Progressive appearance of overlap syndrome together with autoantibodies in a patient with fatal thrombotic microangiopathy

Am J Med Sci. 2000 Nov;320(5):348-51. doi: 10.1097/00000441-200011000-00010.

Abstract

We describe an extraordinary patient with overlap syndrome (systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis) having positive autoantibodies against Sm, double stranded DNA, DNA topoisomerase I, and centromere, together with rheumatoid factor. The patient had multiple organ involvement resulting from thrombotic microangiopathy that mimicked so-called normotensive scleroderma renal crisis, and died mainly of massive pulmonary hemorrhage caused by thrombotic thrombocytopenic purpura. The clinical presentations of the case support the concept of strong associations between disease-specific autoantibodies and clinical features.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Arthritis, Rheumatoid / complications*
  • Arthritis, Rheumatoid / diagnostic imaging
  • Arthritis, Rheumatoid / immunology
  • Autoantibodies / immunology*
  • Autoantigens / immunology
  • Centromere / immunology
  • DNA / immunology
  • DNA Topoisomerases, Type I / immunology
  • Fatal Outcome
  • Female
  • Histocytochemistry
  • Humans
  • Kidney / pathology
  • Lung / pathology
  • Lupus Erythematosus, Systemic / complications*
  • Lupus Erythematosus, Systemic / immunology
  • Peripheral Vascular Diseases / complications
  • Peripheral Vascular Diseases / diagnostic imaging
  • Peripheral Vascular Diseases / immunology
  • Peripheral Vascular Diseases / pathology
  • Purpura, Thrombotic Thrombocytopenic / complications*
  • Purpura, Thrombotic Thrombocytopenic / diagnostic imaging
  • Purpura, Thrombotic Thrombocytopenic / immunology*
  • Purpura, Thrombotic Thrombocytopenic / pathology
  • Radiography
  • Rheumatoid Factor / immunology
  • Ribonucleoproteins, Small Nuclear*
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / immunology
  • Syndrome
  • snRNP Core Proteins

Substances

  • Autoantibodies
  • Autoantigens
  • Ribonucleoproteins, Small Nuclear
  • snRNP Core Proteins
  • DNA
  • Rheumatoid Factor
  • DNA Topoisomerases, Type I