Vasculitis — MCQs
A 24-year-old woman presents with abdominal pain, joint discomfort, and lower limb rash. She was well until 1 week before presentation. On examination, she has a palpable purpuric rash on her legs, nonspecific abdominal discomfort, and no active joints. She has 3+ proteinuria, normal WBC, no eosinophils, and elevated creatinine of 1.6 mg/dL. Biopsy of the rash confirms vasculitis with immunoglobulin A (IgA) 3+and C3 (complement 3) deposition on immunofluorescence.For the above patient with vasculitis syndrome, select the most likely diagnosis.
Investigation of choice for confirming Henoch Schönlein Purpura is
Patient presenting with cutaneous vasculitis, glomerulonephritis, peripheral neuropathy, Which investigation is to be performed next that will help you diagnose the condition?
A child with fever for 6 days, strawberry tongue, conjunctival congestion with peeling of skin. What will be the treatment option for this child?
All of the following features may be used to distinguish PAN from microscopic polyangitis, except:
Which of the following is a 'Small Vessel Vasculitis':
Patient presenting with cutaneous vasculitis, glomerulonephritis, peripheral neuropathy, which investigation is to be performed next that will help you diagnose the condition?
The mode of inheritance of Incontinentia pigmenti is:
A study of persons developing skin lesions following sun exposure is conducted. The lesions are not found on skin protected from ultraviolet light. Biopsies of involved skin show immunoglobulin G deposition along the dermal-epidermal junction, along with vacuolization of the basal layer and a perivascular lymphocytic infiltrate. No other organ involvement is present. Which of the following diseases do these patients most likely have?
Morphea most commonly occurs in which location?
Vasculitis Indian Medical PG Practice Questions and MCQs
Question 1: A 24-year-old woman presents with abdominal pain, joint discomfort, and lower limb rash. She was well until 1 week before presentation. On examination, she has a palpable purpuric rash on her legs, nonspecific abdominal discomfort, and no active joints. She has 3+ proteinuria, normal WBC, no eosinophils, and elevated creatinine of 1.6 mg/dL. Biopsy of the rash confirms vasculitis with immunoglobulin A (IgA) 3+and C3 (complement 3) deposition on immunofluorescence.For the above patient with vasculitis syndrome, select the most likely diagnosis.
- A. vasculitis associated with infectious diseases
- B. Churg-Strauss disease
- C. Henoch-Schonlein purpura (Correct Answer)
- D. polyarteritis nodosa (PAN)
Explanation: ***Henoch-Schonlein purpura*** - The classic triad of **palpable purpura**, **arthralgia**, and **abdominal pain**, along with **renal involvement (proteinuria, elevated creatinine)**, strongly suggests Henoch-Schonlein purpura (HSP) [1]. - **IgA deposition** on biopsy of the rash is the hallmark of HSP, confirming its diagnosis [1]. *vasculitis associated with infectious diseases* - While infections can trigger vasculitis, the specific presentation with **IgA deposition**, palpable purpura, and prominent gastrointestinal/renal involvement points away from this broad category [1]. - No clear infectious trigger or specific pathogen is mentioned, making a more specific diagnosis like HSP more appropriate given the findings. *Churg-Strauss disease* - Characterized by **asthma**, **eosinophilia**, and **granulomatous inflammation**, none of which are present in this patient (normal WBC, no eosinophils mentioned) [1]. - Typically involves **ANCA-positive vasculitis (MPO-ANCA)**, which is not indicated here, and IgA deposition is not a characteristic feature [1]. *polyarteritis nodosa (PAN)* - Primarily affects **medium-sized arteries** and can cause **aneurysms** and **organ ischemia**, but classically spares the capillaries and does not involve IgA deposition or palpable purpura. - Often associated with **hepatitis B infection** and lacks the prominent skin and renal manifestations of HSP with IgA deposition.
