Glomerular Diseases (Nephrotic/Nephritic Syndromes) MCQs

Pathology · 145 free questions with answers & explanations.

1. A 10-year-old boy with nephrotic syndrome undergoes renal biopsy after steroid resistance. LM shows mesangial hypercellularity and segmental sclerosis. EM shows diffuse podocyte foot process effacement without immune deposits. IHC is positive for IgM and C3 in sclerotic segments. This pattern most likely represents:
2. A 30-year-old woman with SLE develops nephrotic syndrome. Renal biopsy reveals diffuse thickening of glomerular basement membranes with 'wire-loop' deposits. IF shows granular IgG and C3 in subendothelial and mesangial locations. This corresponds to which WHO/ISN class of lupus nephritis?
3. A 70-year-old man presents with acute nephritic syndrome: hematuria, proteinuria, hypertension. Serum ANCA (PR3-ANCA/c-ANCA) is positive. Renal biopsy shows focal necrotizing glomerulonephritis with cellular crescents. IF shows NO immune deposits ('pauci-immune'). The diagnosis is:
4. On electron microscopy of a renal biopsy, 'humps' — large, dome-shaped electron-dense deposits on the subepithelial surface of the GBM — are most characteristic of which glomerular disease?
5. Minimal change disease in adults shows no abnormality on light microscopy and immunofluorescence, but electron microscopy reveals diffuse podocyte foot process effacement. The primary immunological driver is thought to be:
6. A 10-year-old boy develops hematuria, edema and hypertension two weeks after streptococcal throat infection. Renal biopsy shows subepithelial 'hump-shaped' immune deposits on electron microscopy and granular IgG/C3 deposits on immunofluorescence. This pattern is most consistent with:
7. Focal segmental glomerulosclerosis (FSGS) on biopsy shows sclerosis affecting some glomeruli (focal) and only part of each affected glomerulus (segmental). The FSGS variant associated with highest risk of recurrence after renal transplantation is:
8. Membranoproliferative glomerulonephritis (MPGN) type II (dense deposit disease) is now classified under C3 glomerulopathy. The characteristic ultrastructural finding that distinguishes it from MPGN type I is:
9. A 35-year-old woman with lupus nephritis class IV undergoes repeat biopsy after 6 months of mycophenolate mofetil plus steroids. The biopsy now shows class II (mesangial) pattern. The NIH activity index has decreased from 14 to 3, but chronicity index is 7. What is the most appropriate next clinical decision?
10. A 10-year-old boy with post-streptococcal glomerulonephritis has electron microscopy showing subepithelial 'hump-shaped' electron-dense deposits. Immunofluorescence shows coarse granular IgG and C3 deposits in a 'starry sky' pattern. Which complement pathway is primarily activated?
11. A 25-year-old man with hematuria and sensorineural deafness has a renal biopsy showing irregular thinning and splitting of the GBM with a 'basket-weave' pattern on electron microscopy. His mother has microscopic hematuria but no renal failure. COL4A5 mutation is found. What is the inheritance pattern and expected prognosis?
12. A 40-year-old woman with proteinuria 8 g/day and normal renal function undergoes renal biopsy. Light microscopy shows a 'spike and dome' pattern with periodic acid-methenamine silver stain, and IF shows granular IgG4 and PLA2R1 deposits. Which serological test is most useful for monitoring treatment response?
13. Electron microscopy of a renal biopsy from a 5-year-old with nephrotic syndrome shows diffuse effacement of podocyte foot processes but NO immune deposits. Light microscopy shows normal glomeruli. Immunofluorescence is negative. The pathogenesis is best explained by:
14. A 25-year-old woman with SLE has nephrotic-range proteinuria. Renal biopsy shows 'wire loop' capillary deposits on light microscopy. Immunofluorescence shows 'full house' positivity (IgG, IgA, IgM, C3, C1q). Electron microscopy shows subendothelial deposits. This represents:
15. A 35-year-old IV drug user develops focal segmental glomerulosclerosis (FSGS). The pattern on light microscopy shows scarring and hyalinosis affecting only PART of the glomerulus and only SOME glomeruli. What is the mechanism by which HIV causes FSGS (HIV-associated nephropathy)?
16. In anti-GBM disease (Goodpasture syndrome), the autoantibodies target the α3 chain of type IV collagen in the GBM. The Goodpasture antigen is normally cryptic (not exposed to immune system). Which event is thought to UNMASK this antigen and trigger the autoimmune response?
