Rheumatology (3)

Question 1

Demographics (epidemiology) of Giant Cell Arthritis

Answer 1

• Common in elderly (rare <55)
• Associated with Polymyalgia Rheumatica in 50%

Question 2

Features of Temporal Arteritis

Answer 2

• Systemic signs: fever, malaise, fatigue
• Headache
• Temporal artery and scalp tenderness
• Jaw claudication
• Amaurosis fugax
• Prominent temporal arteries ± pulsation

Question 3

Management and Ix of Temporal arteritis

Answer 3

If suspected Do ESR and start prednisolone 40-60mg/d PO

• ↑↑ESR and CRP
• ↑ALP
• ↓Hb (normo normo), ↑Plats
• Temporal artery biopsy: but skip lesion occur

Continuing Rx

• Taper steroids gradually, guided by symptoms and ESR
• PPI and alendronate cover (~2yr course usually)

Question 4

Presentation of Polymyalgia Rheumatica

Answer 4

• >50yrs old
• Severe pain and stiffness in shoulders, neck and hips
• Sudden / subacute onset
• Symmetrical
• Worse in the morning: stops pt. getting out of bed
• No weakness (different from myopathy or myositis)
• ± mild polyarthritis, tenosynovitis and carpal tunnel syndrome
• Systemic signs: fatigue, fever, wt. loss
• 15% develop Giant Cell Arteritis

Question 5

Ix of Polymyalgia Rheumatica

Answer 5

• ↑↑ESR and CRP (+ ↑plasma viscosity)
• ↑ALP
• Normal CK

Question 6

Management of Polymyalgia Rheumatica

Answer 6

• Prednisolone 15mg PO: taper according to symptoms and ESR

 PPI and alendronate cover (~2yr course usually)

Question 7

Another name for Takayasu’s Arteritis

Answer 7

Pulseless Disease

Question 8

Epidemiology of Takayasu’s arteritis

Answer 8

• Female Japan/Asian
• Sex: F>M
• Age: 20-40yrs

Question 9

Features of Takayasu’s Arteritis

Answer 9

• Constitutional symptoms: fever, fatigue, wt. loss
• Weak pulses in upper extremities
• Visual disturbance
• HTN

Question 10

Management of Takayasu’s arteritis

Answer 10

Steroids

Question 11

Epidemiology of Polyarteritis Nodosa

Answer 11

• rare in UK
• M>F=2:1
• young adults

Question 12

What’s Polyarteritis Nodosa

Answer 12

• vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation
• more common in middle-aged men and is associated with hepatitis B infection

Question 13

Features of Polyarteritis Nodosa

Answer 13

• fever, malaise, arthralgia
• weight loss
• hypertension
• mononeuritis multiplex, sensorimotor polyneuropathy
• testicular pain
• livedo reticularis
• haematuria, renal failure
• perinuclear-antineutrophil cytoplasmic antibodies (ANCA) are found in around 20% of patients with ‘classic’ PAN
• hepatitis B serology positive in 30% of patients

Question 14

Management of Polyarteritis Nodosa

Answer 14

Prednisolone + cyclophosphamide

Question 15

What’s Wegener’s Granulomatosis?

Answer 15

• Necrotizing granulomatous inflammation and small-vessel vasculitis
• URTI, LRTI and Kidneys problems

Question 16

Features of Wegener’s Granulomatosis

Answer 16

URTI

• Chronic sinusitis
• Epistaxis
• Saddle-nose deformity

LRTI

• Cough
• Haemoptysis
• Pleuritis

Renal

• Rapidly Progressing Gromeluronephritis
• Haematuria and proteinuria

Other

• Palpable purpura
• Ocular: conjunctivitis, keratitis, uveitis

Question 17

Ix for Wegener’s Granulomatosis

Answer 17

• cANCA
• Dipstick: haematuria and proteinuria
• CXR: bilateral nodular and cavity infiltrates

Question 18

Another name for Wegener’s Granulomatosis

Answer 18

Granulomatosis with polyangitis

Question 19

Management of Wegener’s Granulomatosis

Answer 19

• steroids
• cyclophosphamide (90% response)
• plasma exchange
• median survival = 8-9 years

Question 20

Granulomatosis with polyangiitis vs Churg-Strauss

Answer 20

Question 21

Another name for Churg-Strauss syndrome

Answer 21

Eosinophilic granulomatosis with polyangiitis

Question 22

What’s Churg-Strauss Syndrome?

Answer 22

ANCA associated small-medium vessel vasculitis

Question 23

Features of Churg - Strauss syndrome

Answer 23

• late onset asthma
• blood eosinophilia (e.g. > 10%)
• paranasal sinusitis
• mononeuritis multiplex
• pANCA positive in 60%

Leukotriene receptor antagonists may precipitate the disease

Question 24

What’s Henoch-Schonlein Purpura

Answer 24

• IgA mediated small vessel vasculitis
• degree of overlap with IgA nephropathy (Berger’s disease)
• usually seen in children following an infection

Question 25

Features of Henoch-Schonlein purpura

Answer 25

• Children 3-8yrs
• Post-URTI
• Palpable purpura on buttocks
• Colicky abdo pain
• Arthralgia
• Haematuria

Question 26

Management of Henoch-Schonlein purpura

Answer 26

• analgesia for arthralgia
• treatment of nephropathy is generally supportive
• inconsistent evidence for the use of steroids and immunosuppressants (?)

Question 27

Prognosis in Henoch Schonlein Purpura

Answer 27

• usually excellent, HSP is a self-limiting condition, especially in children without renal involvement
• around 1/3rd of patients have a relapse

Question 28

What’s Goodpasture Syndrome?

Answer 28

• rare condition associated with both pulmonary haemorrhage and rapidly progressive glomerulonephritis
• caused by anti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen

Question 29

Epidemiology of Goodpasture syndrome

Answer 29

• more common in men (sex ratio 2:1)
• bimodal age distribution (peaks in 20-30 and 60-70 age bracket)
• associated with HLA DR2

Question 30

Features of Goodpasture syndrome

Answer 30

• pulmonary haemorrhage
• followed by rapidly progressive glomerulonephritis

Question 31

Ix in Goodpasture Syndrome

Answer 31

• renal biopsy: linear IgG deposits along the basement membrane
• raised transfer factor secondary to pulmonary haemorrhages
• X-ray: bilateral lower zone infiltrates (due to pulmonary haemorrhage)

Question 32

Management of Goodpasture syndrome

Answer 32

• plasma exchange (plasmapheresis)
• steroids
• cyclophosphamide