Renal Thrombotic Microangiopathy in Patients With Systemic Lupus Erythematosus and the Antiphospholipid Syndrome

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Current studies indicate that a thrombotic microangiopathy (TIVIA) identifies patients with systemic lupus erythematosus (SLE) who are at high risk of progressing to end-stage renal disease. We have observed two patients with SLE and one patient with a primary antiphospholipid syndrome (APS) who developed acute renal insufficiency with thrombocytopenia. Renal biopsies showed a TIVIA characterized by thrombi or by cellular and mucoid intimal hyperplasia of small arteries and arterioles. No arterial or arteriolar immune-complex deposits were detected by immunofluorescent or electron microscopy. Biopsies from one SLE patient and the APS patient showed no immune-complex glomerular disease. Both had serum antiphospholipid antibodies (aPL). aPL were not detected in the serum of the other SLE patient who had an active lupus nephritis. Acute renal failure and thrombocytopenia resolved in each case following treatment by plasmapheresis or prednisone and heparin. None of the patients were initially treated with cytotoxic drugs. As more knowledge is gained, the accurate identification of renal vascular lesions in SLE or related diseases could influence renal prognosis and choice of therapy. The cases reported here provide further evidence that a TMA can cause acute renal failure independent of lupus nephritis. TMA should be distinguished from other forms of renal vascular disease, particularly a noninflammatory lupus microangiopathy, which is probably mediated by subendothelial immune-complex deposits. The absence of immunoglobulin deposits in vessels involved by a TMA indicates that microvascular thrombosis is promoted by mechanisms other than those usually attributed to immune-complex disease. Phospholipid reactive antibodies may be pathogenetic in some cases.

References (35)

  • KS Kant et al.

    Glomerular thrombosis in systemic lupus erythematosus: Prevalence and significance

    Medicine (Baltimore)

    (1981)
  • P Kincaid-Smith et al.

    Lupus anticoagulant associated with renal thrombotic microangiopathy and pregnancy related renal failure

    Q J Med

    (1988)
  • D Kleinknecht et al.

    Recurrent thrombosis and renal vascular disease in patients with a lupus anticoagulant

    Nephrol Dial Transplant

    (1989)
  • RA Asherson et al.

    The “primary” antiphospholipid syndrome: major clinical and serological features

    Medicine (Baltimore)

    (1989)
  • LL Alpert

    Thrombotic thrombocytopenic purpura and systemic lupus erythematosus Report of a case with immunofluorescent investigation of vascular lesions

    J Mt Sinai Hosp

    (1968)
  • RC Becker et al.

    Thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus

    Clev Clin Q

    (1985)
  • FA Cecere et al.

    Fatal thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus Relationship to circulating immune complexes

    Arthritis Rheum

    (1981)
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