Renal Pathology Practice Questions

Q: p-ANCA is sensitive and specific for which of the following conditions?

Idiopathic crescentic glomerulonephritis. **Explanation:** **1. Why Option B is Correct:** Idiopathic Crescentic Glomerulonephritis (Type III Pauci-immune RPGN) is characterized by the presence of crescents in the glomeruli without significant immune complex or anti-GBM antibody deposits [1]. Approximately **80% of patients** with this condition are positive for **p-ANCA** (anti-myeloperoxidase) [1]. It is considered a renal-limited form of microscopic polyangiitis. **2. Analysis of Incorrect Options:** * **Option A (PSGN):** This is a Type II (Immune-complex mediated) hypersensitivity reaction. Diagnosis is based on low C3 levels and elevated ASO/anti-DNAse B titers, not ANCA. * **Option C (Diffuse glomerulosclerosis):** This is typically the end-stage of diabetic nephropathy or chronic glomerulonephritis. It is characterized by Kimmelstiel-Wilson nodules and basement membrane thickening, unrelated to ANCA. * **Option D (Wegener's Granulomatosis):** Now known as Granulomatosis with Polyangiitis (GPA), this condition is classically associated with **c-ANCA** (anti-proteinase 3), not p-ANCA. **3. High-Yield Clinical Pearls for NEET-PG:** * **c-ANCA (PR3-ANCA):** Associated with Wegener’s Granulomatosis (GPA). Shows cytoplasmic staining [1]. * **p-ANCA (MPO-ANCA):** Associated with Microscopic Polyangiitis (MPA), Churg-Strauss Syndrome (EGPA), and Idiopathic Crescentic Glomerulonephritis [1]. Shows perinuclear staining. * **Pauci-immune status:** Defined by a Negative Immunofluorescence (IF) for IgG and C3 [1]. * **Crescents:** Formed by the proliferation of parietal epithelial cells and infiltration of monocytes/macrophages into Bowman's space. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 917-918.

Q: Polycystic kidney disease is a genetic disorder primarily transmitted in which of the following patterns?

Autosomal dominant. **Explanation:** **Autosomal Dominant Polycystic Kidney Disease (ADPKD)** is the most common inherited kidney disorder [1]. It is primarily transmitted in an **Autosomal Dominant** pattern, meaning an affected individual has a 50% chance of passing the gene to each offspring. It typically manifests in adulthood (3rd to 4th decade), which is why it was historically called "Adult PCKD" [1]. * **Why Option A is correct:** ADPKD is caused by mutations in the **PKD1** (Chromosome 16, ~85% cases) or **PKD2** (Chromosome 4, ~15% cases) genes [1]. These genes encode polycystin-1 and polycystin-2, proteins essential for cilia function and tubular epithelial integrity [1]. * **Why Option B is incorrect:** Autosomal Recessive PCKD (ARPKD) exists but is much rarer. It presents in infancy or childhood (infantile PCKD) and is associated with mutations in the **PKHD1** gene on Chromosome 6. * **Why Options C & D are incorrect:** There are no recognized X-linked or purely multifactorial forms of classic Polycystic Kidney Disease; it is strictly a monogenic Mendelian disorder. **High-Yield Clinical Pearls for NEET-PG:** 1. **Extra-renal manifestations:** The most common is **Liver cysts**. The most life-threatening is **Berry Aneurysms** (Circle of Willis), which can lead to Subarachnoid Hemorrhage (SAH). Other features include Mitral Valve Prolapse (MVP) and diverticulosis. 2. **PKD1 vs. PKD2:** Mutations in PKD1 lead to earlier onset and more rapid progression to End-Stage Renal Disease (ESRD) compared to PKD2 [1]. 3. **Diagnosis:** Ultrasound is the primary screening tool; the presence of bilateral enlarged kidneys with multiple cysts is diagnostic. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 950-951.

Question 1

Minimal change glomerulopathy may be seen in association with all of the following except?

Accepted Answer

Hepatitis B

Answer

HIV

Answer

Drug-induced interstitial nephritis

Answer

Hodgkin's disease

Question 2

What is the commonest renal lesion in Systemic Lupus Erythematosus (SLE)?

Accepted Answer

Diffuse proliferative glomerulonephritis

Answer

Focal proliferative glomerulonephritis

Answer

Membranous nephropathy

Answer

Minimal change disease

Question 3

p-ANCA is sensitive and specific for which of the following conditions?

Accepted Answer

Idiopathic crescentic glomerulonephritis

Answer

Post-streptococcal glomerulonephritis

Answer

Diffuse glomerulosclerosis

Answer

Wegener's granulomatosis

Question 4

Alport syndrome is characterized by antibodies acting against which component?

Accepted Answer

Collagen IV

Answer

Laminin

Answer

Fibronectin

Answer

Heparan sulphate

Question 5

A 36-year-old man presents with an episode of hemoptysis. This is his second episode in several months. Urinalysis shows PBC casts and 1+ protein. An autoantibody screen is ordered, and immunofluorescence on a kidney biopsy shows a linear pattern. The patient is most likely to test positive for which of the following?

Accepted Answer

Anti-GBM

Answer

Anti-CCP

Answer

Anti-Jo-1

Answer

C-ANCA

Question 6

Which one of the following individuals is most likely to have a tumor characterized by undifferentiated mesenchymal cells with immature tubules and abortive glomerular formation?

Accepted Answer

An 8-month-old infant with an abdominal mass and normal urinary vanillylmandelic acid (VMA)

Answer

A 2-week-old infant with a midepigastric mass, projectile vomiting, and normal urinary hydroxy-indoleacetic acid (HIAA)

Answer

A 14-month-old infant with an abdominal mass and increased urinary VMA

Answer

A 39-year-old female with abdominal cramps, watery diarrhea, periodic facial flushing, wheezing, and increased urinary HIAA

Question 7

Renal biopsy in a patient of microscopic polyangiitis (MPA) will typically yield which of the following findings?

Accepted Answer

Pauci-immune crescentic glomerulonephritis

Answer

Subepithelial humps on electron microscopy

Answer

Deposits showing apple-green birefringence on polarized light

Answer

Normal renal histology

Question 8

Polycystic kidney disease is a genetic disorder primarily transmitted in which of the following patterns?

Accepted Answer

Autosomal dominant

Answer

Autosomal recessive

Answer

X-linked

Answer

Multifactorial

Question 9

Idiopathic nephrotic syndrome is associated with which of the following conditions, except?

Accepted Answer

Membranoproliferative glomerulonephritis

Answer

Focal segmental glomerulosclerosis

Answer

Minimal change disease

Answer

Mesangioproliferative glomerulonephritis

Question 10

Which of the following is true regarding amyloidosis?

Accepted Answer

None of the above

Answer

Massive proteinuria

Answer

Mild hypertension

Answer

Normal kidney size

Renal Pathology — MCQs

Renal Pathology — MCQs

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