17. A 30-year-old man with nephrotic syndrome has a renal biopsy. Light microscopy shows no significant changes, electron microscopy shows diffuse effacement of podocyte foot processes, and immunofluorescence is negative. A targeted genetic panel reveals a heterozygous missense mutation in NPHS2 (podocin). The diagnosis and expected response to steroids are:
18. A patient with IgA nephropathy has Oxford MEST-C pathological scoring. The 'E' lesion in MEST-C that predicts faster progression to ESRD refers to:
19. Electron microscopy of a renal biopsy from a patient with membranoproliferative glomerulonephritis (MPGN) shows dense deposits within the GBM lamina densa forming ribbon-like, sausage-shaped osmiophilic deposits. C3 is strongly positive on IF but no immunoglobulin staining. The diagnosis and underlying mechanism are:
20. A 25-year-old with anti-GBM disease (Goodpasture syndrome) has linear IgG along the GBM on immunofluorescence. The target antigen is the NC1 domain of COL4A3 (α3 chain of type IV collagen). This specific chain is absent from which anatomical location, explaining why only glomeruli and lung alveoli are affected?
21. A 28-year-old woman with SLE develops nephrotic-range proteinuria. Renal biopsy shows diffuse global endocapillary hypercellularity and 'wire loop' deposits on light microscopy. Immunofluorescence shows a 'full house' pattern (IgG, IgM, IgA, C3, C1q). This corresponds to which ISN/RPS 2018 class?
22. A child presents with nephrotic syndrome. Renal biopsy on light microscopy appears normal, but electron microscopy reveals diffuse effacement of podocyte foot processes. Which of the following podocyte-specific proteins is mutated in the autosomal recessive congenital form of this condition?
23. A 45-year-old man with a history of hepatitis C develops hematuria and proteinuria. Serum complement (C3) is low. Renal biopsy reveals hypercellularity with double-contour (tram-track) appearance of glomerular basement membranes on PAS/silver stain. Which immunofluorescence pattern is expected?
24. In C3 glomerulopathy (C3G), the pathogenic mechanism that differentiates it from immune-complex MPGN is:
25. Electron microscopy of a kidney biopsy from a child with nephrotic syndrome shows diffuse effacement of podocyte foot processes with no immune deposits. Light microscopy appears normal. The glomerular protein leak is best explained by loss of which molecular component of the glomerular filtration barrier?
26. A 42-year-old woman with SLE develops nephrotic syndrome and hypertension. Renal biopsy shows global mesangial and subendothelial immune complex deposits with 'full house' immunofluorescence (IgG, IgM, IgA, C3, C1q). This pattern corresponds to which WHO/ISN-RPS class of lupus nephritis that requires most aggressive immunosuppression?
27. A 58-year-old man with hepatitis C infection develops nephrotic syndrome, hematuria, and hypocomplementemia (low C3 and C4). Renal biopsy shows mesangial and subendothelial immune deposits with a 'tram-track' appearance on silver stain due to mesangial interposition. Immunofluorescence reveals IgM and C3 deposits. The most likely diagnosis is:
28. A 10-year-old boy presents with macroscopic hematuria following an upper respiratory tract infection (synpharyngitic hematuria — hematuria within 1-3 days of throat infection). Renal biopsy shows mesangial IgA deposits. Which complement pathway is primarily activated in IgA nephropathy?
29. A kidney biopsy from a 35-year-old woman with nephrotic syndrome shows thickened glomerular basement membranes with 'spike and dome' pattern on silver stain and granular IgG and C3 deposits on IF. Electron microscopy shows subepithelial deposits. The pathogenesis of primary membranous nephropathy most commonly involves autoantibodies against which target?
30. A 6-year-old child with steroid-resistant nephrotic syndrome undergoes biopsy showing focal segmental glomerulosclerosis (FSGS) with tip lesion variant. Genetic testing reveals a NPHS1 mutation. NPHS1 encodes which protein critical to the slit diaphragm?
