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.

Q: Normal sized to enlarged kidneys in a patient with chronic failure is indicative of which of the following conditions?

Primary amyloidosis. **Explanation:** In most cases of Chronic Kidney Disease (CKD), kidneys are typically shrunken and scarred (small, echogenic kidneys on ultrasound) [1]. However, certain conditions are classic exceptions where kidneys remain **normal-sized or enlarged** despite advanced renal failure. **1. Why Primary Amyloidosis is correct:** In Primary Amyloidosis (AL), there is an extracellular deposition of insoluble amyloid fibrils within the renal parenchyma (glomeruli, tubules, and interstitium). This progressive accumulation increases the overall mass and volume of the kidney, preventing the typical shrinkage seen in chronic failure. Even as the GFR declines, the kidneys remain large and "waxy." **2. Why the other options are incorrect:** * **Benign Nephrosclerosis:** This is the result of long-standing hypertension, leading to hyaline arteriolosclerosis and ischemic atrophy. It characteristically results in **symmetrically shrunken kidneys** with a finely granular surface. * **Chronic Glomerulonephritis:** This represents the end-stage of various glomerular diseases. It is the most common cause of **small, contracted kidneys** due to extensive fibrosis and loss of nephrons. * **Chronic Interstitial Nephritis:** Chronic inflammation of the tubules and interstitium leads to progressive tubular atrophy and interstitial fibrosis, resulting in **small, irregular kidneys [2].** **3. High-Yield Clinical Pearls for NEET-PG:** To remember the causes of **Large Kidneys in CKD**, use the mnemonic **"SHAPE"**: * **S:** **S**cleroderma (Systemic Sclerosis) * **H:** **H**IV-associated nephropathy (HIVAN) * **A:** **A**myloidosis * **P:** **P**olycystic Kidney Disease (ADPKD) * **E:** **E**ndocrinopathy (**Diabetes Mellitus** – the most common cause) *Note: In Diabetes, kidneys are enlarged in the early stages (hyperfiltration) and typically remain normal-sized even when renal failure develops.*

Question 1

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 2

Alport syndrome is characterized by antibodies acting against which component?

Accepted Answer

Collagen IV

Answer

Laminin

Answer

Fibronectin

Answer

Heparan sulphate

Question 3

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 4

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 5

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 6

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 7

Normal sized to enlarged kidneys in a patient with chronic failure is indicative of which of the following conditions?

Accepted Answer

Primary amyloidosis

Answer

Benign nephrosclerosis

Answer

Chronic glomerulonephritis

Answer

Chronic interstitial nephritis

Question 8

Kimmelstiel-Wilson disease is diagnostic of which of the following conditions?

Accepted Answer

Diabetic glomerulosclerosis

Answer

Benign hypertension

Answer

Malignant hypertension

Answer

Amyloidosis

Question 9

A person positive for Hepatitis B surface antigen (HBsAg) may have all of the following associated renal lesions, except?

Accepted Answer

Focal Segmental Glomerulosclerosis (FSGS)

Answer

Membranous Glomerulonephritis (MGN)

Answer

Membranoproliferative Glomerulonephritis (MPGN)

Answer

Mesangiocapillary Glomerulonephritis

Question 10

All of the following proteins are decreased in Nephrotic syndrome, except?

Accepted Answer

Fibrinogen

Answer

Transferrin

Answer

Albumin

Answer

Thyroxine Binding Globulin

Renal Pathology — MCQs

Renal Pathology — MCQs

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