Vasculitis Syndromes — MCQs
All of the following conditions are associated with raised ANCA, except:
All of the following features may be used to distinguish PAN from microscopic polyangitis, except:
A 40-year-old with chronic hepatitis B develops fever, arthralgia, and palpable purpura. Most likely diagnosis?
A 45-year-old male having a long history of cigarette smoking presented with gangrene of the left foot, which was treated with an amputation. Representative sections from the specimen revealed the presence of arterial thrombus with neutrophilic infiltrate in the arterial wall, as well as inflammation extending into the adjacent veins and nerves. What is the most probable diagnosis?
Which of the following is NOT a large vessel vasculitis?
A 65-year-old patient presents with severe headache, temporal artery tenderness, and decreased pulse. What is the most likely diagnosis?
A 12-year-old boy presents with weak pulses in the upper limbs, a blood pressure of 90/60 mmHg , and retinal hemorrhages. What is the most likely diagnosis?
Which condition is most likely associated with specific angiographic findings such as the rosary sign?
An elderly male presents with pain in his shoulders and hands. ESR is 105 mm/L. History includes transient blindness and unilateral headache.
A child presented with non-blanching purpura in the lower limb and recurrent abdominal pain. Which condition is characterized by the deposition of IgA?
Vasculitis Syndromes Indian Medical PG Practice Questions and MCQs
Question 1: All of the following conditions are associated with raised ANCA, except:
- A. Polyarteritis Nodosa (Correct Answer)
- B. Wegener's granulomatosis
- C. Microscopic Polyangitis
- D. Churg-Strauss syndrome
Explanation: ***Polyarteritis Nodosa*** - Polyarteritis Nodosa is not typically associated with **positive ANCA** (Anti-Neutrophil Cytoplasmic Antibodies) and is mainly characterized by systemic vasculitis affecting medium-sized arteries. [1] - This condition usually presents with symptoms like **weight loss**, **fever**, and **muscle pain**, without the underlying ANCA association. [1] *Wegener's granulomatosis* - Also known as Granulomatosis with Polyangiitis, this condition is strongly associated with **c-ANCA** positivity, often targeting **proteinase 3**. - Clinical features include **respiratory symptoms** and renal involvement, particularly **glomerulonephritis**. *Microscopic Polyangitis* - This vasculitis is associated with **p-ANCA** positivity, commonly targeting **myeloperoxidase** (MPO). - It frequently presents with **pulmonary hemorrhage** and **renal vasculitis**. *Churg-Strauss syndrome* - Known as Eosinophilic Granulomatosis with Polyangiitis, it is associated with **p-ANCA** and involves asthma, eosinophilia, and systemic vasculitis [2]. - Typical manifestations include **respiratory involvement** and peripheral neuropathy, further linking it to ANCA positivity.
Question 2: 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 3: A 40-year-old with chronic hepatitis B develops fever, arthralgia, and palpable purpura. Most likely diagnosis?
- A. Polyarteritis nodosa (Correct Answer)
- B. IgA vasculitis
- C. Wegener's granulomatosis
- D. Cryoglobulinemia
Explanation: ***Polyarteritis nodosa*** - The triad of **fever**, **arthralgia**, and **palpable purpura** in a patient with **chronic hepatitis B** is highly suggestive of polyarteritis nodosa [1]. - **Hepatitis B infection** is strongly associated with polyarteritis nodosa due to the formation of **immune complexes** that deposit in vessel walls, leading to inflammation and vasculitis [1]. *IgA vasculitis* - Typically presents with **palpable purpura**, arthralgia, and abdominal pain, often preceded by an **upper respiratory tract infection** [1]. - It is more common in **children** and is not typically linked to chronic hepatitis B infection [1]. *Wegener's granulomatosis* - Characterized by **granulomatous inflammation** of the upper and lower respiratory tracts, **glomerulonephritis**, and systemic vasculitis. - It is associated with **anti-neutrophil cytoplasmic antibodies (c-ANCA)**, which are not suggested by the clinical picture or hepatitis B status. *Cryoglobulinemia* - Often presents with **palpable purpura**, arthralgia, and neuropathy, and is strongly associated with **hepatitis C infection**. - While hepatitis B can rarely be a cause, hepatitis C is the dominant viral association with **mixed cryoglobulinemia**.
