15th July 2024

Diagnosis and Management

If the clinical situation allows, renal imaging followed by biopsy is the gold standard method for diagnosing renal involvement in vasculitis.

A positive ELISA PR3/MPO autoantibody screen, in combination with clinical suspicion and signs, such as haematuria and acute kidney injury (AKI), is highly sensitive for ANCA associated renal disease. It must be noted that false positive ANCA screens are seen in many other inflammatory conditions, such as lupus nephritis or chronic infections.

Magnetic resonance angiography of the kidneys is sensitive for detection of microaneurysms in PAN.  In addition, testing and treatment for hepatitis B positive patients with PAN should not be forgotten.

The standard regimes for inducing remission are high dose glucocorticoids in combination with either pulsed cyclophosphamide or rituximab. Plasma exchange may be considered on an individual basis in refractory disease. Following successful induction of remission, steroid sparing agents such as azathioprine can be added as maintenance therapy.

Renin-angiotensin systemic blockade is suggested in the first instance for IgA vasculitis with the addition of corticosteroids in the case of persistent proteinuria.

Unfortunately, despite best efforts and interventions, a number of patients will progress to end stage renal disease requiring haemodialysis/transplant.