[alpha]1 Antitrypsin deficiency in a patient with systemic vasculitis and primary Sjögren's syndrome

1 Homozygous α1 antitrypsin deficiency is known to predispose to emphysema and chronic liver disease. 2, 3 Recently, a strong correlation has been found between systemic small vessel necrotising vasculitis and both heterozygous and homozygous α1 antitrypsin deficiency. 4 Also, such deficiency has been found to confer a more disseminated disease and worse prognosis to patients with antineutrophil cytoplasmic antibody (ANCA) positive vasculitis. 5 However, there is disagreement about the clinical implication of an intermediate α1 antitrypsin deficiency: is it an accidental finding or does it imply susceptibility to autoimmune disease? [...]reports have demonstrated an increased incidence of α1 antitrypsin deficiency in patients with acute anterior uveitis, a finding that was refuted by others. 6 Similarly, several reports have suggested an increased frequency of α1 antitrypsin deficiency in patients with rheumatoid arthritis, whereas others found no such association in similar patients. 7 CASE REPORT Here, we report a severe vasculitic episode in a patient with primary Sjögren's syndrome (pSS) and heterozygous α1 antitrypsin deficiency. [...]during the inflammatory episode, α1 antitrypsin levels remained normal, while other acute phase proteins were raised. [...]a genetically determined heterozygous α1 antitrypsin deficiency was suspected .