Renal Pathology Practice Questions

Q: Which of the following statements about fibronectin glomerulopathy is false?

Autosomal recessive inheritance. **Autosomal recessive inheritance** - **Fibronectin glomerulopathy** is predominantly inherited in an **autosomal dominant** pattern, not autosomal recessive. - This condition is caused by mutations in the **FN1 gene**, which codes for fibronectin, a large glycoprotein involved in tissue organization. *Glomerulus do not consistently stain for Ig and complement* - This statement is **true** for fibronectin glomerulopathy; immunofluorescence typically shows a lack of consistent staining for immunoglobulins and complements. - This absence of immune complex deposition helps differentiate it from immune-mediated glomerular diseases. *Ultrastructural feature is presence of large electron mesangial or subendothelial deposit* - This statement accurately describes a key ultrastructural finding in fibronectin glomerulopathy. - Electron microscopy reveals characteristic **electron-dense deposits** predominantly in the mesangium and subendothelial space. *Glomerular enlargement and PAS+ trichrome mesangial deposit* - This statement also correctly describes features of fibronectin glomerulopathy. - Light microscopy often shows **glomerular enlargement** and the presence of **Periodic Acid-Schiff (PAS)-positive and trichrome-positive mesangial deposits**.

Q: Renal papillary necrosis is almost always associated with one of the following conditions:

Analgesic-nephropathy. ***Analgesic-nephropathy*** - Chronic use of certain analgesics (especially **phenacetin**, aspirin, and NSAIDs) can lead to **ischemia** and damage to the renal papillae, causing **papillary necrosis**. - This condition is considered the **classic** cause of renal papillary necrosis and is the most frequently emphasized in medical education. - Analgesic nephropathy shows a very **strong and direct association** with papillary necrosis as a hallmark feature. *Diabetes-mellitus* - **Diabetes mellitus** is actually one of the **most common causes** of renal papillary necrosis in clinical practice, particularly when complicated by **infection** or **ischemia** [1]. - While clinically very common, it causes papillary necrosis through multiple mechanisms and is often associated with coexisting factors like **pyelonephritis** [1], [2] or NSAID use. - In the context of "almost always associated," analgesic nephropathy has a more direct and consistent association. *Chronic pyelonephritis* - **Chronic pyelonephritis** involves recurrent bacterial infections of the kidney parenchyma and can lead to scarring and kidney damage. - While it is indeed a recognized cause of **papillary necrosis** (part of the POSTCARDS mnemonic), it is not as consistently associated as analgesic nephropathy [2]. *Post streptococcal GN* - **Post-streptococcal glomerulonephritis (PSGN)** is an immune-mediated inflammatory kidney disease that typically follows a **streptococcal infection**. - It primarily affects the **glomeruli** and does **not** cause necrosis of the renal papillae, making this option incorrect. **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. 543-544. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 937-940.

Q: Crescents in glomerular diseases are derived from which of the following components?

Epithelial cells + fibrin + macrophages. ***Epithelial cells + fibrin + macrophage*** - Crescents are formed due to the accumulation of **epithelial cells**, **fibrin**, and **macrophages** in the urinary space [1], commonly seen in rapid progressive glomerulonephritis. - This composition leads to crescent formation, which is associated with **severe glomerular injury** and can indicate a poor prognosis. *Mesangium + fibrin* - Mesangial cells primarily contribute to the structural integrity of the glomeruli but are not involved in crescent formation, which requires epithelial cells. - The absence of **macrophages** in this composition makes it **incomplete** for defining crescents. *Mesangium + fibrin + macrophage* - While macrophages are involved in the inflammatory process, crescents specifically arise from **epithelial cells**, not mesangial cells. - This option fails to highlight the critical role of **epithelial cells** needed for crescent formation in glomeruli. *Tubule + mesangium + fibrin* - Crescents are not formed from tubules or mesangial components but rather from the **proliferation of epithelial cells**. - This oes not accurately represent the **cellular components** necessary for crescent formation, leading to an incorrect conclusion. **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. 528-529.

Q: Polycystic disease of the kidney may have cysts in all of the following organs except?

Lung. ***Lung*** - While Polycystic Kidney Disease (PKD) is a **systemic disorder**, cysts are exceptionally rare or not typically found in the **lungs**. - PKD primarily affects organs that develop from common embryonic pathways, such as the kidney, liver, pancreas, and spleen. *Liver* - **Hepatic cysts** are the most common extrarenal manifestation of **autosomal dominant polycystic kidney disease (ADPKD)**, occurring in a majority of patients [1]. - These cysts can range from asymptomatic to causing significant hepatomegaly, pain, and liver dysfunction. *Pancreas* - **Pancreatic cysts** are another common finding in ADPKD, though generally **smaller and less clinically significant** than hepatic cysts. - They are usually asymptomatic but can sometimes lead to pancreatitis or pain. *Spleen* - **Splenic cysts** are a recognised but **less common** extrarenal manifestation in patients with ADPKD. - Like other extrarenal cysts, they are often asymptomatic and discovered incidentally.

