Vasculitic phase

This phase has a mean age of onset of 38 years. It often commences with malaise, fever and weight loss. Frequently, the patient complains of leg cramps.

Specific features of the vasculitic phase depend upon the organ system involved:

• skin - rash (palpable purpura, erythema or urticaria) in 70% of patients, subcutaneous nodules forming from confluent granulomas
  
  
• cardiac - may present as a pericarditis or abnormal ECG
  
  
• pulmonary - may present as nodular infiltrates but, unlike in Wegener's granulomatosis, they rarely cavitate
  
  
• renal - characterized by a focal segmental glomerulonephritis
  
  
• nervous system - mononeuritis multiplex, diffuse symmetric neuropathy
  
  
• gastrointestinal - diarrhoea, bleeding
  
  
• locomotor - arthritis, arthralgia, myalgia