31. A young man has hematuria, sensorineural hearing loss, and ocular abnormalities (anterior lenticonus). Renal biopsy electron microscopy shows 'basket-weave' (irregular thinning and splitting) of the glomerular basement membrane. The underlying molecular defect is:
32. A 50-year-old diabetic woman has microalbuminuria that progresses to nephrotic-range proteinuria over 5 years. Renal biopsy shows nodular glomerulosclerosis. The pathognomonic nodule in diabetic nephropathy is called:
33. Kidney biopsy from a 35-year-old with nephrotic syndrome shows diffuse mesangial proliferation, subendothelial deposits on electron microscopy, and C3-dominant granular deposits on immunofluorescence with 'mesangial fingerprint' pattern. No ANA, ANCA, or anti-GBM antibodies. C3 is low; C4 is normal. Serum shows a C3 nephritic factor. This pattern is most consistent with:
34. A 9-year-old boy has nephrotic syndrome with minimal change on light microscopy, effacement of podocyte foot processes on EM, and no immune deposits. His condition responds completely to steroids. The primary pathogenic mechanism in minimal change disease involves:
35. A 40-year-old woman with recurrent deep vein thrombosis, prolonged aPTT, and positive lupus anticoagulant develops proteinuria. Renal biopsy shows segmental glomerular capillary thrombosis, arteriolar fibrin thrombi, and focal cortical infarcts without immune complex deposits. Immunofluorescence is negative. This pattern corresponds to:
36. A 52-year-old diabetic has proteinuria of 4.5 g/day and creatinine of 2.1 mg/dL. Renal biopsy shows diffuse glomerulosclerosis with Kimmelstiel-Wilson nodules and arteriolar hyalinosis affecting the efferent arteriole more than the afferent arteriole. The pathogenic significance of efferent arteriolar hyalinosis in diabetic nephropathy specifically is:
37. A 40-year-old man with hepatitis C infection presents with proteinuria, hematuria, and low C3. Renal biopsy shows mesangial and endocapillary hypercellularity with a 'tram-track' appearance on silver stain. Immunofluorescence shows IgM, IgG, C3 deposits. The pathogenetic mechanism most specific to this entity is:
38. C3 glomerulopathy (C3G) is defined by glomerular C3 deposits with absent or minimal immunoglobulin deposits on immunofluorescence. The unifying pathogenetic mechanism across all C3G variants is:
39. In focal segmental glomerulosclerosis (FSGS), the Columbia classification recognizes five histological variants. Which variant carries the worst prognosis and highest risk of progression to ESRD?
40. A 7-year-old child with idiopathic nephrotic syndrome undergoes renal biopsy. Electron microscopy shows diffuse foot process effacement and microvillous transformation of podocytes. Light microscopy and immunofluorescence are essentially normal. The major molecular target of the circulating permeability factor in this condition is:
41. A renal biopsy from a 10-year-old boy with steroid-resistant nephrotic syndrome shows normal light microscopy, no immune deposits on immunofluorescence, and diffuse podocyte foot process effacement on electron microscopy. Genetic testing reveals a homozygous missense mutation in NPHS2 encoding podocin. Which of the following correctly describes the molecular role of podocin and how this mutation disrupts the glomerular filtration barrier?
42. A 28-year-old woman with SLE develops hematuria, RBC casts, proteinuria of 3.2 g/day and rising serum creatinine. Renal biopsy shows diffuse proliferative changes with 'wire loop' deposits; immunofluorescence reveals full-house staining (IgG, IgM, IgA, C3, C1q). Electron microscopy shows subendothelial and mesangial deposits with occasional subepithelial ('humps'). Which ISN/RPS class does this represent, and what makes the 'wire loop' lesion pathognomonic of severe disease?
43. In the evaluation of a patient with rapidly progressive glomerulonephritis (RPGN) showing crescents in >50% of glomeruli, immunofluorescence reveals linear IgG deposits along the GBM. Anti-GBM antibody ELISA is strongly positive. Which domain of which molecule do the pathogenic antibodies target, and why does this cause such rapid crescent formation compared to other RPGN types?
44. In anti-GBM disease (Goodpasture syndrome), autoantibodies target the non-collagenous domain 1 (NC1) of which specific collagen chain?
45. A 7-year-old child with nephrotic syndrome undergoes renal biopsy showing normal light microscopy, no immune deposits on immunofluorescence, and diffuse effacement of podocyte foot processes on electron microscopy. The primary pathophysiological defect responsible for proteinuria in this condition is:
46. A 35-year-old woman with SLE develops renal involvement. Renal biopsy shows endocapillary hypercellularity, wire-loop lesions, hyaline thrombi, and full-house immunofluorescence (IgG, IgA, IgM, C3, C1q). What WHO/ISN-RPS class does this represent, and what is its significance?
47. A 10-year-old boy with nephrotic syndrome fails steroid therapy. Kidney biopsy shows no light-microscopic abnormality, effacement of podocyte foot processes on EM, and negative immunofluorescence. NPHS2 mutation is found on genetic testing. Which protein is encoded by NPHS2 and why does its defect cause proteinuria?
48. A 24-year-old woman presents with haematuria, proteinuria, and hypertension 10 days after a throat infection. Kidney biopsy shows diffuse endocapillary hypercellularity and 'humps' on electron microscopy. Immunofluorescence shows 'starry sky' C3 predominant granular deposits. Serum C3 is low and C4 is normal. Which pathway of complement is primarily activated in this condition?