Q: HIV-specific renal nephropathy is characterized by which type of glomerular disease?

FSGS. ***FSGS*** - **Focal segmental glomerulosclerosis (FSGS)** is the most common and classic renal pathology associated with **HIV-associated nephropathy (HIVAN)** [2]. - HIVAN is characterized by **collapsing variant FSGS**, which involves **podocyte proliferation** and **tubulointerstitial disease** [1], [3]. - This is the **pathognomonic finding** in HIV-specific nephropathy [3]. *Mesangioproliferative GN* - While mesangial proliferation can be seen in some HIV-infected patients, it is not the **defining or most common feature** of **HIV-specific nephropathy** [1]. - This pattern is more broadly associated with various immune-complex mediated glomerulonephritides. *Membranous glomerulonephritis* - **Membranous glomerulonephritis** is characterized by **subepithelial immune complex deposition** and **thickening of the glomerular basement membrane** [1]. - It is not specifically associated with HIV infection but rather with conditions like hepatitis B, systemic lupus erythematosus, and certain medications [1]. *Membranoproliferative glomerulonephritis* - **Membranoproliferative glomerulonephritis (MPGN)** is characterized by **mesangial and endocapillary proliferation** and **glomerular basement membrane thickening** with a "tram-track" appearance [2]. - While sometimes seen in HIV patients, especially those co-infected with **hepatitis C**, it is not the primary or defining lesion of **HIV-associated nephropathy** [2]. **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. 531-532. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 927-928. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 924-925.

Q: Which receptor do antibodies target in primary membranous nephropathy?

Phospholipase A2 receptor. ***Phospholipase A2 receptor*** - Primary membranous nephropathy is characterized by the presence of antibodies against **Phospholipase A2 receptor**, which plays a crucial role in glomerular injury [1]. - These antibodies can be detected in the serum of many patients and are associated with **nephrotic syndrome** in this condition. *Phospholipase A1 receptor* - This receptor is not associated with primary membranous nephropathy and does not play a significant role in renal pathology. - There is a lack of evidence linking antibodies against **Phospholipase A1 receptor** to nephrotic syndrome or kidney damage. *Phospholipase A4 receptor* - There are no known associations between antibodies against **Phospholipase A4 receptor** and primary membranous nephropathy. - This receptor does not typically contribute to the pathogenic mechanisms seen in **membranous nephropathy**. *Phospholipase A3 receptor* - This receptor is not implicated in primary membranous nephropathy and lacks relevant clinical significance regarding antibody production. - Research has not demonstrated involvement of **Phospholipase A3 receptor** in nephrotic syndrome. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 911-913.

Q: Which of these is the characteristic feature of membranoproliferative glomerulonephritis?

Splitting of glomerular basement membrane. ***Splitting of glomerular basement membrane*** - Membranoproliferative glomerulonephritis is characterized by the **splitting of the glomerular basement membrane**, visible on electron microscopy [1]. - This finding is associated with **immune complex deposition** and is a hallmark of the disease process [1]. *Sub epithelial deposits* - Sub epithelial deposits are more characteristic of conditions like **post-streptococcal glomerulonephritis** or **membranous nephropathy** rather than membranoproliferative glomerulonephritis. - In membranoproliferative glomerulonephritis, deposits are typically **subendothelial or intramembranous** [1]. *Foamy cells* - Foamy cells are typically seen in conditions like **lipid nephrosis** or **hyperlipidemia**, not specifically in membranoproliferative glomerulonephritis. - They are a result of **lipid accumulation** within macrophages, rather than reflecting a characteristic feature of this condition. *All* - While some features may be present in varying glomerular conditions, **not all options are characteristic of membranoproliferative glomerulonephritis**. - The key feature of splitting of the glomerular basement membrane is solely associated with this specific condition [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 907-908, 911, 925-926.

Q: All of the following are true about xanthogranulomatous pyelonephritis except which of the following?