Question 2: Investigation of choice for confirming Henoch Schönlein Purpura is
- A. Serum IgA levels
- B. CRP levels
- C. DTPA
- D. Renal Biopsy (Correct Answer)
Explanation: ***Renal Biopsy*** - **Biopsy (renal or skin)** showing **IgA deposition** is the **confirmatory investigation** for Henoch-Schönlein Purpura (HSP) when histological confirmation is needed [1]. - **Renal biopsy** demonstrates characteristic **IgA-dominant immune deposits** in the mesangium and glomerular capillaries, along with **mesangial proliferation** [1]. - While HSP is primarily a **clinical diagnosis** based on palpable purpura, age < 20 years, abdominal pain, and renal involvement, biopsy provides **definitive confirmation** in atypical presentations or when diagnosis is uncertain. - Immunofluorescence showing **IgA deposition** is the pathognomonic finding [1]. *Serum IgA levels* - Serum IgA levels may be elevated in approximately **50% of HSP cases**, but this is **neither sensitive nor specific**. - **Normal serum IgA does NOT exclude HSP**, making it unreliable as a confirmatory test. - Elevated IgA can occur in many other conditions (IgA nephropathy without vasculitis, liver disease, infections). - Provides only supportive evidence, not confirmation. *CRP levels* - **C-reactive protein (CRP)** is a **non-specific inflammatory marker** that may be elevated in HSP. - Cannot distinguish HSP from other inflammatory or infectious conditions. - Has no role in confirming the diagnosis. *DTPA* - **DTPA scan** assesses **renal perfusion and function** but does not provide diagnostic information about the underlying pathology. - Cannot detect the characteristic **IgA-mediated vasculitis** of HSP. - Not useful for confirming the diagnosis. **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. 526-527.
Question 3: Patient presenting with cutaneous vasculitis, glomerulonephritis, peripheral neuropathy, Which investigation is to be performed next that will help you diagnose the condition?
- A. ANCA (Correct Answer)
- B. RA factor
- C. Hbsag
- D. MIF
Explanation: ### ANCA - The combination of **cutaneous vasculitis**, **glomerulonephritis**, and **peripheral neuropathy** points towards a small-vessel vasculitis, for which **ANCA (anti-neutrophil cytoplasmic antibodies)** testing is crucial [1]. - ANCA is highly specific for conditions like **Granulomatosis with Polyangiitis (GPA)** and **Microscopic Polyangiitis (MPA)** [1]. ### RA factor - **Rheumatoid factor (RF)** is primarily associated with **rheumatoid arthritis**, which typically presents with symmetrical polyarthritis, not the constellation of symptoms described. - While RF can be positive in some vasculitides, it is not the most specific initial test for the given clinical presentation. ### Hbsag - **Hepatitis B surface antigen (HbsAg)** typically screens for **Hepatitis B infection**, which can cause **polyarteritis nodosa (PAN)**, a medium-vessel vasculitis. - However, the patient's symptoms (cutaneous vasculitis, glomerulonephritis) are more characteristic of **small-vessel vasculitis**, making ANCA a more direct investigation [1]. ### MIF - **MIF (Macrophage Migration Inhibitory Factor)** is a cytokine involved in inflammation, but it is not a routine diagnostic marker for vasculitis. - It is not used as a primary investigation to diagnose specific autoimmune or inflammatory conditions like vasculitis.
Question 4: A child with fever for 6 days, strawberry tongue, conjunctival congestion with peeling of skin. What will be the treatment option for this child?
- A. Antibiotics
- B. Steroids
- C. Antipyretics
- D. IVIG (Correct Answer)
Explanation: ***IVIG*** - The constellation of **fever for 6 days (prolonged fever)**, **strawberry tongue**, **conjunctival congestion**, and **peeling skin** is highly indicative of **Kawasaki disease**. - **Intravenous immunoglobulin (IVIG) 2 g/kg as a single infusion** is the cornerstone of treatment for Kawasaki disease to reduce the risk of **coronary artery aneurysms** (from ~25% to <5%). - IVIG should be administered within **10 days of fever onset** for maximum efficacy. - **High-dose aspirin** (80-100 mg/kg/day) is given concurrently until the fever subsides, then switched to low-dose aspirin (3-5 mg/kg/day) for antiplatelet effect. *Antibiotics* - Kawasaki disease is a **vasculitis**, not a bacterial infection, so antibiotics are ineffective. - While other conditions like scarlet fever can present with strawberry tongue, the prolonged fever and other classic Kawasaki features differentiate it. *Steroids* - While steroids can reduce inflammation, they are **not the primary treatment** for Kawasaki disease and are typically used in conjunction with IVIG in **refractory cases** or for IVIG-resistant disease. - **Monotherapy with steroids** is not recommended for acute Kawasaki disease due to potential for increased aneurysm risk. *Antipyretics* - **Antipyretics** like acetaminophen can help manage the fever symptomatically. - However, they **do not treat the underlying vasculitis** or prevent the serious cardiac complications of Kawasaki disease. - Note: **NSAIDs like ibuprofen should be avoided** when high-dose aspirin is being used due to risk of drug interactions.
