Thrombotic microangiopathic hemolytic anemia in systemic lupus erythematosus

doi:10.1016/0049-0172(94)90072-8

Seminars in Arthritis and Rheumatism

Volume 24, Issue 3, December 1994, Pages 165-172

https://doi.org/10.1016/0049-0172(94)90072-8 Get rights and content

Abstract

Thrombotic microangiopathic hemolytic anemia (TMHA) is characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever, neurological symptoms, and kidney involvement. It presents as thrombotic thrombocytopenic purpura (TTP) or hemolytic uremic syndrome (HUS). TMHA has been considered to occur only rarely in systemic lupus erythematosus (SLE). However, there has been an increase in the reporting of this association in recent years, and autopsy studies have suggested that TMHA may be underdiagnosed in SLE because of the similarity in symptoms. We report four patients with SLE-related TMHA and describe 24 more patients from a literature review. All patients were women, 50% had active SLE, 89% presented as TTP, and 11% presented as HUS. Those patients with active SLE had low complement levels. Antiphospholipid antibodies or lupus anticoagulant were positive in 5 of 8 cases. Patients treated with plasma infusions or plasmapheresis had a lower mortality rate at 25% compared with 57% mortality in patients who were not treated with plasma infusions or plasmapheresis. It is suggested that TMHA should be considered in any SLE patient presenting with neurological symptoms or renal failure associated with fever, hemolytic anemia, and thrombocytopenia. Early recognition and appropriate therapy with plasmapheresis may improve prognosis.

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^☆: Supported in part by a grant from the State of Missouri for Regional Arthritis Centers and a Fellowship Grant (Dr. Hanna) from the Eastern Missouri Chapter of The Arthritis Foundation.

¹: From the Division of Rheumatology, Saint Louis University Health Sciences Center, St. Louis, MO.

²: From the Intensive Care Unit, Department of Internal Medicine, Shaare-Zedek Medical Center, Jerusalem, Israel.

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Thrombotic microangiopathic hemolytic anemia in systemic lupus erythematosus☆

Abstract

Hematol Oncol Clin North Am

Clin Immunol Immunopathol

Am J Med

Am J Kidney Dis

Am J Kidney Dis

Lancet

Miscellaneous secondary thrombotic microangiopathy

Semin Hematol

SLE-clinical aspects and treatment

Systemic lupus erythematosus presenting as thrombotic thrombocytopenic purpura

J Rheumatol

Thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus

J Rheumatol

Thrombotic thrombocytopenic purpura preceding systemic lupus erythematosus

Ann Rheum Dis

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

J Rheumatol

Thrombotic thrombocytopenic purpura in systemic lupus erythematosus

J Rheumatol

Widespread vasculopathy with hemolytic uremie syndrome, perimyocarditis, and cystic pancreatitis in a young woman with mixed connective tissue disease

Rheumatol Int

Clinical and neuropathological findings in systemic lupus erythematosus: the role of vasculitis, heart emboli, and thrombotic thrombocytopenic purpura

Ann Neurol

Splenic pathology in thrombotic thrombocytopenic purpura

Am J Surg Pathol

Thrombotic thrombocytopenic purpura and systemic lupus erythematosus

Arch Intern Med