Question 4: A 45-year-old male having a long history of cigarette smoking presented with gangrene of the left foot, which was treated with an amputation. Representative sections from the specimen revealed the presence of arterial thrombus with neutrophilic infiltrate in the arterial wall, as well as inflammation extending into the adjacent veins and nerves. What is the most probable diagnosis?
- A. Takayasu arteritis
- B. Giant cell arteritis
- C. Hypersensitivity angiitis
- D. Thromboangiitis obliterans (Correct Answer)
Explanation: ***Thromboangiitis obliterans*** - This condition is strongly linked to **heavy smoking** and is characterized by segmental, thrombosing inflammation of medium-sized and small arteries, along with associated veins and nerves, leading to **gangrene** in the extremities [1]. - The presence of **arterial thrombus with neutrophilic infiltrate** in the arterial wall, and inflammation extending to adjacent **veins and nerves**, is a classic histopathological finding [1]. *Takayasu arteritis* - This is a **large-vessel vasculitis** primarily affecting the aorta and its main branches, leading to **absent pulses** ("pulseless disease") and claudication in the upper extremities [2]. - It typically does not involve the small and medium-sized arteries of the distal extremities or present with inflammation extending to adjacent veins and nerves as described. *Giant cell arteritis* - This is a **large-vessel vasculitis** predominantly affecting the temporal arteries and other arteries originating from the aorta in individuals over 50 years of age, presenting with **headache**, **jaw claudication**, and **visual disturbances** [2]. - Histopathology reveals **granulomatous inflammation** with giant cells, not the neutrophilic infiltrate and involvement of veins/nerves seen in this case [2]. *Hypersensitivity angiitis* - This refers to **leukocytoclastic vasculitis** affecting small vessels (arterioles, capillaries, venules) and is often associated with drug reactions or systemic diseases, typically presenting with **palpable purpura** [3]. - It primarily involves small vessels and lacks the characteristic segmental thrombosing inflammation of arteries, veins, and nerves seen in the given scenario, nor is it definitively linked to smoking leading to gangrene [3]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Cardiovascular Disease, pp. 280-281. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 516-517. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Cardiovascular Disease, pp. 279-280.
Question 5: Which of the following is NOT a large vessel vasculitis?
- A. Giant cell arteritis
- B. Aortitis
- C. PAN (Correct Answer)
- D. Takayasu disease
Explanation: ***PAN*** - **Polyarteritis Nodosa (PAN)** is a **medium-sized vessel vasculitis** that causes necrotizing inflammation of the muscular arteries [4]. - It specifically spares arterioles, capillaries, and venules, distinguishing it from large vessel vasculitides [4]. - PAN typically presents with **systemic symptoms**, **renal involvement**, **peripheral neuropathy**, and **skin manifestations** [1]. *Giant cell arteritis* - **Giant cell arteritis (GCA)**, or temporal arteritis, is a **large vessel vasculitis** affecting the aorta and its major branches, particularly the temporal artery [2]. - It commonly presents in individuals over 50 years old with symptoms like **headache**, **jaw claudication**, and **vision loss** [3]. *Aortitis* - **Aortitis** refers to inflammation of the aorta, which is the body's largest artery, making it by definition a **large vessel vasculitis**. - It can be seen in conditions like **Takayasu arteritis** [3] or **syphilis**, affecting the vessel wall. *Takayasu disease* - **Takayasu arteritis** is a chronic inflammatory condition primarily affecting the **aorta** and its main branches, classifying it as a **large vessel vasculitis** [3]. - It often affects young women and can lead to **stenosis** or **aneurysm formation** in the affected vessels [3]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Osteoarticular And Connective Tissue Disease, pp. 687-688. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 516-517. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Osteoarticular And Connective Tissue Disease, pp. 688-689. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 517-518.