Question 5: All of the following features may be used to distinguish PAN from microscopic polyangitis, except:
- A. RBC cast in urine
- B. ANCA positivity
- C. HBV infection
- D. Necrotizing vasculitis (Correct Answer)
Explanation: ***Necrotizing vasculitis*** - Both **polyarteritis nodosa (PAN)** and **microscopic polyangiitis (MPA)** are characterized by **necrotizing vasculitis**, making it a shared feature rather than a distinguishing one. [1] - This pathological finding describes the **inflammation** and **necrosis** of vessel walls, which is central to the pathogenesis of both conditions. [1] *RBC cast in urine* - **Red blood cell (RBC) casts** in the urine are indicative of **glomerulonephritis**, which is a prominent feature of **microscopic polyangiitis (MPA)** but typically absent in **polyarteritis nodosa (PAN)**. [1] - The presence of RBC casts points to **renal involvement**, particularly in the small vessels of the glomeruli, which distinguishes MPA's pattern of injury. [1] *ANCA positivity* - **Anti-neutrophil cytoplasmic antibodies (ANCAs)**, particularly **p-ANCA (MPO-ANCA)**, are frequently positive in **microscopic polyangiitis (MPA)** but usually negative in **polyarteritis nodosa (PAN)**. - ANCA positivity helps classify MPA as an **ANCA-associated vasculitis**, a distinction not typically applied to PAN. *HBV infection* - **Hepatitis B virus (HBV) infection** is strongly associated with a significant subset of **polyarteritis nodosa (PAN)** cases, whereas this association is rare in **microscopic polyangiitis (MPA)**. - Serological testing for HBV can therefore help differentiate between the two conditions, with a positive result favoring PAN.
Question 6: Which of the following is a 'Small Vessel Vasculitis':
- A. Takayasu's Disease
- B. Polyaeritis Nodosa (PAN)
- C. Microscopic polyangitis (Correct Answer)
- D. Giant cell vasculitis
Explanation: ***Microscopic polyangitis*** - This is a form of **ANCA-associated vasculitis** that primarily affects **small vessels** (capillaries, venules, and arterioles) [1]. - It is characterized by **necrotizing vasculitis** within these small vessels, often presenting with features like **glomerulonephritis** and **pulmonary hemorrhage** [1]. *Takayasu's Disease* - This is a **large vessel vasculitis** that primarily affects the **aorta** and its major branches [1]. - It often presents with symptoms related to **ischemia** in the upper limbs or head, such as claudication or syncope. *Polyaeritis Nodosa (PAN)* - This is a **medium-sized vessel vasculitis** that typically spares capillaries, venules, and arterioles, distinguishing it from small vessel vasculitis [1]. - It classically presents as a **necrotizing vasculitis** affecting multiple organs, often without affecting the lungs or glomeruli significantly. *Giant cell vasculitis* - Also known as **Temporal Arteritis**, this is a **large vessel vasculitis** affecting the aorta and its major branches, particularly the branches of the **carotid artery** [1]. - It is characterized by the presence of **giant cells** in the inflamed vessel wall and is a common cause of **headache**, **jaw claudication**, and **vision loss** in older adults.
Question 7: Patient presenting with cutaneous vasculitis, glomerulonephritis, peripheral neuropathy, which investigation is to be performed next that will help you diagnose the condition?
