Vasculitis

Question 1

Describe large vessel vasculitis and Takayasu’s vasculitis

Answer 1

-Inflammatory involvement of aorta and its branches
-Cause unknown
-Most patients are female
-Systemic features & abdominal pain
-Symptoms related to vascular insufficiency & aneurysms

Question 2

Diagnosis of LVV

Answer 2

-Difference in blood pressure between arms
-Absent or diminished pulses
-Raised ESR and CRP
- Mild anaemia
-Autoantibodies negative
-Vascular imaging pivotal
=CT or MR Angiogram
=PET-CT

Question 3

Management of LVV

Answer 3

-Prednisolone 40-60mg /day
-Reduce dose by 10mg/month
-When at 10mg, reduce dose by 1mg/month
-Steroid sparing medicines
=Tocilizumab 8mg/ 2-weekly (unlicensed)
=Methotrexate, azathioprine, Mycophenolate (unlicensed)
-Osteoporosis prophylaxis
-Manage vascular risk factors

Question 4

Classification of systemic vasculitis

Answer 4

Antineutrophil cytoplasmic antibody (ANCA) positive
-Granulomatosis with polyangiitis (GPA)- “Wegener’s Granulomatosis”
=Microscopic polyangiitis (MPA)
=Eosinophilic granulomatosis with polyangiitis (EPGA)- “Churg-Strauss Syndrome”

Antineutrophil cytoplasmic antibody (ANCA) negative
-Polyarteritis nodosa (PAN)
-Cryoglobulinaemic vasculitis
-Behcet’s disease

Question 5

Epidemiology of systemic vasculitis

Answer 5

-Gender distribution equal for most forms
-Age range 50-80 years (Behcet’s age 20-30)
-ANCA positive vasculitides:
=GPA: 10/1,000,000
=MPA: 8/1,000,000
=EPGA: 3/1,000,000

-ANCA negative vasculitides:
=PAN: 2-8/1,000,000
=Cryoglobulinaemic vasculitis: 1/1,000,000
=Behcet’s disease 1-100 / 100,000 (geographical variation)

Question 6

Pathophysiology of ANCA vasculitis

Answer 6

-Genetic - HLA-DQ (Class II)
-Necrotising vasculitis
=Granulomas
=Nasal passages
=Upper lobes
=Kidneys
-Eosinophilic infiltrate in EGPA
-Widespread small vessel vasculitis in MPA
-Autoantibodies are pathogenic in preclinical models

Question 7

Clinical presentation of ANCA vasculitis

Answer 7

–Myalgia, arthralgia, malaise
–Cutaneous vasculitis
–Mononeuritis multiplex
–Nasal crusting, bleeding, orbital
mass (GPA)
–Dyspnoea, haemoptysis (GPA)
–Asthma (EGPA)
–Haematuria, proteinuria (GPA, MPA)

Question 8

Diagnosis of systemic vasculitis

Answer 8

-Clinical picture
-Raised ESR and CRP
-Bloods
=Eosinophilia (EGPA)
=Renal impairment
-ANCA – anti MPO, PR3
-Tissue biopsy
=Nasal passages, Kidney, Skin

Question 9

Management of systemic vasculitis

Answer 9

-Acute Management
=High dose steroids
=Cyclophosphamide or Rituximab
=Mepolizumab (anti-IL-5) in EGPA
-Longer term
=Gradually reduce steroids
=Transition to azathioprine or
methotrexate for maintenance
=Intermittent rituximab

Question 10

Describe long term follow up of systemic vasculitis

Answer 10

-Keep patients under clinical review
-Maintain immunosuppression long term
-Patients can relapse several years after initial diagnosis
-Withdraw steroids if possible
-Bone protection if on long term steroids

Question 11

What is polyarteritis nodosa

Answer 11

Vasculitis and aneurysms of medium sized vessels

Question 12

Clinical presentation of polyarteritis nodosa

Answer 12

–Abdominal pain
–Neuropathy
–Skin nodules or vasculitic rash
–Malaise, fatigue, myalgia, arthralgia
–Haematuria, Myocardial infarction
-Some cases associated with chronic hepatitis B infection

Question 13

Diagnosis of polyarteritis nodosa

Answer 13

-Raised ESR and CRP
-ANCA – negative
-Tissue biopsy
=Kidney, Skin, muscle, sural nerve
-Mesenteric or renal angiogram
-Check for Hepatitis B

Question 14

Management of polyarteritis nodosa

Answer 14

-Acute Management
=High dose steroids
=Cyclophosphamide
=Treat hepatitis B (if applicable)
=Plasma exchange
-Longer term
=Gradually reduce steroids
=Transition to azathioprine or methotrexate

Question 15

What is cryoglobulinaemic vasculitis?

Answer 15

Immune complex deposition in blood vessels stimulating an inflammatory reaction

Question 16

Predisposing factors to cryoglobulinaemic vasculitis

Answer 16

–Myeloma, Waldenstrom’s macroglobulinaemia
–Chronic inflammatory disease
–Hepatitis C infection

Question 17

Management of cryoglobulinaemic vasculitis

Answer 17

–Treat underlying condition where possible
–Glucocorticoids and immunosuppressive therapy
–Rituximab

Question 18

Describe Behcet’s disease

Answer 18

-Most common in people of middle-eastern descent
(highest incidence worldwide in Turkey)
-Genetic Association with HLA-B51

Question 19

Clinical presentation of Behcet’s disease

Answer 19

–Oral and genital ulcers,
–Uveitis
–Pustular skin lesions, erythema nodosum
–Arthritis & arthralgia
–Venous thrombosis
–Neurological involvement

Question 20

Diagnosis of Behcet’s disease

Answer 20

-Diagnosis is clinical – oral or genital ulceration almost always present
-Autoantibodies negative
-Raised ESR and CRP in patients with active inflammatory disease
-Pathergy test – development of pustule at site of needle puncture
-Imaging may show evidence of venous thrombosis and cerebral vasculitis or infarction

Question 21

Management of Behcet’s disease

Answer 21

-Topical steroids and colchicine for oral and genital ulcer
-Systemic steroids and immunosuppressive therapy for vasculitis and venous thrombosis
-Systemic steroids and immunosuppressive therapy for uveitis
-Anti-TNF therapy for resistant patients