Wegener’s Granulomatosis

Question 1

What is Wegener’s Granulomatosis also known as?

Answer 1

Granulomatosis w polyangiitis

Question 2

Wegener’s granulomatosis is a multi-system disease characterised by the triad of

1) Systemic necrotising vasculitis of the ___________
2) Necrotising granulomatous inflammation of the ______________
3) Necrotising ______________

Therefore, WG can result in ______________

Answer 2

small and medium-sized vessels;

upper and lower respiratory tract;

glomerulonephritis;

Pulmonary Renal Syndrome (GN/Renal Failure + Alveolar Haemorrhage)

Question 3

What are the clinical presentations of Wegeners granulomatosis?

Answer 3

ENT symptoms (separates WG from MPA) e.g. nose bleeds, sensorineural deafness and neuropathy

Constitutional symptoms of LOW, LOA, Fever generally precede onset of prominent organ involvement.

URTI

• Bloody/purulent nasal discharge, Epistaxis
• Nasal perforation
• Saddle-Nose Deformity (due to destruction from chronic inflammation)
• Chronic sinusitis: causing chronic pain
• Oral ulcers
• Tracheal Collapse

LRTI

• Haemoptysis 2’ diffuse alveolar haemorrhage -> may be life-threatening
• CP, SOB, dyspnea

Renal: Glomerulonephritis hence

• Haematuria
• Proteinuria

Question 4

What are the investigations of Wegeners granulomatosis?

Answer 4

• ESR, CRP raised
• c-ANCA +ve (PR3)
• UFEME – haematuria, RBC cell casts, proteinuria
• Granulomatous inflammation on biopsy (within wall of artery / perivascular / extravascular area), predominant neutrophilic (instead of eosinophilic)

Question 5

What is the treatment of Wegeners granulomatosis?

Answer 5

• Limited -> GC + MTX
• Severe (immediate threat to life or vital organ) -> GC + cyclophosphamide/ rituximab
• Additional prophylactic treatment for PCP (Co-Trimoxazole): due to immunosuppression by cyclophosphamide, it is of value to also give concurrent PCP prophylaxis