- A. HbsA
- B. RA factor
- C. Complement levels (C3, C4)
- D. ANCA (Correct Answer)
Explanation: ***ANCA*** - The combination of **cutaneous vasculitis**, **glomerulonephritis**, and **peripheral neuropathy** strongly suggests a **systemic vasculitis**, for which ANCA testing is crucial for diagnosis and classification [1]. - **ANCA (anti-neutrophil cytoplasmic antibodies)** are primarily associated with **ANCA-associated vasculitides** such as **granulomatosis with polyangiitis (GPA)**, **microscopic polyangiitis (MPA)**, and **eosinophilic granulomatosis with polyangiitis (EGPA)** [1]. *HbsA* - **Hepatitis B surface antigen (HbsAg)** testing is relevant in the context of **polyarteritis nodosa (PAN)**, a type of vasculitis sometimes associated with hepatitis B infection. - However, the clinical presentation here, particularly the glomerulonephritis, is less typical for PAN and more suggestive of ANCA-associated vasculitis. *RA factor* - **Rheumatoid factor (RF)** is primarily associated with **rheumatoid arthritis**, which typically presents with inflammatory polyarthritis rather than widespread vasculitis, glomerulonephritis, and peripheral neuropathy. - While some cases of **rheumatoid vasculitis** can occur, the constellation of symptoms points more strongly towards a primary systemic vasculitis. *Complement levels (C3, C4)* - **Low complement levels (C3, C4)** are characteristic of **cryoglobulinemic vasculitis** (often associated with **HCV infection**), **lupus nephritis**, or certain forms of **post-streptococcal glomerulonephritis**. - While vasculitis and glomerulonephritis can occur in these conditions, the specific combination presented (cutaneous vasculitis, glomerulonephritis, peripheral neuropathy) makes **ANCA-associated vasculitis** a more direct and urgent consideration for initial investigation.
Question 8: The mode of inheritance of Incontinentia pigmenti is:
- A. Autosomal dominant
- B. Autosomal recessive
- C. X-linked dominant (Correct Answer)
- D. X-linked recessive
Explanation: **Explanation:** **Incontinentia Pigmenti (Bloch-Sulzberger syndrome)** is a rare multisystem neurocutaneous disorder caused by a mutation in the **IKBKG gene** (formerly NEMO). 1. **Why X-linked Dominant is correct:** The inheritance is **X-linked dominant**. The mutation is typically **lethal in males** in utero, which is why the clinical phenotype is seen almost exclusively in females. Affected females survive due to functional mosaicism resulting from **X-chromosome inactivation (Lyonization)**. 2. **Why other options are wrong:** * **Autosomal Dominant/Recessive:** The gene is located on the X chromosome (Xq28), ruling out autosomal inheritance. * **X-linked Recessive:** In recessive conditions, heterozygous females are usually asymptomatic carriers. In IP, a single mutated allele is sufficient to cause the disease phenotype in females. **Clinical Phases (High-Yield for NEET-PG):** The skin lesions characteristically follow the **Lines of Blaschko** and evolve through four distinct stages: 1. **Vesicular stage:** Linear vesicles (present at birth or shortly after). 2. **Verrucous stage:** Hyperkeratotic, wart-like plaques. 3. **Hyperpigmented stage:** "Swirl-like" or "Marble cake" pigmentation (due to melanin incontinence into the dermis). 4. **Hypopigmented/Atrophic stage:** Linear streaks of hypopigmentation and hair loss. **Clinical Pearls:** * **Associated findings:** Peg-shaped (conical) teeth, delayed dentition, seizures, and cicatricial alopecia. * **Histology:** Eosinophilic spongiosis is a characteristic feature of the first stage. * **Key differentiator:** Unlike other X-linked dominant conditions, the male-to-female ratio is heavily skewed due to male lethality.
Question 9: A study of persons developing skin lesions following sun exposure is conducted. The lesions are not found on skin protected from ultraviolet light. Biopsies of involved skin show immunoglobulin G deposition along the dermal-epidermal junction, along with vacuolization of the basal layer and a perivascular lymphocytic infiltrate. No other organ involvement is present. Which of the following diseases do these patients most likely have?