49. Kidney biopsy of a patient with nephrotic syndrome shows focal segmental glomerulosclerosis (FSGS). Electron microscopy reveals diffuse podocyte foot process effacement. IHC with anti-phospholipase A2 receptor (PLA2R) antibody stains the glomerular capillary walls strongly positive. How does this finding alter the diagnosis?
50. A patient with C3 glomerulopathy (C3GN) undergoes genetic testing. A heterozygous gain-of-function mutation in C3 (p.Lys155Gln) is found. What is the direct consequence of this mutation on the alternative complement pathway?
51. A 40-year-old woman with HIV infection (CD4 count 120/μL, not on ART) presents with nephrotic syndrome and rapidly progressive renal failure. Renal biopsy shows collapsing focal segmental glomerulosclerosis (FSGS) with prominent tubuloreticular inclusions on electron microscopy. Which molecular mechanism underlies HIV-associated nephropathy (HIVAN)?
52. A 55-year-old man with IgA nephropathy (IgAN) undergoes renal biopsy that shows Oxford Classification features: M1 (mesangial hypercellularity), E0, S1 (segmental glomerulosclerosis), T1 (tubular atrophy/interstitial fibrosis 25-50%), C0. Based on these features, what is the expected renal prognosis?
53. Anti-GBM disease (Goodpasture syndrome) produces linear IgG deposits along the GBM. The target antigen is the NC1 domain of alpha-3(IV) collagen. Why does the disease also cause pulmonary hemorrhage in some patients but spare the lung in others despite systemic autoantibodies?
54. C3 glomerulopathy (C3G) is characterized by C3-dominant glomerular deposits by immunofluorescence with absent or trace immunoglobulin. Which pathogenetic mechanism most accurately explains C3G?
55. A 35-year-old HIV-positive African American man presents with nephrotic-range proteinuria. Renal biopsy shows collapsing variant of focal segmental glomerulosclerosis (FSGS) with podocyte hypertrophy and hyperplasia overlying collapsed and sclerotic glomerular tuft. Electron microscopy shows extensive podocyte foot process effacement and tubulo-reticular inclusions (TRIs) in endothelial cells. The pathogenesis most directly involves:
56. In C3 glomerulopathy (C3G), which distinguishes the two main entities — dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) — by electron microscopy and the underlying pathomechanism?
57. A kidney biopsy shows linear IgG deposition along the glomerular basement membrane (GBM) on immunofluorescence with no electron-dense deposits. The patient has anti-GBM antibodies. The epitope targeted by pathogenic antibodies in anti-GBM (Goodpasture) disease resides in:
58. A 12-year-old boy with recurrent gross hematuria following upper respiratory infections has IgA nephropathy. Renal biopsy shows mesangial IgA deposits. The current understanding of IgA nephropathy pathogenesis involves which molecular mechanism as the initiating event?
59. A 40-year-old HIV-positive patient develops nephrotic syndrome. Renal biopsy shows collapsing focal segmental glomerulosclerosis (FSGS) with microcystic tubular dilatation. Electron microscopy shows prominent tubuloreticular inclusions in endothelial cells. What is the pathogenesis of this lesion?
60. A 15-year-old boy presents with recurrent macroscopic hematuria following upper respiratory infections. Renal biopsy shows mesangial hypercellularity with mesangial IgA and C3 deposits on immunofluorescence. Electron microscopy confirms mesangial electron-dense deposits. The current pathogenic model of IgA nephropathy involves aberrant glycosylation of which immunoglobulin?
61. A 35-year-old woman presents with rapidly progressive glomerulonephritis. Renal biopsy shows crescentic glomerulonephritis. Immunofluorescence: linear IgG along the glomerular basement membrane. Anti-GBM antibodies are detected in serum. What is the specific target antigen for these antibodies?
62. A renal biopsy from a 50-year-old with nephrotic syndrome shows thickened GBM and 'spike and dome' pattern on silver stain, with granular IgG and PLA2R deposits on immunofluorescence. Electron microscopy shows subepithelial deposits. Anti-PLA2R antibodies are positive. This patient's membranous nephropathy is classified as:
63. A 7-year-old boy has nephrotic syndrome with 4.5 g/day proteinuria, serum albumin 1.8 g/dL, and hypocomplementemia. Renal biopsy shows mesangial and subendothelial deposits on EM, positive C3 and C1q on immunofluorescence, and mesangial hypercellularity with double contours on PAS stain. The child's serum C3 is 40 mg/dL (low) and C4 is normal. Which condition is the most likely underlying diagnosis?