Question 6: A 65-year-old patient presents with severe headache, temporal artery tenderness, and decreased pulse. What is the most likely diagnosis?
- A. Giant cell arteritis (Correct Answer)
- B. Wegener's granulomatosis
- C. Microscopic polyangiitis
- D. Takayasu arteritis
Explanation: ***Giant cell arteritis*** - This presentation with **severe headache**, **temporal artery tenderness**, and a **decreased pulse** in a 65-year-old patient is highly classic for giant cell arteritis (GCA). GCA characteristically affects **medium and large arteries**, often the **temporal artery**. - **Decreased pulse** can indicate involvement of other large vessels, such as the subclavian artery, which can occur in GCA. Urgent diagnosis and treatment are crucial due to the risk of **permanent vision loss** [1]. *Wegener's granulomatosis* - This condition (**granulomatosis with polyangiitis**) is characterized by **upper and lower respiratory tract granulomatous inflammation**, **glomerulonephritis**, and small vessel vasculitis. - While it can manifest with systemic symptoms, **temporal artery tenderness** and a **decreased pulse** are not primary features of Wegener's. *Microscopic polyangiitis* - This is a **small vessel vasculitis** that primarily affects capillaries, venules, and arterioles. - It typically presents with **glomerulonephritis** and **pulmonary capillaritis**, but without granuloma formation, and does not involve the temporal arteries or lead to a decreased pulse in the manner described. *Takayasu arteritis* - Takayasu arteritis primarily affects the **aorta and its major branches**, leading to **claudication**, **pulse deficits** in the extremities, and often occurs in **younger women**. - While it can cause a decreased pulse, it is less likely to present with **temporal artery tenderness** and severe headache in a 65-year-old, as these symptoms are more characteristic of GCA.
Question 7: A 12-year-old boy presents with weak pulses in the upper limbs, a blood pressure of 90/60 mmHg , and retinal hemorrhages. What is the most likely diagnosis?
- A. Henoch-Schönlein purpura (HSP)
- B. Polyarteritis nodosa (PAN)
- C. Takayasu arteritis (Correct Answer)
- D. Microscopic polyangiitis
Explanation: ***Takayasu arteritis*** - **Weak pulses** in the upper limbs, **lower blood pressure** (90/60 mmHg), and **retinal hemorrhages** are classic signs of Takayasu arteritis, which primarily affects the aortic arch and its major branches. [1] - This condition is also known as "pulseless disease" due to the significant narrowing of peripheral arteries, leading to diminished or absent pulses. [1] *Henoch-Schönlein purpura (HSP)* - HSP is characterized by a **palpable purpuric rash**, **arthralgia**, **abdominal pain**, and **renal involvement** (hematuria/proteinuria), none of which are explicitly mentioned here. - It typically affects **small vessels** and does not cause weak pulses in the upper limbs or systemic hypotension in this manner. *Polyarteritis nodosa (PAN)* - PAN is a **necrotizing vasculitis** of medium-sized arteries, often presenting with **fever**, **weight loss**, **myalgia**, and visceral infarcts. - While it can affect various organs, it does not typically cause the specific pattern of weak upper limb pulses and retinal hemorrhages observed here, which points to large vessel involvement. *Microscopic polyangiitis* - This is a **small-vessel vasculitis** characterized by **glomerulonephritis** and **pulmonary capillaritis**, often presenting with hemoptysis and rapidly progressive renal failure. - It does not cause the large vessel symptoms like weak upper limb pulses or significant systemic hypotension seen in the patient.
Question 8: Which condition is most likely associated with specific angiographic findings such as the rosary sign?
