Vasculitides

Question 1

Presentation of GCA

Answer 1

Primarily >50

Assoc w/ polymyalgia rheumatica

Acute-onset blindness

Jaw claudication

Headache/pain when brushing hair - acutely tender infarcting scalp

Raised ESR/platelets

Question 2

Management of GCA

Answer 2

1mg/kg prednisolone PO up to 60mg

Consider alendronate, PPI (bone + gastro protection), aspirin (to protect second eye)

Question 3

Presentation of Takayasu arteritis

Answer 3

Japanese women

<50yo

“pulseless” disease > apparent hypertension

Subclavian bruit

Claudication

Ocular disturbance

Constitutional symptoms: fever, fatigue, weight loss, night sweats

Question 4

Management of large/medium vessel vasculitis

Answer 4

High-dose immunosuppression

Likely MR angiography

Question 5

Demographics of polyarteritis nodosa

Answer 5

M:F 2:1

Assoc w/ HBV infection (check serology)

Question 6

Presentation of polyarteritis nodosa

Answer 6

Constitutional symptoms

Sterile orchitis

Myalgia (esp. back/flank pain)

Levido reticularis (esp in non-cold areas)

Ischaemia:

• Mononeuritis multiplex
• punched out skin ulcers
• mesenteric angina post-prandially

Question 7

Presentation of Kawasaki

Answer 7

AFI in children (<4yo)

Strawberry tongue, widespread erythema, palmar rash

Lymphaenopathy

Fatal complication: coronary arteritis > ruptured aneurysm

Question 8

Management of Kawasaki

Answer 8

IV Ig and aspirin

Question 9

ANCA +ve small-vessel vasculitides

Answer 9

Wegener’s (aka GPA)

Microscopic polyangiitis

Question 10

ANCA -ve small-vessel vasculitides

Answer 10

Henoch-Schonlein purpura

Churg-Strauss (NB: may be ANCA +ve)

Cryoglobulinaemia

Question 11

Microscopic polyangiitis presentation

Answer 11

Rapidly progressive glomerulonephritis +/- pulmonary haemmorhage

Rare: GI pain, arthralgia, peripheral neuropathy

Question 12

Investigations in microscopic polyangiitis

Answer 12

Renal biopsy

pANCA (MPO) +ve

Question 13

Management of microscopic polyangiitis

Answer 13

Steroids + cyclophosphamide/methotrexate/rituximab/azathioprine

Question 14

Presentation of GPA (aka Wegener’s)

Answer 14

Age: 40s-70s

Upper ariway: epistaxis, sinusitis, ulcers, blockage

Lower airway: Cough, haemoptysis, pleuritis, tracheal stenosis

Renal: Rapidly progressive glomerulonephritis

Other: Skin, nerves, joints

Question 15

Investigations in GPA

Answer 15

ESR/CRP

cANCA

CXR

?renal biopsy

Question 16

Presentation of Henoch-Schlonein purpura

Answer 16

Young males

IgA mediated

Purpura (Esp over extensor surfaces)

Arthritis

abdo pain (+/- intususseption)

glomerulonephritis

Question 17

Management of Henoch-Schlonein purpura

Answer 17

Supportive

Question 18

Presentation of Churg-Strauss syndrome

Answer 18

Adult-onset asthma

Eosinophilia

Vasculitis: lungs, renal, heart, skin

May present with septic-shock/SIRS picture

Question 19

Presentation of cryoglobulinaemic vasculitis

Answer 19

(Palpable!) Purpura

Arthralgia

Glomerulonephritis

High RF, low C3/C4

Assoc w/ HepC

Question 20

Presentation of polymyalgia rheumatica

Answer 20

Age >50

Subacute (2-week) onset

bilateral aching, tenderness, stiffness

Weight loss, anorexia

Depression

No weakness!

Question 21

Muscle groups affected in PMR

Answer 21

Hips, shoulders, Proximal limb

Question 22

Investigations in PMR

Answer 22

High ESR/CRP

AlkP may be raised

CK levels normal (c.f. myopathies, myositis)

Question 23

Presentation of fibromyalgia

Answer 23

Chronic (>3mo) pain + morning stiffness

Widespread (bilateral, upper + lower + axial)

Profound fatigue, sleep disturbance

Low mood, poor concentration

Headaches

Question 24

Management of fibromyalgia

Answer 24

Graded exercise programmes

Amitryptilline/pregabalin for sleep

CBT/treatment of comorbid depression