64. Anti-GBM disease (Goodpasture syndrome) occurs when autoantibodies target the non-collagenous (NC1) domain of which collagen type, and what is the specific alpha chain involved?
65. A 45-year-old man with longstanding type 2 diabetes has nephrotic-range proteinuria and renal biopsy shows Kimmelstiel-Wilson nodules. Immunohistochemistry reveals these nodules stain with which combination of markers, confirming their nature?
66. Focal segmental glomerulosclerosis (FSGS) due to mutation in the NPHS2 gene encoding podocin is classified as which variant of FSGS in the Columbia classification, and why does it fail to respond to steroid therapy?
67. IgA nephropathy is pathogenetically linked to defective galactosylation of IgA1 hinge-region O-glycans. The resultant poorly galactosylated IgA1 (Gd-IgA1) promotes glomerular injury via:
68. On electron microscopy of a renal biopsy, 'subepithelial humps' (electron-dense deposits under podocyte foot processes) are the hallmark of which nephritis?
69. The anti-PLA2R antibody in primary membranous nephropathy targets the M-type phospholipase A2 receptor expressed on:
70. A child with nephrotic syndrome has normal-appearing glomeruli on light microscopy, effacement of podocyte foot processes on EM, and negative immunofluorescence. In steroid-resistant cases, which gene mutation should be screened?
71. A 40-year-old man with HIV infection develops nephrotic syndrome. Renal biopsy shows collapsing focal segmental glomerulosclerosis (FSGS) with microcystic tubular dilatation and no immune deposits on immunofluorescence. What is the underlying pathogenetic mechanism?
72. A 12-year-old boy develops hematuria and proteinuria 1 week after streptococcal skin infection. Renal biopsy electron microscopy shows subepithelial 'hump-shaped' electron-dense deposits. Which complement pathway is primarily activated?
73. A 35-year-old woman presents with nephrotic syndrome. Renal biopsy shows diffuse thickening of the glomerular basement membrane with 'spike and dome' pattern on Jones silver stain. Immunofluorescence shows granular IgG and C3. Which antibody is most commonly responsible for the primary form of this disease?
74. A 25-year-old man presents with rapidly progressive glomerulonephritis (RPGN). Renal biopsy shows circumferential crescents in >50% of glomeruli with parietal cell proliferation and fibrin. Direct immunofluorescence is negative (pauci-immune). What is the MOST likely serological finding?
75. A renal biopsy from a 10-year-old child with nephrotic syndrome shows normal light microscopy, negative immunofluorescence, and electron microscopy demonstrating diffuse effacement of podocyte foot processes. Which of the following mechanisms BEST explains the massive proteinuria?
76. Membranoproliferative glomerulonephritis type II (Dense Deposit Disease) is characterised by electron-dense deposits within the glomerular basement membrane (intramembranous). Which complement pathway abnormality is MOST responsible for its pathogenesis?
77. A 42-year-old HIV-positive man from sub-Saharan Africa presents with nephrotic syndrome. Biopsy shows collapsing focal segmental glomerulosclerosis with tubuloreticular inclusions on electron microscopy. The FSGS variant here is specifically linked to which viral mechanism?
78. Electron microscopy of a renal biopsy from a 10-year-old with nephrotic syndrome shows diffuse effacement of podocyte foot processes, no immune deposits, and normal light microscopy. What is the MOST likely diagnosis?
79. A 35-year-old woman with lupus develops nephrotic-range proteinuria. Renal biopsy shows diffuse global thickening of capillary walls with 'wire loop' appearance on light microscopy and granular subendothelial deposits on immunofluorescence. This corresponds to which ISN/RPS class of lupus nephritis?
80. Anti-PLA2R (phospholipase A2 receptor) antibodies are detectable in the serum in what percentage of cases of primary membranous nephropathy and have diagnostic significance?
81. A 7-year-old boy presents with cola-colored urine, periorbital edema, and hypertension 2 weeks after a throat infection. Renal biopsy shows diffuse hypercellularity with neutrophil infiltration and 'humps' on electron microscopy. Complement C3 is low. What is the underlying immunopathologic mechanism?
82. A 10-year-old boy presents with haematuria, hypertension, and periorbital oedema 2 weeks after a streptococcal throat infection. Electron microscopy of the renal biopsy shows large subepithelial electron-dense deposits described as 'humps'. Which complement pathway is primarily activated in this condition?
