Renal Pathology Practice Questions

Q: In a case of glomerulonephritis (GN), C3 is normal in all the following except?

Diffuse lupus nephritis. ***Diffuse lupus nephritis*** - This condition is characterized by **immune complex deposition** in the glomeruli [2], leading to activation of the **classical complement pathway** and consumption of C3, resulting in low C3 levels. [3] - Patients with diffuse lupus nephritis often present with **systemic lupus erythematosus (SLE)** symptoms, and **hypocomplementemia** is a hallmark. [3] *Goodpasture's syndrome* - This is an **autoimmune disease** targeting the **glomerular basement membrane (GBM)** and lung alveolar basement membranes. [1] - It involves **anti-GBM antibodies** directly, rather than widespread immune complex formation and complement consumption, so C3 levels are typically normal. [1] *IgA nephropathy* - Characterized by the deposition of **IgA immune complexes** in the glomeruli. [2] - While there is immune activity, C3 levels are generally **normal** because the alternative complement pathway, which primarily involves C3, is not extensively activated or consumed. *HSP* - **Henoch-Schönlein Purpura (HSP)** is a form of **IgA vasculitis** that can affect the kidneys, causing a glomerulonephritis similar to IgA nephropathy. - Similar to IgA nephropathy, C3 levels are usually **normal** in HSP-associated glomerulonephritis as the primary immune mechanism does not typically involve significant C3 consumption. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, p. 915. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, p. 911. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 910-911.

Q: Chromosomes associated with autosomal dominant polycystic kidney disease (ADPKD).

4 and 16. ***4 and 16*** - **ADPKD** is primarily associated with mutations in two genes: **PKD1** on chromosome **16** and **PKD2** on chromosome **4** [1]. - Mutations in **PKD1** account for approximately 85% of cases and are associated with a more severe disease course, while mutations in **PKD2** lead to a milder phenotype [1]. *12 and 16* - While chromosome 16 is involved in ADPKD through the **PKD1** gene, chromosome 12 is not typically associated with the primary genes causing this condition. - No major genes for ADPKD have been identified on chromosome 12. *6 and 14* - Neither chromosome 6 nor chromosome 14 harbor the primary gene mutations **(PKD1 or PKD2)** responsible for autosomal dominant polycystic kidney disease. - These chromosomes are known to be associated with other genetic conditions but not ADPKD. *12 and 14* - Similar to the previous option, neither chromosome 12 nor chromosome 14 are linked to the major genetic causes of **ADPKD**. - The key genes **PKD1** and **PKD2** are specifically located on chromosomes 16 and 4, respectively.

Q: Bellini duct cancer is seen in which of the following?

Kidney. ***Kidney*** - Bellini duct carcinoma is a **rare and aggressive subtype of renal cell carcinoma (RCC)**, originating from the collecting ducts of Bellini in the kidney. [1] - It accounts for a very small percentage of all RCCs and is characterized by a **poor prognosis**. *Liver* - The liver is affected by primary cancers like **hepatocellular carcinoma** and **cholangiocarcinoma**, or by metastatic disease, none of which arise from Bellini ducts. - Bellini ducts are structures **exclusive to the kidney**, not found in the liver. *Spleen* - Primary cancers of the spleen are **extremely rare**, with most malignant lesions being **lymphomas** or metastases. [2] - The spleen is a **lymphoid organ** and does not contain Bellini ducts. *Heart* - Primary cardiac tumors are uncommon, with the majority being **benign myxomas**. Malignant tumors include **sarcomas**. - The heart's anatomy is distinct and **does not contain any structures analogous to Bellini ducts**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 959-961. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 605-606.

Q: Loss of foot processes seen on electron microscopy of renal biopsy is a classical feature in which of the following?

Minimal change disease. ***Minimal change disease*** - **Loss of foot processes** (podocyte effacement) is the hallmark ultrastructural finding in **minimal change disease** on electron microscopy [1]. - This effacement of podocyte foot processes leads to increased permeability of the **glomerular filtration barrier** to albumin, causing **nephrotic syndrome** [1], [2]. *IgA nephropathy* - Characterized by **IgA immune complex deposition** in the **mesangium** on immunofluorescence. - Electron microscopy typically shows **mesangial immune deposits**, not primarily foot process effacement. *Membranous nephropathy* - Identified by the presence of **subepithelial immune deposits** and **thickening of the glomerular basement membrane** (GBM) [3]. - On electron microscopy, these deposits are visible, often with overlying **spikes** of GBM material separating them. *Rapidly progressive glomerulonephritis* - Defined by the rapid loss of renal function and the presence of **crescents** in more than 50% of glomeruli on light microscopy [2]. - While there may be secondary podocyte changes due to severe inflammation, **foot process effacement** is not its primary diagnostic feature. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 927-928. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 527-528. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 532-533.

