal Venin thrombosis and other thrombotic complications
V
[Lupus Nephritis] Which class?
> 90% sclerotic glomeruli and ESRD with interstitial fibrosis
VI
Renal transplantation in renal failure from lupus, usually performed after____ of inactive disease, results in allograft survival rates comparable to patients transplanted for other reasons.
~6 months
[anti GBM disease]
When they present with lung hemorrhage and glomerulonephritis, they have a pulmonary-renal syndrome called ___
Goodpasture’s syndrome.
[anti GBM disease]
The target epitopes for this disease is ____
α3 NC1 domain of collagen IV
Goodpasture’s syndrome in two age groups. Which age group present with full Goodpasture’s syndrome with hemoptysis, a sudden fall in hemoglobin, fever, dyspnea, and hematuria,
Younger men in late 20s
Goodpasture’s syndrome in two age groups. Which age group present with isolated glomerulonephritis
men and women in 60s
[anti-GBM disease] Renal biopsy
Renal biopsies typically show focal or segmental necrosis that later, with aggressive destruction of the capillaries by cellular proliferation, leads to crescent formation in Bowman’s space
[antiGBM disease] Prognosis at presentation is worse if there are _____ on renal biopsy with advanced fibrosis, if serum creatinine is >5–6 mg/dL, if oliguria is present, or if there is a need for acute dialysis.
> 50% crescents
Which of the following best describes the antigenic target in antiglomerular basement membrane (anti-GBM) disease?
A. α1 NC1 domain of collagen IV
B. α3 NC1 domain of collagen IV
C. Collagen III fibrils
D. Laminin-beta chains
B
Which of the following clinical presentations is most likely in a young patient with Goodpasture’s syndrome?
A. Isolated hematuria without other symptoms
B. Progressive dyspnea and hemoptysis
C. Polyuria and nocturia
D. Painless jaundice and fatigue
B
What is the most important histologic finding on kidney biopsy in patients with anti-GBM disease?
A. Mesangial proliferation
B. Subepithelial immune deposits
C. Linear IgG staining along the GBM
D. Fibrinoid necrosis of the arterioles
C
: Which of the following best describes the expected renal biopsy findings in advanced anti-GBM disease?
A. Diffuse mesangial immune deposits with hyaline casts
B. Segmental necrosis with crescents in Bowman’s space
C. Proliferative glomerulonephritis with neutrophilic infiltrates
D. Thrombotic microangiopathy with glomerular thrombosis
B
Which of the following factors most strongly predicts a poor prognosis in anti-GBM disease?
A. Serum anti-GBM titers of any level
B. Low C3 and C4 complement levels
C. >50% crescent formation on renal biopsy
D. Persistent proteinuria of >500 mg/day
C
After successful treatment of anti-GBM disease, how long should kidney transplantation be delayed?
A. 1 month after serum antibody titers normalize
B. 3 months after completion of immunosuppressive therapy
C. 6 months after serum anti-GBM antibodies become undetectable
D. Immediate transplant after resolution of hemoptysis
C
It is classically characterized by episodic hematuria associated with the deposition of IgA in the mesangium.
IgA Nephropathy
Henoch-Schönlein purpura is distinguished clinically from IgA nephropathy by ______
prominent systemic symptoms, a younger age (<20 years old), preceding infection, and abdominal complaints
[IgA Nephropathy] The pathognomonic finding on kidney biopsy is dominant or codominant ____
mesangial IgA deposits, either alone or with IgG, IgM, or C 3 .
The two most common presentations of IgA nephropathy are ____
-Recurrent Macroscopic Hematuria
-Persistent Asymptomatic Microscopic Hematuria:
[IgA Nephropathy]
Cumulatively, risk factors for the loss of renal function identified thus far account for <50% of the variation in observed outcome but include the presence of _____
hypertension or proteinuria, the absence of episodes of macroscopic hematuria, male sex, and older age of onset.
Which of the following clinical presentations is most commonly associated with IgA nephropathy?
A. Persistent painless hematuria 2 weeks after a sore throat
B. Episodic macroscopic hematuria concurrent with an upper respiratory infection
C. Progressive swelling and proteinuria without hematuria
D. Acute kidney injury after a recent urinary tract infection
B
The pathognomonic finding on kidney biopsy is dominant or codominant mesangial IgA deposits, either alone or with IgG, IgM, or C
IgA Nephropathy
Abnormalities in the ______ of the hinge region of primarily polymeric IgA1 seem to best account for the pathogenesis of sporadic IgA nephropathy.
O glycosylation
Which treatment strategy is first-line for a patient with proteinuria and declining renal function due to IgA nephropathy?
A. Plasmapheresis
B. Cyclophosphamide and prednisone
C. Angiotensin-converting enzyme (ACE) inhibitors
D. Hydroxychloroquine
C
Which of the following clinical features is least likely to be associated with IgA nephropathy?
