Granulomatosis with Polyangitis (Wegner's Granulomatosis) Flashcards
What is Granulomatosis with polyangitis?
Wegener’s Granulomatosis
A systemic disorder that involves both granulomatosis and polyangiitis. It is a form of vasculitis (inflammation of blood vessels) that affects small- and medium-size vessels in many organs.
What is the pathophysiology of Granulomatosis with polyangitis?
- Necrotising granulomatous inflammation
- Usually involving the upper and lower respiratory tract
- Affecting predominantly small to medium vessels
- Necrotising glomerulonephritis is common
What are the ENT features of Wegener’s Granulomatosis?
- Epistaxis
- Nasal obstruction
- Nasal septum destruction - saddle nose deformity
What are pulmonary features of Wegener’s?
- Cough
- Haemoptysis
- Sinnusitis
- Pleuritis
What GI features can present in Wegener’s Granulomatosis?
Mouth Ulcers
What are skin features of Wegener’s Granulomatosis?
- Purpura - Vasculitic rash
What are neurological features of Wegener’s Granulomatosis?
- Peripheral neuropathy
- Mononeuritis multiplex
What are musculoskeletal features of WEgener’s?
- Arthralgia
- Arthritis
What are eye features of Wegener’s?
- Keratitis
- Conjunctivitis
- Scleritis
- Episcleritis
- Uveitis
What renal features can occur in Wegener’s?
- Proteinuria
- Haematuria
What renal pathology most commonly occurs in Wegener’s?
Rapidly progressive glomerulonephritis
What Blood test is specific for Wegeners?
c-ANCA for PR3
What Tests would you do if you suspected vasculitis?
- Bloods - ESR, CRP, FBC, U+E’s, LFTs, ANCA, ANA
- Urinalysis
- CXR
- Renal Biopsy
