Granulomatosis with polyangitis

Question 1

What is granulomatosis?

Answer 1

A multi system disorder of unknown cause characterised by necrotising granulomatous inflammation and vasculitis of small and medium vessels. It normally affects the lungs, kidneys and upper respiratory tract

Question 2

What will you find in a history of granulomatosis?

Answer 2

Symptoms: Triad of upper and lower respiratory tract disease with glomerulonephritis
Upper repiratory disease - nasal ulcers, nose bleeds, ulcers, destruction of the nasal septum causing saddle nose deformity, sinusitis
Renal disease - Rapid glomerulonephritis with haematuria and proteinurua
Pulmonary disease - cough, haemoptysis, pleuritis
Typically have non-specific symptoms for 2-3 months before presentation.- Skin pupura, nodules, peripheral neuropathy, mononeuritis, Arthiritis, ocular involvement (conjunctivitis, uveitis, sclerosis, episcleritits)

Question 3

What will you find on examination of Granulomatosis?

Answer 3

End of the bed:
Weight loss.
Fever
Skin Nodules
Haemorrhagic and ulcerative skin lesions
Joint tenderness or swelling
Muscle weakness

Hands:
Splinter Haemorrhages

Neck:

Face:
URTI Ulceration
Saddle nose deformity
Sinus tenderness
Reduced visual acuity
Red eyes

Chest:
Focal dullness to percussion
Crackles

Abdomen:

Legs:
Look for signs of VTE - Patients are at high risk of VTE

Question 4

What investigations will you order in granulomatosis?

Answer 4

Bedside:
Urinalysis - haematuria/proteinuria

Bloods:
ESR/CRP - raised
FBC - Anaemia due to renal involvement, pulmonary haemorrhage or chronic disease

Imaging:
CXR - Pulmonary nodules or pulmonary haemorrhage (fluffy infiltrates)
CT - if positive CXR ( Diffuse alveolar haemorrhage)

Special tests:
cANCA 
pANCA 
Renal biopsy - if positive urinalysis 
Respiratory function tests - elevated diffusion capacity is suggestive of pulmonary haemorrhage

Question 5

What is the treatment of granulomatosis?

Answer 5

Medical:
Severe disease - Corticosteroids and cyclophosphamide to induce remission
Moderate - Corticosteroids and methotrexate to induce remission
Azathioprine and methotrexate as maintenance
Co-trimoxazole as prophylaxis against pneumocystis jirovecii and staphylococcal infection