Q: Which of the following is associated with pauci-immune glomerulonephritis?

Granulomatosis with polyangiitis (GPA). ***Granulomatosis with polyangiitis (GPA)*** - **Pauci-immune glomerulonephritis** is characterized by the absence or scarcity of immune complex deposits in the glomeruli. - This is typical of **ANCA-associated vasculitides**, such as GPA (formerly Wegener's granulomatosis), which cause severe necrotising glomerulonephritis with few immune deposits [1]. *Anti-GBM glomerulonephritis* - This condition is characterized by **linear deposition of anti-GBM antibodies** along the glomerular basement membrane, making it an **immune complex-mediated disease**, not pauci-immune [1]. - It involves autoantibodies attacking the **collagen type IV** in the GBM. *SLE nephritis* - Systemic lupus erythematosus (SLE) nephritis is a classic example of **immune complex-mediated glomerulonephritis**, with abundant immune deposits containing immunoglobulins and complement [1]. - The pathology often shows **full-house immunofluorescence** with IgG, IgA, IgM, C3, and C1q. *IgA nephropathy* - This is characterized by prominent **mesangial deposition of IgA immune complexes**, which is clearly an immune complex-mediated process [1]. - While it can present with different histological patterns, the presence of **IgA deposition** means it is not pauci-immune [1].

Q: Immunofluorescence staining pattern from a kidney biopsy from a 35-year-old patient presenting with proteinuria has been shown below. What is the most probable cause?

Lupus Nephritis. ***Lupus Nephritis*** - A **"full house" pattern** on immunofluorescence (IF) with granular staining for IgG, IgA, IgM, C3, and C1q, as depicted in the image, is highly characteristic of **lupus nephritis** [1]. - This pattern indicates widespread immune complex deposition within the glomeruli, which is the underlying pathology in lupus nephritis. *Goodpasture syndrome* - Characterized by **linear staining** for IgG along the glomerular basement membrane (GBM), in contrast to the granular pattern seen in the image [2]. - Often associated with **anti-GBM antibodies** and rapidly progressive glomerulonephritis with pulmonary hemorrhage. *FSGS* - Focal segmental glomerulosclerosis (FSGS) is primarily a **podocytopathy** and typically shows **negative or minimal immunofluorescence** staining [1]. - The granular, multi-immunoglobulin staining pattern observed in the image is not consistent with FSGS. *PSGN* - Post-streptococcal glomerulonephritis (PSGN) typically presents with **"lumpy-bumpy" granular deposits primarily of C3 and IgG**, often in a star-spangled sky or humps pattern, especially in the subepithelial space. - While it has granular staining, the "full house" pattern involving all immunoglobulins and complement components is more specific to lupus nephritis [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 530-533. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 527-528.

Q: Immunofluorescence staining pattern from a kidney biopsy from a 35-year-old patient presenting with proteinuria has been shown below. What is the most probable cause?

Lupus Nephritis. ***Lupus Nephritis*** - The image illustrates a **"full-house" immunofluorescence pattern**, characterized by positive staining for multiple immunoglobulins (IgG, IgM, IgA) and complement components (C3, C1q) [1]. - This "full-house" pattern is highly characteristic of **Lupus Nephritis**, reflecting the deposition of immune complexes containing these components in the glomeruli [1]. *FSGS* - Focal segmental glomerulosclerosis (FSGS) typically shows **minimal or no immune complex deposition** on immunofluorescence [2]. - If any staining is present, it is usually focal and segmental, primarily for IgM and C3 in sclerotic lesions, and does not exhibit a widespread "full-house" pattern [2]. *Goodpasture syndrome* - Goodpasture syndrome is characterized by a **linear pattern of IgG deposition** along the glomerular basement membrane (GBM). - This linear pattern is distinctly different from the granular, widespread, and multi-component "full-house" staining seen in the image. *PSGN* - Post-streptococcal glomerulonephritis (PSGN) typically presents with a **"lumpy-bumpy" or granular pattern** due to subepithelial immune deposits [3]. - While it involves C3 and IgG, it usually does not show the full spectrum of IgA, IgM, and C1q deposition as prominently or diffusely as seen in the "full-house" pattern of Lupus Nephritis [3]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 532-533. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 530-531. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 915-916.

Q: Renal biopsy shows 'spike and dome' appearance on silver methenamine stain. Immunofluorescence shows granular IgG deposits. Diagnosis?