Seen only in infancy. ***Seen only in infancy*** - Xanthogranulomatous pyelonephritis can occur in **adults** as well, not just in infancy, making this statement false [1]. - It is often associated with obstructive uropathy and chronic infection across various age groups. *Proteus is most common organism* - While **Proteus** species are commonly associated [1], the most frequent organism in xanthogranulomatous pyelonephritis can also include **E. coli**. - It reflects a polymicrobial infection rather than being limited to a single organism. *It is a form of chronic pyelonephritis* - Xanthogranulomatous pyelonephritis is indeed a variant of **chronic pyelonephritis**, characterized by extensive **granulomatous inflammation** [1]. - This chronic condition arises typically due to obstructive pathology leading to chronic infection. *Focal form is common in children* - The **focal form** is less common in children and is more frequently observed in **adults** with underlying conditions. - Children tend to present with a more diffuse form of **xanthogranulomatous pyelonephritis** when it occurs. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 939-940.

Q: Clear cell variety of renal cell carcinoma is related to a gene located on which chromosome?

3. ***3*** - The **clear cell variety of renal cell carcinoma** (RCC) is associated with **mutations in the VHL gene**, which is located on chromosome **3** [1]. - This gene plays a crucial role in the **regulation of angiogenesis**, and its inactivation leads to tumor development. *22* - Chromosome **22** is associated with other disorders but not specifically with clear cell RCC or its genetic mutations. - Renal cell carcinoma primarily relates to **chromosome 3**, not chromosome 22 [1]. *X* - The **X chromosome** has other oncogenes and tumor suppressor genes but is not linked to clear cell RCC specifically. - Mutations in the **VHL gene** on chromosome 3 are the key factors, not those found on the X chromosome. *20* - Chromosome **20** does not contain significant genes linked to clear cell renal cell carcinoma. - The notable genetic association is with chromosome **3**, specifically the VHL gene [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 958-959.

Question 1

Which of the following statements about fibronectin glomerulopathy is false?

Accepted Answer

Autosomal recessive inheritance

Answer

Ultrastructural feature is presence of large electron-dense mesangial or subendothelial deposits

Answer

Glomerular enlargement and PAS+ trichrome mesangial deposit

Answer

Glomeruli do not consistently stain for Ig and complement

Question 2

Renal papillary necrosis is almost always associated with one of the following conditions:

Accepted Answer

Analgesic-nephropathy

Answer

Diabetes-mellitus

Answer

Chronic pyelonephritis

Answer

Post streptococcal GN

Question 3

Crescents in glomerular diseases are derived from which of the following components?

Accepted Answer

Epithelial cells + fibrin + macrophages

Answer

Mesangium + fibrin + macrophages

Answer

Tubule + mesangium + fibrin

Answer

Mesangium + fibrin

Question 4

Polycystic disease of the kidney may have cysts in all of the following organs except?

Accepted Answer

Lung

Answer

Liver

Answer

Pancreas

Answer

Spleen

Question 5

HIV-specific renal nephropathy is characterized by which type of glomerular disease?

Accepted Answer

FSGS

Answer

Membranous glomerulonephritis

Answer

Membranoproliferative glomerulonephritis

Answer

Mesangioproliferative GN

Question 6

Which receptor do antibodies target in primary membranous nephropathy?

Accepted Answer

Phospholipase A2 receptor

Answer

Phospholipase A3 receptor

Answer

Phospholipase A4 receptor

Answer

Phospholipase A1 receptor

Question 7

A 28-year-old woman has noticed increasing lower limb swelling and shortness of breath, with a 2-year history of facial rash, hair loss, arthralgias, and thrombocytopenia. On examination, her blood pressure is 150/90 mmHg, pulse 80/min, with a maculopapular rash on her face, JVP of 4 cm, normal heart sounds, clear lungs, and pedal and periorbital edema. Her creatinine is very high, and a urinalysis reveals many RBCs and RBC casts. For this patient with glomerulonephritis, select the most likely diagnosis on renal biopsy.

Accepted Answer

diffuse proliferative GN

Answer

crescentic glomerulonephritis

Answer

focal segmental glomerulosclerosis

Answer

membranoproliferative glomerulonephritis

Question 8

Which of these is the characteristic feature of membranoproliferative glomerulonephritis?

Accepted Answer

Splitting of glomerular basement membrane

Answer

Foamy cells

Answer

All of the options

Answer

Subepithelial deposits

Question 9

All of the following are true about xanthogranulomatous pyelonephritis except which of the following?

Accepted Answer

Seen only in infancy

Answer

It is a form of chronic pyelonephritis

Answer

Focal form is common in children

Answer

Proteus mirabilis is the most common organism

Question 10

Clear cell variety of renal cell carcinoma is related to a gene located on which chromosome?

Accepted Answer

3

Answer

X

Answer

22

Answer

20

Renal Pathology — MCQs

Renal Pathology — MCQs

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