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α1 Antitrypsin deficiency in a patient with systemic vasculitis and primary Sjögren's syndrome
  1. F D Lindström1,
  2. T Skogh1,
  3. I M C Lundström2
  1. 1Division of Rheumatology, Department of Molecular and Clinical Medicine, Faculty of Health Sciences, University Hospital, 581 85 Linköping, Sweden
  2. 2Department of Oral Medicine, University Hospital, 581 85 Linköping, Sweden
  1. Correspondence to:
    Dr F D Lindström;
    folke.lindstrom{at}swipnet.se

https://doi.org/10.1136/ard.61.10.945

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    The internal homoeostasis in man is critically dependent on regulation of proteolytic enzymes in tissues and fluids by endogenous inhibitors. α1 Antitrypsin is the most abundant protease inhibitor (Pi) in plasma, and controls tissue degradation by proteases such as trypsin, neutrophil elastase, and proteinase 3.1 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? Thus, reports have …

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