Membranous nephropathy. ***Membranous nephropathy*** - The characteristic **'spike and dome' appearance** on silver methenamine stain is due to new basement membrane material laid down between subepithelial deposits, a hallmark of membranous nephropathy [1], [2]. - **Granular IgG deposits** on immunofluorescence reflect the immune complex deposition in the subepithelial space, leading to nephrotic syndrome [1]. *FSGS* - Characterized by **focal (some glomeruli) and segmental (part of glomeruli) scarring** on light microscopy, without the 'spike and dome' pattern [3]. - Immunofluorescence typically shows **negative or non-specific staining** for IgG, unlike the prominent granular deposits seen in membranous nephropathy [3]. *MPGN* - Exhibits a **'tram track' appearance** on light microscopy due to mesangial cell interposition and GBM splitting, not 'spike and dome' [4]. - Immunofluorescence can show IgG and C3 deposits, but the pattern and location are different from membranous nephropathy [4]. *Minimal change* - Light microscopy is often normal, and **electron microscopy** is required to identify **effacement of podocyte foot processes**, which is the primary pathology. - **Immunofluorescence is typically negative** as there are no immune complex deposits, distinguishing it from membranous nephropathy. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, p. 921. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 531-532. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 530-531. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 925-926.

Q: Most specific test for diagnosing Fabry disease on kidney biopsy?

Electron microscopy. ***Electron microscopy*** - **Electron microscopy** is the most specific test as it can visualize the characteristic **lamellated zebra bodies** (lysosomal inclusions of globotriaosylceramide) in various cell types, including podocytes, which are pathognomonic for Fabry disease. - While other stains might show lipid accumulation, EM provides the definitive ultrastructural evidence by identifying the specific morphology of the accumulated glycosphingolipids. *Silver stain* - **Silver stains** are primarily used to highlight **reticular fibers**, basement membranes, or certain microorganisms, and are not specific for the lipid inclusions seen in Fabry disease. - They would not differentiate Fabry inclusions from other forms of cellular deposits or normal cellular components. *H&E stain* - **Hematoxylin and Eosin (H&E) stain** is a general histological stain that can show enlarged podocytes or vacuolization in Fabry disease, but these findings are **non-specific** and can be seen in other conditions. - H&E does not specifically highlight the characteristic lysosomal lipid inclusions. *PAS stain* - **Periodic Acid-Schiff (PAS) stain** detects **carbohydrates** and mucosubstances and may stain some of the accumulated glycosphingolipids with variable intensity. - However, PAS staining is not specific for the diagnosis of Fabry disease, as other conditions can also show PAS-positive material, and it does not reveal the characteristic lamellated structure of the inclusions.

Question 1

In a case of glomerulonephritis (GN), C3 is normal in all the following except?

Accepted Answer

Diffuse lupus nephritis

Answer

Goodpasture's syndrome

Answer

IgA nephropathy

Answer

HSP

Question 2

Chromosomes associated with autosomal dominant polycystic kidney disease (ADPKD).

Accepted Answer

4 and 16

Answer

12 and 16

Answer

6 and 14

Answer

12 and 14

Question 3

Bellini duct cancer is seen in which of the following?

Accepted Answer

Kidney

Answer

Liver

Answer

Spleen

Answer

Heart

Question 4

Loss of foot processes seen on electron microscopy of renal biopsy is a classical feature in which of the following?

Accepted Answer

Minimal change disease

Answer

Membranous nephropathy

Answer

Rapidly progressive glomerulonephritis

Answer

IgA nephropathy

Question 5

Which of the following is associated with pauci-immune glomerulonephritis?

Accepted Answer

Granulomatosis with polyangiitis (GPA)

Answer

Anti-GBM glomerulonephritis

Answer

SLE nephritis

Answer

IgA nephropathy

Question 6

Immunofluorescence staining pattern from a kidney biopsy from a 35-year-old patient presenting with proteinuria has been shown below. What is the most probable cause?

Accepted Answer

Lupus Nephritis

Answer

Goodpasture syndrome

Answer

FSGS

Answer

PSGN

Question 7

Immunofluorescence staining pattern from a kidney biopsy from a 35-year-old patient presenting with proteinuria has been shown below. What is the most probable cause?

Accepted Answer

Lupus Nephritis

Answer

FSGS

Answer

Goodpasture syndrome

Answer

PSGN

Question 8

Renal biopsy shows 'spike and dome' appearance on silver methenamine stain. Immunofluorescence shows granular IgG deposits. Diagnosis?

Accepted Answer

Membranous nephropathy

Answer

FSGS

Answer

MPGN

Answer

Minimal change

Question 9

Feature NOT seen in acute rejection of transplanted kidney?

Accepted Answer

Wire-loop lesions

Answer

Tubulitis

Answer

Endothelialitis

Answer

Interstitial infiltrate

Question 10

Most specific test for diagnosing Fabry disease on kidney biopsy?

Accepted Answer

Electron microscopy

Answer

Silver stain

Answer

H&E stain

Answer

PAS stain

Renal Pathology — MCQs

Renal Pathology — MCQs

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