Males are affected more than females (1.2:1). Non-white males are virtually never affected. Peak incidence is in the fifth decade but it may occur in young children.
Ganulomatosis with polyangiitis (GPA) is characterised by necrotising granulomata of the upper and lower respiratory tract, necrotising vasculitis and focal glomerulonephritis
Presentation is with:
- respiratory features
- a more limited form, with lesions limited to the upper and lower respiratory tract, can occur
- upper-airways disease is the most common presenting feature, occurring in more than 70% of patients at presentation, and develops in >90% of patients
- nasal disease presents with obstruction, nasal ulcers and
- septal perforation, serosanguinous discharge or epistaxis
- destruction of the nasal septum results in the typical saddle-nose deformity
- sinusitis is present in 85% at some time during the disease
- laryngotracheal disease may be asymptomatic, but can present with hoarseness or stridor and upper-airways obstruction
- pulmonary involvement is one of the main features of GPA, occurring in 45% at presentation and 87% during the course of the disease
- cough, haemoptysis and pleuritis are most common
- most frequent radiological features are pulmonary infiltrates, haemorrhage and hilar lymphadenopathy
- renal features
- renal disease is a feature of GPA, occurring in 18% of patients at presentation and in 77% subsequently
- patients may present with life-threatening renal failure due to rapidly progressive glomerulonephritis requiring urgent dialysis and immunosuppression
- at the other end of the spectrum are patients with proteinuria or haematuria with no impairment of renal function
- any renal involvement is associated with a significantly worse outcome
- vascular lesions resembling those of polyarteritis nodosa - nodules and ulceration
- cutaneous manifestations occur in around 50% of patients, and include ulcers, palpable purpura, papules and nodules
- fever, malaise, weight loss
- neurological involvement with peripheral neuropathy or mononeuritis multiplex is less common at presentation but can be detected (often subclinically) in <=50% of patients with time
- ocular disease occurs in 50%, with any compartment of the eye potentially being affected - keratitis, conjunctivitis, scleritis, episcleritis, uveitis, retro-orbital pseudotumour, retinal vessel occlusion and optic neuritis
- visual loss is reported in <=8% of patients
- musculoskeletal symptoms are common, with most patients experiencing arthralgias and/or myalgias
- a true synovitis can be seen in 25% of patients.
Reference:
- 1) ARC Autumn 2012. Topical Reviews - ANCA-associated vasculitis; 1:1-12.