Extremely rare: c-ANCA vasculitis (granulomatosis with polyangiitis)

(formerly Wegener's granulomatosis or Wegener's disease) This inflammation of small and very small blood vessels is caused by autoantibodies in the blood.

Granulomas, nodular tissue growths, are found in the upper and lower respiratory tract. Bloody deposits may be visible on the nasal mucosa. In advanced stages, the kidneys are involved in 80% of affected individuals. c-ANCA vasculitis is a very rare comorbidity in alpha-1 antitrypsin deficiency. If corresponding symptoms are present, the c-ANCA level in the blood should be determined in patients with AATD.

Summary: Prof. Gratiana Steinkamp, as published in Alpha1 Journal 1-2018.