Vasculitis

Question 1

Granulomatosis with Polyangitis (GPA)

Answer 1

Small or medium sized vessel inflammation causing pulmonary-renal syndrome
Upper & lower respiratory tract invariably involved; symptoms include
- nasal congestion
- rhinorrhoea
- bloody nasal discharge
- cough ( initially dry, may become haemoptysis)
- breathlessness

Question 2

Giant cell arteritis

Answer 2

Patients usually sixth, seventh or eighth decade
~20% patients with polymyalgia rheumatica develop GCA
Specific symptoms:
- severe bitemporal headache
- Visual disturbance or visual loss
- jaw claudication (quite specific), scalp tenderness or tongue claudication

Question 3

Polyarteritis Nodosa

Answer 3

Medium sized arteries

Specific symptoms depend on which arteries ivoved - coronary arteries, mesenteric arteries, renal arteries

Question 4

Eosinophilic granulomatosis with Polyangiitis (EGPA)

Answer 4

Small vessels causing a pulmonary-renal syndrome
Asthma first then a peripheral eosinophilia
Ask about previous history of:
- asthma
- allergic rhinitis, nasal polyps
- eczema
- cough and breathlessness
- peripheral nervous system disease - symmetrical peripheral neuropathy or mononeuritis multiplex

Question 5

Microscopic polyarteritis (polyangiitis)

Answer 5

Small vessels causing a pulmonary-renal syndrome
Renal impairment
Lung disease
Systemic symptoms of vasculitis

Question 6

Mixed essential cryoglobulinaemia

Answer 6

Small vessels due to rheumatoid factor bound to IgG
Present with 
- palpable purpura of the extremities 
- Raynaud’s disease
- arthritis 
- neuropathy 
Hep C is common

Question 7

DDx for livedo reticularis

Answer 7

Vasculitis
Cholesterol atheroembolism after a vascular procedure
Antiphospholipid syndrome

Question 8

Examination- Granulomatosis with Polyangitis

Answer 8

Classical collapse of the nasal septum
Tachypnoea & crackles throughout the lung
Fluid overload in worsening renal failure

Question 9

Examination - Giant Cell Arteritis

Answer 9

Tender & indurated temporal arteries
Abnormal eye signs if there is retinal investigations
Focal neurological signs

Question 10

Examination- polyarteritis nodosa

Answer 10

Painful skin nodules
Palpable purpura
Livedo reticularis
Mononeuritis multiolex

Question 11

Examination - Eosinophilic granulomatosis with Polyangitis

Answer 11

Signs of allergic disease e.g. swollen nasal turbinates, nasal polyps, bronchospasm
Peripheral nervous system- look for single nervous lesions or symmetrical peripheral neuropathy involving the lower limbs

Question 12

Examination - Microscopic polyarteritis

Answer 12

Urinalysis - proteinuria or haematuria

Question 13

Examination- mixed essential cryoglobulinaemia

Answer 13

Raynaud’s disease
Palpable purpura
Peripheral neuropathy
Signs of liver disease (Hepatitis C)

Question 14

Investigations- granulomatosis with Polyangitis

Answer 14

CRITICAL anti-neutrophil cytoplasmic antibody (c-ANCA)

Anti-pr3 antibodies

Question 15

Investigations- EGPA

Answer 15

50% patients will have a positive perinuclear (p) ANCA test
Tissue biopsy will reveal intense eosinophilia
Sural nerve biopsy (in setting of neuropathy) will show vasculitis

Question 16

Investigations - microscopic polyarteritis

Answer 16

Positive p-ANCA

Renal biopsy

Question 17

Cyclophosphamide

Answer 17

Monitor blood count
Counsel re adequate fluid intake & to avoid haemorrhagic cystitis
Annual urine analysis once treatment is ceased - haematuria may indicate the development of bladder Ca