Title | Alpha-1 anti-trypsin deficiency and Henoch-Schönlein purpura associated with anti-neutrophil cytoplasmic and anti-endothelial cell antibodies of immunoglobulin-A isotype. |
Publication Type | Journal Article |
Year of Publication | 2005 |
Authors | Patterson CC, Ross P, Pope-Harman AL, Knight DA, Magro CM |
Journal | J Cutan Pathol |
Volume | 32 |
Issue | 4 |
Pagination | 300-6 |
Date Published | 2005 Apr |
ISSN | 0303-6987 |
Keywords | Adult, alpha 1-Antitrypsin Deficiency, Antibodies, Antineutrophil Cytoplasmic, Antibodies, Antiphospholipid, Autoantibodies, Fatal Outcome, Fluorescent Antibody Technique, Direct, Humans, Immunoglobulin A, Immunoglobulin Isotypes, Liver, Lung, Male, Purpura, Schoenlein-Henoch |
Abstract | 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. |
DOI | 10.1111/j.0303-6987.2005.00304.x |
Alternate Journal | J Cutan Pathol |
PubMed ID | 15769280 |
Related Faculty:
Cynthia M. Magro, M.D.