83. A 35-year-old woman with SLE develops nephrotic-range proteinuria. Renal biopsy shows global diffuse proliferative glomerulonephritis with 'wire loop' lesions on light microscopy and subendothelial immune deposits on EM. This is classified as:
84. A 50-year-old man with hepatitis C develops nephrotic syndrome and hypocomplementaemia. Renal biopsy shows a 'tram-track' pattern on PAS staining with mesangial interposition. Immunofluorescence shows IgG, IgM, and C3 in a granular mesangial and capillary wall distribution. The most likely diagnosis is:
85. A renal biopsy from a 45-year-old man with nephrotic syndrome shows thickened GBM with 'spike and dome' pattern on silver stain and granular IgG + PLA2R deposits on immunofluorescence. The primary autoantigen in this condition is located on:
86. A child with minimal change disease (MCD) has selective proteinuria of albumin with preserved excretion of IgG. Which ultrastructural glomerular change explains the selective loss of albumin?
87. A patient with post-streptococcal glomerulonephritis (PSGN) has granular immune deposits on immunofluorescence. These deposits have a characteristic pattern described as:
88. Focal segmental glomerulosclerosis (FSGS) secondary to HIV nephropathy (HIVAN) shows a characteristic histological variant. Which variant and which ultrastructural feature distinguish HIVAN-associated FSGS?
89. In IgA nephropathy (Berger disease), the pathogenic mechanism involves deposition of galactose-deficient IgA1. In which part of the nephron do these deposits characteristically localise?
90. A 6-year-old boy presents with periorbital oedema and heavy proteinuria. Renal biopsy shows normal glomeruli on light microscopy, negative immunofluorescence, and fusion (effacement) of podocyte foot processes on electron microscopy. What is the underlying pathophysiological mechanism of proteinuria?
91. Membranoproliferative glomerulonephritis (MPGN) type I shows a characteristic 'tram-track' or double contour of the GBM on silver stain. This appearance results from:
92. IgA nephropathy (Berger disease) is the most common primary glomerulonephritis worldwide. The key immunofluorescence finding is:
93. A 28-year-old man with a recent sore throat now presents with haematuria, red cell casts, hypertension, and mild oliguria 2 weeks later. Serum C3 is low; ASO titre is elevated. Which lesion is most likely seen on renal biopsy?
94. The 'tram-track' double contour of the GBM in MPGN type I on silver stain results from:
95. A 28-year-old man presents with haematuria, red cell casts, hypertension, and mild oliguria 2 weeks after a sore throat. Serum C3 is low and ASO titre is elevated. The renal biopsy finding most likely to be seen is:
96. A 6-year-old boy presents with periorbital edema, massive proteinuria (8 g/day), hypoalbuminemia, and hyperlipidemia. Renal biopsy shows normal glomeruli on light microscopy, negative immunofluorescence, and foot process effacement on electron microscopy. First-line treatment is:
97. A 35-year-old HIV-positive man presents with nephrotic-range proteinuria and progressive renal failure. Biopsy shows segmental glomerulosclerosis with collapse of glomerular capillaries and prominent podocyte hypertrophy and hyperplasia. The pattern is best described as:
98. A 25-year-old man presents with hematuria, proteinuria, hypertension, and reduced GFR after an upper respiratory tract infection. Biopsy shows diffuse mesangial IgA deposits on immunofluorescence. The pathognomonic immunofluorescence finding in IgA nephropathy is:
99. A 45-year-old with SLE has class IV lupus nephritis on biopsy. The WHO/ISN class IV is characterized by:
100. A 7-year-old presents with hematuria, oliguria, and edema 2 weeks after a throat infection. Serum complement C3 is low while C4 is normal. The pattern of complement consumption (low C3, normal C4) suggests activation via:
101. On electron microscopy of a renal biopsy from a child with nephrotic syndrome, diffuse effacement of podocyte foot processes is seen with no immune deposits. Light microscopy shows normal glomeruli. Immunofluorescence is negative. This pattern is diagnostic of:
102. A 35-year-old man presents with hematuria, proteinuria, and hypertension. Serum complement (C3) is low acutely but returns to normal within 8 weeks. Renal biopsy shows mesangial and subendothelial deposits with a 'lumpy-bumpy' granular IgG/C3 pattern on immunofluorescence. The most likely diagnosis is:
103. Anti-GBM antibody disease (Goodpasture syndrome) targets which specific antigen in the glomerular basement membrane?
104. A renal biopsy from a patient with nephrotic syndrome shows thickening of the GBM with silver stain 'spike and dome' pattern on EM showing subepithelial deposits. Serology reveals anti-PLA2R antibodies. The most appropriate first-line diagnosis is:
105. On electron microscopy, a biopsy from a child with nephrotic syndrome shows diffuse effacement of podocyte foot processes with no immune deposits. Light microscopy is normal. The most likely diagnosis is:
106. In anti-GBM (Goodpasture) disease, immunofluorescence of the kidney shows a characteristic pattern of IgG staining. Which pattern is pathognomonic?
