Alpha-1 anti-trypsin deficiency and Henoch-Schönlein purpura associated with anti-neutrophil cytoplasmic and anti-endothelial cell antibodies of immunoglobulin-A isotype Academic Article uri icon


MeSH Major

  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies
  • Immunoglobulin A
  • Immunoglobulin Isotypes
  • Purpura, Schoenlein-Henoch
  • alpha 1-Antitrypsin Deficiency


  • Alpha-1 anti-trypsin (A1AT) deficiency is an inherited enzyme deficiency that manifests with fatal lung and liver complications. In addition to pulmonary and hepatic involvement, the disease has also been linked to an increased incidence of vasculitic syndromes and autoimmune diseases, including Wegener's granulomatosis, microscopic polyarteritis nodosa and Henoch-Schonlein purpura (HSP). HSP, a systemic, small-vessel vasculitis syndrome, is characterized by a non-thrombocytopaenic purpuric rash, arthralgia, abdominal pain and nephritis. Both A1AT deficiency and HSP have been associated with anti-neutrophil cytoplasmic antibodies (ANCA) and anti-endothelial cell antibodies (AECA). We report a case of a 40-year-old man with severe A1AT deficiency, who developed HSP associated with AECA, ANCA and anti-phospholipid antibodies of the immunoglobulin-A isotype.

publication date

  • April 2005



  • Academic Article



  • eng

Digital Object Identifier (DOI)

  • 10.1111/j.0303-6987.2005.00304.x

PubMed ID

  • 15769280

Additional Document Info

start page

  • 300

end page

  • 6


  • 32


  • 4