Systemic lupus erythematosus complicated by thrombotic microangiopathy

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Abstract

Seven patients with systemic lupus erythematosus (SLE) or SLE-like disease developed thrombotic microangiopathy. Prominent features of their acute illnesses were microangiopathic hemolytic anemia (7), thrombocytopenia (7), fever (1), nervous system disease (4), and renal dysfunction (5). Laboratory data were significant for antinuclear antibody (ANA) (7), DNA (5), low C3 level (3), low C4 level (2), antiphospholipid antibody (6), schistocytes (7), and lactate dehydrogenase > 500 (7). All seven patients received treatment that initially included steroids but later included cyclophosphamide (4), plasma infusion (1), plasmapheresis (5), intravenous γ-globulin (2), anti-platelet agents (2), or vincristine (3). Six patients improved, and one patient expired during treatment. Of the six patients who survived this complication, three expired within the year following their acute illnesses. Histology, available in two cases, showed vascular changes consistent with a microangiopathic process. We conclude that the spectrum of vascular diseases in SLE extends beyond vasculitis to include noninflammatory vascular processes that can cause equally devastating complications.

References (33)

  • DA Fox et al.

    Thrombotic thrombocytopenic purpura and systemic lupus erythematosus

    Ann Rheum Dis

    (1986)
  • PA Gatenby et al.

    Systemic lupus erythematosus and thrombotic thrombocytopenic purpura. A case report and a review of a relationship

    J Rheumatol

    (1981)
  • DC Hess et al.

    Thrombotic thrombocytopenic purpura in systemic lupus erythematosus and antiphospholipid antibodies: Effective treatment with plasma exchange and immunosuppression

    J Rheumatol

    (1992)
  • OG Jonsson et al.

    Systemic lupus erythematosus presenting as thrombotic thrombocytopenic purpura

    J Rheumatol

    (1990)
  • AM Kosloske et al.

    The syndrome of thrombocytopenic purpura as the presenting manifestation in systemic lupus erythematosus

    Calif Med

    (1961)
  • MH Laszlo et al.

    The association of thrombotic thrombocytopenic purpura and disseminated lupus erythematosus; a report of 2 cases with successful treatment of one

    Ann Intern Med

    (1974)

    • Cited by (0)

    1

    From the Division of Rheumatology and Allergy-Clinical Immunology, Departments of Medicine, Laboratories, and Pathology North Shore University Hospital and Cornell University Medical College, Manhasset, NY.

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    Copyright © 1994 Published by Elsevier Inc.