107. A patient with SLE develops nephrotic syndrome. Renal biopsy shows global thickening of all glomerular capillary walls on light microscopy, subepithelial and intramembranous deposits on EM, and granular IgG along the GBM on immunofluorescence. This corresponds to which WHO/ISN class of lupus nephritis?
108. In post-streptococcal glomerulonephritis, the pathological mechanism involves 'humps' on electron microscopy. These subepithelial humps consist primarily of:
109. A renal biopsy from a child with nephrotic syndrome shows normal light microscopy, negative immunofluorescence, and effacement of podocyte foot processes on electron microscopy. The pathogenesis involves dysfunction of which podocyte protein most directly linked to the filtration slit diaphragm?
110. A 35-year-old man with hepatitis B infection develops nephrotic syndrome. Biopsy shows subepithelial 'spike and dome' deposits on silver stain and granular IgG on immunofluorescence. What is the most likely diagnosis?
111. In post-streptococcal glomerulonephritis, the key mechanism of glomerular injury is:
112. Alport syndrome is caused by mutations in genes encoding which glomerular basement membrane component, leading to progressive nephritis with sensorineural deafness?
113. A 40-year-old man with IV drug abuse develops nephrotic syndrome. Renal biopsy shows focal segmental glomerulosclerosis with collapse of the glomerular tuft and prominent podocyte hypertrophy and hyperplasia. Which FSGS variant is this?
114. Electron microscopy shows subepithelial electron-dense deposits with 'spike and dome' pattern on silver stain. Immunofluorescence shows granular IgG and C3 along the GBM. This is diagnostic of:
115. A 10-year-old boy develops gross hematuria 2 weeks after strep throat. C3 is low but C4 is normal. Which complement pathway activation mechanism explains this?
116. A 25-year-old man has hematuria, proteinuria, and sensorineural hearing loss. His mother has the same condition. Electron microscopy shows 'basket-weave' lamellation of the GBM. The underlying genetic defect is:
117. Electron microscopy of a renal biopsy from a 35-year-old woman with nephrotic syndrome and SLE shows subendothelial and mesangial immune complex deposits, with tubuloretiuclar inclusions in endothelial cells. Light microscopy shows a 'tram-track' pattern on silver stain. What is the WHO/ISN-RPS classification of this lupus nephritis?
118. A 10-year-old boy with recent throat infection develops hematuria, edema, and hypertension. Complement C3 is low but C4 is normal. Renal biopsy shows 'humps' on electron microscopy. Which complement pathway is primarily activated in this condition?
119. A 45-year-old man presents with nephrotic syndrome. Renal biopsy shows irregular spikes on silver stain along the outer aspect of the GBM, and granular IgG deposits on immunofluorescence in a subepithelial pattern. Anti-phospholipase A2 receptor (PLA2R) antibodies are elevated. What is the pathological diagnosis?
120. A 35-year-old HIV-positive man presents with nephrotic-range proteinuria. Renal biopsy shows focal segmental glomerulosclerosis with collapse of capillary loops and overlying podocyte hypertrophy. This pattern is called:
121. Immunofluorescence of a renal biopsy shows linear IgG deposits along the glomerular basement membrane (GBM). This pattern is MOST consistent with:
122. A child develops nephrotic syndrome 2 weeks after a bee sting. Renal biopsy shows no light microscopy abnormality but electron microscopy reveals diffuse podocyte foot process effacement. The most likely diagnosis is:
123. In membranous nephropathy, the antigen targeted by circulating autoantibodies in the primary (idiopathic) form is:
124. A 30-year-old woman develops nephrotic syndrome. Renal biopsy light microscopy shows diffuse basement membrane thickening without increase in cellularity. Silver stain shows a 'spike and dome' pattern along the capillary walls. Immunofluorescence shows granular IgG and C3 deposition along GBM. Electron microscopy shows subepithelial electron-dense deposits. What is the diagnosis?
125. IgA nephropathy (Berger disease) is the most common primary glomerulonephritis worldwide. The pathognomonic immunofluorescence finding is:
126. A 7-year-old boy presents with periorbital edema, heavy proteinuria (5g/day), hypoalbuminemia, and hyperlipidemia. Renal biopsy light microscopy is normal. EM shows diffuse effacement of podocyte foot processes with no immune deposits. Which of the following pathophysiological mechanisms best explains the proteinuria?
