Mixed connective tissue disease with multiple organ damage: successful treatment with plasmapheresis

Intern Med. 2000 Dec;39(12):1119-22. doi: 10.2169/internalmedicine.39.1119.

Abstract

A 24-year-old-woman with mixed connective tissue disease (MCTD) developed multiple organ failure, disseminated intravascular coagulation (DIC), metabolic acidosis, and respiratory and renal failure resulting from visceral vasospasm, so-called visceral Raynaud's phenomenon. After plasmapheresis, the condition of multiple organ failure was markedly improved. The successful treatment with plasmapheresis was dependent upon the removal of immune complexes in serum and improvement of visceral circulation. Thus plasma exchange is recommended as a possible a treatment for multiple organ damage in MCTD.

Publication types

  • Case Reports

MeSH terms

  • Acidosis / etiology
  • Adult
  • Autoantigens / immunology
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / pathology
  • Autoimmune Diseases / therapy*
  • Bronchitis / etiology
  • Calcinosis / etiology
  • Combined Modality Therapy
  • Cyclosporine / therapeutic use
  • Disseminated Intravascular Coagulation / etiology
  • Female
  • Hemorrhage / etiology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Ischemia / etiology*
  • Methylprednisolone / therapeutic use
  • Mixed Connective Tissue Disease / complications
  • Mixed Connective Tissue Disease / drug therapy
  • Mixed Connective Tissue Disease / pathology
  • Mixed Connective Tissue Disease / therapy*
  • Multiple Organ Failure / etiology*
  • Multiple Organ Failure / therapy
  • Plasmapheresis*
  • Ribonucleoprotein, U1 Small Nuclear / immunology
  • Vasoconstriction
  • Viscera / blood supply

Substances

  • Autoantigens
  • Immunosuppressive Agents
  • Ribonucleoprotein, U1 Small Nuclear
  • Cyclosporine
  • Methylprednisolone