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Shwartzman phenomenon in a patient with active systemic lupus erythematosus preceding fatal disseminated intravascular coagulation

Department of Rheumatology and Clinical Immunology, Humboldt University of Berlin, Berlin, Germany; Department of Rheumatology and Clinical Immunology, Charité University Hospital, Schumannstr. 20/21, D-10117 Berlin, Germanygabriela.riemekasten{at}charite.de

S Ziemer

Department of Clinical Biochemistry, Humboldt University of Berlin, Berlin, Germany

T Häupl

Department of Rheumatology and Clinical Immunology, Humboldt University of Berlin, Berlin, Germany

C Melzer

Department of Cardiology, Humboldt University of Berlin, Berlin, Germany

K Loddenkemper

Department of Rheumatology and Clinical Immunology, Humboldt University of Berlin, Berlin, Germany

S Hauptmann

Department of Pathology, Charité University Hospital, Humboldt University of Berlin, Berlin, Germany

G R Burmester

F Hiepe

Department of Rheumatology and Clinical Immunology, Humboldt University of Berlin, Berlin, Germany

The recurrence of widespread and diverse vascular lesions is a hallmark of systemic lupus erythematosus (SLE). Inflammatory and thrombotic mechanisms almost invariably associated with circulating antiphospholipidantibodies play a role in the pathogenesisof SLE-related vascular disease. Both mechanisms can coexist in the same patient. Vasculitis is most commonly induced by the local deposition of immune complexes. However, some SLE patients have an inflammatory complement-mediated vascular injury in the absence of immune complex deposition.

We report on a fatal case of disseminated intravascular coagulation (DIC) in a young woman with active SLE. Hemorrhagic lesions due to localized intravascular coagulation (Shwartzman phenomenon) preceded disseminated intravascular coagulation accompanied by disseminated cardiac necrosis. Immune complex ‘independent’ and other mechanisms of vascular injury and states of hypercoagulability will be discussed.

Lupus, Vol. 11, No. 4, 204-207 (2002)
DOI: 10.1191/0961203302lu193xx


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