- A. Bullous pemphigoid
- B. Celiac disease
- C. Discoid lupus erythematosus (Correct Answer)
- D. Dysplastic nevus syndrome
Explanation: ### Explanation The clinical presentation and histopathology described are classic for **Discoid Lupus Erythematosus (DLE)**, a form of Chronic Cutaneous Lupus Erythematosus (CCLE). **1. Why Discoid Lupus Erythematosus (DLE) is correct:** * **Photosensitivity:** DLE lesions are typically triggered or exacerbated by UV light and are confined to sun-exposed areas (face, scalp, ears). * **Histopathology:** The "vacuolization of the basal layer" (interface dermatitis) and "perivascular lymphocytic infiltrate" are hallmark features. * **Direct Immunofluorescence (DIF):** The "Lupus Band Test" shows a granular deposition of IgG and C3 along the dermal-epidermal junction (DEJ). In DLE, this is positive **only in involved (lesional) skin**, consistent with the question stating no other organ involvement (systemic lupus would often show deposition in uninvolved skin as well). **2. Why the other options are incorrect:** * **Bullous Pemphigoid:** While it shows IgG at the DEJ, it presents with subepidermal blisters and a linear (not granular) pattern. It is not typically induced by sun exposure. * **Celiac Disease:** This is associated with Dermatitis Herpetiformis, which presents with itchy vesicles on elbows/knees and shows **IgA** (not IgG) deposition in the dermal papillae. * **Dysplastic Nevus Syndrome:** This involves pigmented melanocytic lesions with architectural atypia, not an autoimmune inflammatory process with IgG deposition. **High-Yield Clinical Pearls for NEET-PG:** * **Lupus Band Test:** Positive in lesional skin in DLE; positive in both lesional and non-lesional skin in SLE. * **DLE Triad:** Erythema, adherent scales (carpet tack sign/follicular plugging), and atrophic scarring. * **Progression:** Only about 5-10% of patients with DLE progress to Systemic Lupus Erythematosus (SLE). * **Treatment:** Sun protection is the first step; topical corticosteroids or antimalarials (Hydroxychloroquine) are first-line medical therapies.
Question 10: Morphea most commonly occurs in which location?
- A. Forehead (Correct Answer)
- B. Sternum
- C. Limbs
- D. Back
Explanation: **Explanation:** **Morphea**, also known as localized scleroderma, is characterized by excessive collagen deposition leading to thickening and hardening of the skin. Unlike systemic sclerosis, it typically lacks internal organ involvement or Raynaud’s phenomenon. **Correct Option: A. Forehead** While morphea can occur anywhere on the body, the **forehead** is the most characteristic and classic site for the linear subtype of morphea, specifically known as **"En coup de sabre"** (resembling a stroke from a sword). This presentation involves a linear induration and atrophy on the forehead and scalp, often associated with alopecia and, occasionally, underlying hemi-facial atrophy (Parry-Romberg syndrome). In the context of standard medical examinations like NEET-PG, the forehead is recognized as the most frequently tested and clinically significant site for localized linear morphea. **Incorrect Options:** * **B. Sternum:** While morphea can occur on the trunk, the sternum is not the most common site. The trunk is more frequently involved in "Generalized Morphea," but the forehead remains the classic site for localized linear variants. * **C. Limbs:** Linear morphea can affect the extremities, potentially leading to joint contractures or limb-length discrepancies, but it is statistically less "classic" for board-style questions than the forehead presentation. * **D. Back:** Plaque-type morphea often appears on the trunk (including the back), but it is not the most common or characteristic site compared to the forehead in clinical vignettes. **Clinical Pearls for NEET-PG:** * **En coup de sabre:** Linear morphea on the forehead/scalp. * **Histology:** Shows "squared-off" biopsy specimens, thickened collagen bundles, and loss of adnexal structures (eccrine glands/hair follicles). * **Treatment:** First-line therapy typically involves topical corticosteroids or calcineurin inhibitors; methotrexate or UVA1 phototherapy is used for deeper or progressive disease.
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