Lupus vasculopathy: Diagnostic, pathogenetic and therapeutic considerations

M. L. Gonzalez-Suarez, A. A. Waheed, David Andrews, Dana Ascherman, X. Zeng, Ali Nayer

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

A rare form of vascular disease in systemic lupus erythematosus (SLE), lupus vasculopathy is characterized by necrosis and accumulation of immunoglobulins (IGs) and complements in the wall of arterioles and small arteries resulting in luminal narrowing. Lupus vasculopathy often accompanies lupus nephritis and portends a poor prognosis. Although there is general agreement on the treatment of lupus nephritis, effective treatment strategies for lupus vasculopathy remain to be defined. We report a 20-year-old woman with SLE who presented with generalized tonic-clonic seizure. Her immunosuppressive regimen consisted of mycophenolate mofetil, prednisone and hydroxychloroquine. On physical examination, she was Cushingoid in appearance and hypertensive. Laboratory tests indicated renal disease. Coagulation studies disclosed de novo lupus anticoagulant. Magnetic resonance imaging of the brain demonstrated acute focal cerebral hemorrhage. Echocardiography revealed reduced ejection fraction and severe mitral regurgitation. Despite high-dose glucocorticoids and mycophenolate mofetil, renal function remained poor. Kidney biopsy demonstrated lupus vasculopathy and glomerulonephritis. Plasma exchange therapy and intravenous cyclophosphamide were administered. Over the ensuing four weeks, renal function improved, complement levels increased, autoantibody titers decreased and lupus anticoagulant disappeared. In conclusion, lupus vasculopathy can occur in SLE despite a heavy immunosuppressive regimen. Antiphospholipid antibodies might be involved in the pathogenesis of lupus vasculopathy. Plasma exchange therapy in conjunction with intravenous cyclophosphamide may represent an effective treatment strategy for lupus vasculopathy.

Original languageEnglish
Pages (from-to)421-427
Number of pages7
JournalLupus
Volume23
Issue number4
DOIs
StatePublished - Jan 1 2014

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Lupus Nephritis
Systemic Lupus Erythematosus
Mycophenolic Acid
Kidney
Lupus Coagulation Inhibitor
Plasma Exchange
Immunosuppressive Agents
Cyclophosphamide
Hydroxychloroquine
Therapeutics
Antiphospholipid Antibodies
Cerebral Hemorrhage
Mitral Valve Insufficiency
Arterioles
Prednisone
Vascular Diseases
Autoantibodies
Glucocorticoids
Physical Examination
Echocardiography

Keywords

  • antiphospholipid antibodies
  • cyclophosphamide
  • lupus anticoagulant
  • lupus nephritis
  • Lupus vasculopathy
  • plasma exchange

ASJC Scopus subject areas

  • Rheumatology
  • Medicine(all)

Cite this

Lupus vasculopathy : Diagnostic, pathogenetic and therapeutic considerations. / Gonzalez-Suarez, M. L.; Waheed, A. A.; Andrews, David; Ascherman, Dana; Zeng, X.; Nayer, Ali.

In: Lupus, Vol. 23, No. 4, 01.01.2014, p. 421-427.

Research output: Contribution to journalArticle

Gonzalez-Suarez, M. L. ; Waheed, A. A. ; Andrews, David ; Ascherman, Dana ; Zeng, X. ; Nayer, Ali. / Lupus vasculopathy : Diagnostic, pathogenetic and therapeutic considerations. In: Lupus. 2014 ; Vol. 23, No. 4. pp. 421-427.
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