A. Rapidly progressive glomerulonephritis (RPGN)
B. Nephrotic syndrome
C. Episodic hematuria during a respiratory infection
D. Persistent microscopic hematuria
B
[ANCA Small-Vessel Vasculitis]
Autoantibodies against neutrophil cytoplasmic antigens (ANCA):
Anti-proteinase 3 (PR3): Common in _____
granulomatosis with polyangiitis (GPA)
[ANCA Small-Vessel Vasculitis]
Autoantibodies against neutrophil cytoplasmic antigens (ANCA):
Anti-myeloperoxidase (MPO): Common in ____
microscopic polyangiitis (MPA) and Churg-Strauss syndrome (EGPA).
[ANCA Small-Vessel Vasculitis]
Induction therapy usually includes _____ and either ____
glucocorticoids
cyclophosphamide or rituximab.
Biopsy of involved tissue will show a small-vessel vasculitis and adjacent noncaseating granulomas.
Granulomatosis with Polyangitis
The disease is more common in patients exposed to silica dust and those with α 1 -antitrypsin deficiency, which is an inhibitor of PR3.
Granulomatosis with Polyangitis
Biopsy without granulomas
Microscopic Polyangitis
Which of the following ANCA antibodies is most commonly associated with Granulomatosis with Polyangiitis (GPA)?
A. MPO-ANCA (p-ANCA)
B. PR3-ANCA (c-ANCA)
C. Anti-GBM antibody
D. ANA antibody
B
Which biopsy finding is most characteristic of microscopic polyangiitis (MPA)?
A. Granulomatous inflammation with necrosis
B. Pauci-immune necrotizing vasculitis without granulomas
C. Subepithelial immune deposits in the glomeruli
D. Linear IgG staining along the basement membrane
B
A patient presents with hematuria, hemoptysis, nasal ulcers, and a chest x-ray showing nodular cavitary lesions. Which of the following conditions is most likely?
A. Granulomatosis with Polyangiitis (GPA)
B. Microscopic Polyangiitis (MPA)
C. Churg-Strauss Syndrome (EGPA)
D. Goodpasture’s Syndrome
A
Which of the following findings is least likely to be associated with Churg-Strauss Syndrome (EGPA)?
A. Peripheral eosinophilia
B. Migratory pulmonary infiltrates
C. Subepithelial immune deposits on biopsy
D. Asthma and allergic rhinitis
C
Which risk factor is most associated with the development of Granulomatosis with Polyangiitis (GPA)?
A. Hepatitis B infection
B. Silica dust exposure
C. Smoking
D. Chronic alcoholism
B
Which treatment regimen is most appropriate for a patient with active ANCA-associated vasculitis involving the kidneys and lungs?
A. Oral prednisone only
B. High-dose prednisone + Rituximab or Cyclophosphamide
C. Azathioprine and low-dose prednisone
D. Mycophenolate mofetil alone
B
Which of the following laboratory findings is most suggestive of ANCA-associated vasculitis?
A. Positive ANA and anti-dsDNA antibodies
B. Low C3 and C4 complement levels
C. Positive PR3-ANCA or MPO-ANCA
D. Positive anti-GBM antibodies
C
Which of the following histopathologic findings is most characteristic of Dense Deposit Disease (DDD) on electron microscopy?
A. Subepithelial immune deposits
B. Subendothelial deposits with “humps”
C. Ribbon-like intramembranous deposits in the GBM
D. Mesangial hypercellularity with crescent formation
C
Which of the following laboratory findings is most characteristic of C3 glomerulopathy?
A. Low C3 and low C4 levels
B. Normal C3 and C4 levels
C. Low C3 with normal C4 levels
D. High C3 and low C4 levels
C
A patient with proteinuria, hematuria undergoes kidney biopsy showing dominant C3 deposits with minimal immunoglobulin staining. What is the most likely diagnosis?
A. Dense Deposit Disease (DDD)
B. Membranous nephropathy
C. IgA nephropathy
D. Goodpasture’s Syndrome
A
Which of the following genetic abnormalities is most commonly associated with C3 glomerulopathy?
A. Mutations in the CFHR protein genes
B. Mutations in the COL4A5 gene
C. Deletion of the α3 NC1 domain of collagen IV
D. Mutations in the NPHS1 gene, and partial lipodystrophy
A
____ is characterized by thickening of the GBM with mesangioproliferative changes often leading to a lobular appearance of the glomerular tuf
MPGN
Type___MPGN is immune complex–mediated and commonly associated with persistent hepatitis B and C, fungal and parasitic infections, SBE, autoimmune diseases such as lupus or cryoglobulinemia, or monoclonal gammopathies,
I
MPGN Type?
The most proliferative of the three types
Type 1
MPGN Type?
Tram tracking
Type 1
MPGN Type?
Subendothelial deposits with low serum levels of C3. May have normal C3 levels in 50% of patients
Type 1
MPGN Type?
Low serum C 3and a dense thickening of the GBM containing ribbons of dense deposits and C3
Type 2
MPGN Type?
Dense Deposit Disease
Type 2
