Vasculitis

Question 1

What is Microscopic Polyangiitis (MPA)?

Answer 1

A necrotizing small-vessel vasculitis, strongly associated with MPO-ANCA (p-ANCA), that primarily affects the kidneys and lungs, without granuloma formation.

Question 2

How is MPA different from GPA?

Answer 2

MPA lacks granulomas and upper airway involvement. GPA has granulomatous inflammation and often involves sinuses and lungs with cavitations.

Question 3

What are common renal features of MPA?

Answer 3

Rapidly progressive glomerulonephritis (RPGN) with hematuria and proteinuria.

Question 4

What pulmonary feature is seen in MPA?

Answer 4

Pulmonary capillaritis causing alveolar hemorrhage → hemoptysis, dyspnea, infiltrates.

Question 5

What constitutional symptoms occur in MPA?

Answer 5

Fever, weight loss, fatigue.

Question 6

What skin features are associated with MPA?

Answer 6

Palpable purpura, livedo reticularis.

Question 7

What nervous system findings may occur in MPA?

Answer 7

Mononeuritis multiplex, peripheral neuropathy.

Question 8

What ANCA pattern is typically seen in MPA?

Answer 8

p-ANCA (MPO-ANCA) positive in ~70–80% of cases.

Question 9

What biopsy finding is typical in MPA?

Answer 9

Pauci-immune necrotizing vasculitis without granulomas.

Question 10

What will urinalysis show in MPA?

Answer 10

RBC casts, hematuria, proteinuria.

Question 11

What is the induction treatment for MPA?

Answer 11

High-dose corticosteroids + cyclophosphamide or rituximab.

Question 12

What is the role of plasmapheresis in MPA?

Answer 12

Considered in severe renal disease or alveolar hemorrhage.

Question 13

What is used for maintenance therapy in MPA?

Answer 13

Azathioprine, methotrexate, or mycophenolate.

Question 14

What organs are less involved in MPA compared to PAN?

Answer 14

GI and mesenteric vessels are less commonly involved in MPA.

Question 15

What is the prognosis of MPA with treatment?

Answer 15

5-year survival >75%; relapses are common.

Question 16

What is Takayasu arteritis?

Answer 16

A granulomatous large-vessel vasculitis affecting the aorta and its major branches, primarily in young women (<40 years), especially in Asia.

Question 17

What is another name for Takayasu arteritis?

Answer 17

“Pulseless disease.”

Question 18

What are systemic symptoms in early Takayasu?

Answer 18

Fever, malaise, weight loss, night sweats.

Question 19

What vascular symptoms define Takayasu?

Answer 19

Arm claudication, asymmetric BP, diminished or absent pulses, bruits over carotid or subclavian arteries.

Question 20

What eye symptom may occur in Takayasu?

Answer 20

Visual disturbances from ischemia or hypertension-related retinopathy.

Question 21

What neurologic symptoms can occur?

Answer 21

Dizziness, syncope, strokes due to cerebral ischemia.

Question 22

What imaging is diagnostic in Takayasu arteritis?

Answer 22

CT angiography or MR angiography – shows narrowing, occlusion, or aneurysms in the aorta and branches.

Question 23

What blood test abnormalities are seen?

Answer 23

Elevated ESR and CRP; ANCA is negative.

Question 24

What is first-line treatment in active Takayasu?

Answer 24

High-dose steroids (e.g., prednisolone 1 mg/kg/day).

Question 25

What steroid-sparing agents may be used?

Answer 25

Methotrexate, azathioprine, or mycophenolate.

Question 26

When is surgery indicated in Takayasu?

Answer 26

For critical stenoses or aneurysms – e.g., angioplasty or bypass.

Question 27

What pulse abnormality is characteristic?

Answer 27

Asymmetric or absent upper limb pulses; BP discrepancy >10 mmHg between arms.

Question 28

What cardiac complications may arise?

Answer 28

Aortic regurgitation, hypertension from renal artery stenosis.

Question 29

What population is most affected by Takayasu?

Answer 29

Young Asian women under 40.

Question 30

How does Takayasu differ from Giant Cell Arteritis (GCA)?

Answer 30

Takayasu = young, large-vessel (aorta); GCA = older adults, cranial arteries.

Question 31

What is Polyarteritis Nodosa (PAN)?

Answer 31

A necrotizing medium-vessel vasculitis affecting visceral arteries, without glomerulonephritis or ANCA, and spares the lungs.

Question 32

What infection is strongly associated with PAN?

Answer 32

Hepatitis B virus (up to 30% of cases).

Question 33

What skin findings occur in PAN?

Answer 33

Livedo reticularis, palpable purpura, nodules, ulcers.

Question 34

What renal manifestations occur in PAN?

Answer 34

Hypertension, renal infarcts or ischemia (not glomerulonephritis).

Question 35

What GI symptoms occur in PAN?

Answer 35

Abdominal pain after meals, nausea, bleeding, bowel infarction or perforation.

Question 36

What neurologic symptoms are seen in PAN?

Answer 36

Mononeuritis multiplex (e.g., wrist or foot drop).

Question 37

What are systemic features of PAN?

Answer 37

Fever, weight loss, arthralgia, myalgia.

Question 38

What blood test findings are seen in PAN?

Answer 38

Elevated ESR/CRP, normal ANCA, may have positive Hep B serology.

Question 39

What imaging modality shows aneurysms in PAN?

Answer 39

CT angiography or mesenteric arteriography – shows “string of beads” from microaneurysms.

Question 40

What is found on biopsy in PAN?

Answer 40

Transmural necrotizing inflammation of medium-sized arteries.

Question 41

What is the treatment for PAN without Hep B?

Answer 41

High-dose steroids; cyclophosphamide in severe cases.

Question 42

How is PAN associated with Hepatitis B treated?

Answer 42

Antiviral therapy, short course steroids, plasma exchange.

Question 43

Is there glomerulonephritis in PAN?

Answer 43

No – kidneys affected via infarction/ischemia, not GN.

Question 44

Does PAN involve the lungs?

Answer 44

No – unlike GPA and MPA, PAN spares pulmonary vessels.

Question 45

What is the prognosis of untreated PAN?

Answer 45

Poor – 5-year survival <20%; improves significantly with immunosuppression.