- A. Giant cell arteritis
- B. Polyarteritis Nodosa (Correct Answer)
- C. Kawasaki disease
- D. Takayasu arteritis
Explanation: ***Polyarteritis Nodosa*** - The **rosary sign** on angiography, characterized by alternating areas of stenosis and dilation in medium-sized arteries, is a classic finding in **Polyarteritis Nodosa (PAN)**. - This sign represents **aneurysms and stenoses** resulting from inflammatory destructive lesions in the arterial wall. *Kawasaki disease* - Primarily affects young children and causes **coronary artery aneurysms**, which appear as focal dilations, rather than the "rosary sign" pattern of multiple stenoses and dilations seen in PAN. - While it can involve other medium-sized arteries, the widespread and characteristic "rosary sign" is not typical. *Takayasu arteritis* - This condition primarily affects the **aorta and its major branches**, leading to **stenosis or occlusion** of large arteries, often described as "pulseless disease." - It does not typically present with the **venous beads-like appearance** or rosary sign found in PAN. *Giant cell arteritis* - Characterized by inflammation of **large and medium-sized arteries**, predominantly affecting the temporal arteries. - Angiographic findings often include **long segments of smooth vessel wall thickening, stenosis, or occlusion**, but not the characteristic alternating aneurysms and stenoses of the "rosary sign."
Question 9: An elderly male presents with pain in his shoulders and hands. ESR is 105 mm/L. History includes transient blindness and unilateral headache.
- A. Polyarteritis nodosa
- B. Polymyalgia rheumatica (Correct Answer)
- C. Ankylosing spondylitis
- D. Behçet syndrome
Explanation: ***Polymyalgia rheumatica*** - The combination of **shoulder and hand pain** in an elderly male, along with a **very high ESR**, is highly suggestive of polymyalgia rheumatica [1]. - **Sudden transient blindness** and **unilateral headache** are concerning for giant cell arteritis, which is often associated with polymyalgia rheumatica and requires prompt treatment. *Polyarteritis nodosa* - This is a **necrotizing vasculitis** of medium-sized arteries, often presenting with systemic symptoms, **renal involvement**, and **neuropathy**. - It typically does not cause shoulder and hand pain as the primary presenting symptom, and the transient blindness and headache are more characteristic of giant cell arteritis. *Ankylosing spondylitis* - This condition primarily affects the **axial skeleton**, causing **inflammatory back pain** and stiffness, particularly in younger individuals [2]. - It does not typically present with shoulder and hand pain, transient blindness, or a temporal headache in an elderly patient. *Behçet syndrome* - Characterized by **recurrent oral and genital ulcers**, **uveitis**, and **skin lesions**. - The presenting symptoms of shoulder and hand pain, high ESR, transient blindness, and unilateral headache are not typical features of Behçet syndrome.
Question 10: A child presented with non-blanching purpura in the lower limb and recurrent abdominal pain. Which condition is characterized by the deposition of IgA?
- A. Henoch-Schönlein purpura (Correct Answer)
- B. Granulomatosis with polyangiitis
- C. Kawasaki syndrome
- D. Takayasu arteritis
Explanation: ***Henoch-Schönlein purpura*** - This condition is a **leukocytoclastic vasculitis** [1] primarily affecting small vessels, characterized by palpable **purpura**, **abdominal pain** [2], and often **arthralgia** and **renal involvement**. - It is defined by the deposition of **IgA immune complexes** in affected small vessels, a key diagnostic feature. *Granulomatosis with polyangiitis* - This is an **ANCA-associated vasculitis** [1] characterized by granulomatous inflammation of the respiratory tract and necrotizing vasculitis, typically affecting small to medium-sized vessels. - It is associated with **antineutrophil cytoplasmic antibodies (ANCA)**, specifically **c-ANCA**, and does not involve IgA deposition. *Kawasaki syndrome* - This is an **acute febrile illness** primarily in young children [3], characterized by inflammation of medium-sized arteries, most notably the **coronary arteries**. - It presents with fever, rash, **conjunctivitis**, and **lymphadenopathy**, but not IgA deposition [3]. *Takayasu arteritis* - This is a **large-vessel vasculitis** [1] primarily affecting the **aorta** and its major branches, leading to stenoses, occlusions, and aneurysms. - It usually presents in young women with symptoms related to **ischemia** of affected organs, and is not associated with IgA deposition or purpura in the lower limbs.
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