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Antiphospholipid Syndrome Nephropathy and Other Thrombotic Microangiopathies Among Patients With Systemic Lupus Erythematosus

  • Elizabeth S. Kotzen
    Affiliations
    Division of Nephrology and Hypertension, Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC
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  • Sanjeet Roy
    Affiliations
    Division of Nephropathology, Department of Pathology and Laboratory Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC
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  • Koyal Jain
    Correspondence
    Address correspondence to Koyal Jain, MD, FASN, University of North Carolina Kidney Center, 7024 Burnett Womack Bldg, CB 7155, Chapel Hill, NC 27599.
    Affiliations
    Division of Nephrology and Hypertension, Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC
    Search for articles by this author
      Antiphospholipid syndrome (APS) and other causes of thrombotic microangiopathy (TMA) negatively impact the renal outcomes of patients with systemic lupus erythematosus (SLE) and lupus nephritis. Here we review the diagnosis and management of occlusive renal vascular lesions due to APS and other TMAs, with a focus on patients with SLE and lupus nephritis. The presence of a thrombotic event, unexplained hypertension, thrombocytopenia, or hemolytic anemia should prompt consideration for TMA syndromes. The differential diagnosis of a TMA in a patient with SLE includes APS, thrombocytopenic purpura, complement-mediated or infection-associated hemolytic uremic syndrome, drug-mediated TMA (particularly due to calcineurin inhibitor toxicity), and malignant hypertension. Treatment of APS with a documented thrombotic event focuses on anticoagulation to reduce the risk for further thrombotic events. Treatment of classic presentations of thrombocytopenic purpura and hemolytic uremic syndrome in the SLE population is the same as in patients without SLE. Treatment of APS nephropathy or TMA when it is diagnosed by biopsy with concomitant lupus nephritis presents a challenge to clinicians because there is no clear standard of care. Small and retrospective studies suggest potential benefit of complement inhibition, mammalian target of rapamycin (mTOR) inhibition, B cell depleting therapy, and plasma exchange therapy for patients with lupus nephritis and TMA, and prospective investigation of these therapies should be a research priority.

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