127. A 22-year-old man presents with hematuria, proteinuria (2.5 g/day), and sensorineural hearing loss. His maternal uncle died of renal failure. Electron microscopy of his renal biopsy shows irregular thinning and thickening of the GBM with a characteristic 'basket-weave' or 'moth-eaten' pattern of lamellation. The diagnosis is:
128. A 10-year-old boy develops nephritic syndrome 2 weeks after a streptococcal throat infection. Renal biopsy immunofluorescence shows 'starry sky' pattern of coarse, granular IgG and C3 deposits in a lumpy-bumpy distribution. EM shows large, hump-shaped subepithelial deposits. The correct name for these deposits is:
129. A 25-year-old man with HIV infection presents with nephrotic syndrome. Renal biopsy shows collapsing focal segmental glomerulosclerosis (FSGS). Electron microscopy reveals tubuloreticular inclusions in endothelial cells. What is the pathogenic mechanism specific to HIV-associated nephropathy (HIVAN)?
130. In minimal change disease (MCD), the glomeruli appear normal on light microscopy but electron microscopy shows diffuse podocyte foot process effacement. What is the current evidence-supported primary pathogenic hypothesis for MCD?
131. Membranoproliferative glomerulonephritis (MPGN) type II (dense deposit disease) is characterised by intramembranous dense deposits and low C3 complement levels with normal C1q and C4. What is the primary mechanism of complement dysregulation in dense deposit disease?
132. A 4-year-old child presents with periorbital edema, heavy proteinuria (>3.5 g/day), hypoalbuminemia, and hyperlipidemia. Renal biopsy by light microscopy appears normal, but electron microscopy shows diffuse foot process effacement with no immune deposits. The pathological diagnosis is:
133. IgA nephropathy (Berger disease) characteristically presents with gross hematuria coinciding with upper respiratory infections ('synpharyngitic hematuria'). The immune complex deposits are found predominantly in the:
134. Goodpasture syndrome causes rapidly progressive glomerulonephritis by which immune mechanism?
135. A patient with membranous nephropathy is found to have anti-PLA2R antibodies. PLA2R (phospholipase A2 receptor) is expressed on which glomerular cell, and what type of immune deposits are seen on electron microscopy?
136. Electron microscopy of a renal biopsy shows subepithelial 'hump-shaped' dense deposits along glomerular basement membranes. This pattern is most consistent with which condition?
137. A 25-year-old man with nephrotic syndrome undergoes renal biopsy showing segmental sclerosis and hyalinosis in some glomeruli on PAS stain, with foot process effacement on EM. Mutations in which structural protein are commonly identified in the hereditary forms of this condition?
138. A 45-year-old woman with longstanding hepatitis C infection presents with nephrotic-range proteinuria and hypocomplementemia (low C3 and C4). Cryoglobulins are detected in serum. Renal biopsy shows a lobular 'tram-track' pattern on silver stain. What is the underlying mechanism of glomerular injury?
139. A 10-year-old boy with a 3-day history of oliguria and hematuria after an upper respiratory infection is found to have crescents in >50% of glomeruli on biopsy with linear IgG deposits along the GBM on immunofluorescence. Which additional autoantibody test is most likely to be positive?
140. On electron microscopy of a renal biopsy from a 6-year-old child with nephrotic syndrome, diffuse effacement of podocyte foot processes is seen with no immune deposits and normal light microscopy. Immunofluorescence is negative. The pathogenesis involves:
141. A 35-year-old woman presents with nephrotic syndrome. Renal biopsy shows uniform thickening of the GBM on light microscopy (silver stain — no spikes), negative IF for IgG/IgM/C3, and thin GBM with focal effacement on EM. Genetic testing reveals a mutation in NPHS2 (podocin). This pattern represents:
142. In membranoproliferative glomerulonephritis (MPGN) type II (Dense Deposit Disease), the 'tram-track' appearance on PAS stain and massive mesangial/subendothelial dense deposits are caused by uncontrolled activation of which complement pathway?
143. A 25-year-old man presents with hemoptysis and hematuria, anti-GBM antibody positive. Renal biopsy shows linear IgG deposits along the GBM and crescentic (rapidly progressive) glomerulonephritis. The antigen targeted by anti-GBM antibody is:
144. IgA nephropathy (Berger disease) is the most common primary glomerulonephritis worldwide. The mesangial IgA deposits in IgA nephropathy are predominantly which subclass, and what is the key structural abnormality in the IgA molecule?
145. M-type phospholipase A2 receptor (PLA2R) antibodies are found in approximately 70-80% of primary membranous nephropathy. These antibodies deposit as immune complexes in which glomerular compartment?