Fulminant onset of cerebral immunocomplex vasculitis as first manifestation of neuropsychiatric systemic lupus erythematosus (NPSLE)

Abstract

Fulminant onset of neuropsychiatric symptoms as first manifestation of neuropsychiatric systemic lupus erythematosus (NPSLE) is rare and diagnosis is difficult if only one organ is involved. Here, we report the case of a previously healthy woman who presented with a clinical syndrome most compatible with acute onset of NPSLE. However, American College of Rheumatology (ACR) criteria were not sufficiently met. Brain biopsy showed an autoimmune complex vasculitis consistent with central nervous system (CNS) lupus. Because the prognosis of SLE-related CNS involvement is poor, aggressive immunosuppressive treatment was initiated using methylprednisolone in combination with cyclophosphamide.

Keywords

neuropsychiatric systemic lupus erythematosus systemic lupus erythematosus vasculitis brain biopsy neurological ICU

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References

1 Mitchell I Webb M Hughes R Stewart J . Cerebral lupus . Lancet 1994; 343: 579 – 582 .

2 Jongen PJ Boerbooms AM Lamers KL Raes BC Vierwindan G . Diffuse CNS involvement in systemic lupus erythematosus: intrathecal synthesis of the 4th component of complement . Neurology 1990; 40: 1593 – 1596 .

3 Fady G Joseph G Lammie A Neil J . Scolding. CNS lupus: a study of 41 patients . Neurology 2007; 69: 644 – 654 .

4 Hochberg MC . Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus . Arthritis Rheum 1997; 40: 1725 .

5 Teasdale G Jennett B . Assessment of coma and impaired consciousness. A practical scale . Lancet 1974; 2: 81 – 84 .

6 Rankin J . Cerebral vascular accidents in patients over the age of 60. II. Prognosis . Scott Med J 1957; 5: 200 – 215 .

7 Lindley RI Waddell F Livingstone M . Can simple questions assess outcomes after stroke . Cerebrovasc Dis 1994; 4: 314 – 324 .

8 ACR ad hoc committee on Neuropsychiatric Lupus Nomenclature. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes . Arthritis Rheum 1999; 42: 599 – 608 .

9 Tan EM Feltkamp TE Smolen JS . Range of antinuclear antibodies in "healthy" individuals . Arthritis Rheum 1997; 40: 1601 – 1611 .

10.

10 Hahn BH . Antibodies to DNA . N Engl J Med 1998; 338: 1359 – 1368 .

11.

11 Afeltra A Garzia P Mitterhofer AP . Neuropsychiatric lupus syndromes. Relationship with antiphospholipid antibodies . Neurology 2003; 61: 108 – 110 .

12.

12 Hanly JG Walsh NMG Sangalang V . Brain pathology in systemic lupus erythematosus . J Rheumatol 1992; 19: 732 – 741 .

13.

13 Hess DC . Cerebral lupus vasculopathy. Mechanisms and clinical relevance . Ann N Y Acad Sci 1997; 823: 154 – 168 .

14.

14 Johnson RT Richardson EP . The neurological manifestations of systemic lupus erythematosus. A clinical-pathological study of 24 cases and review of the literature . Medicine 1968; 47: 337 – 369 .

15.

15 Jonson A Bengtsson A Nived O Ryberg B Sturfelt G . Outcome of neuropsychiatric systemic lupus erythematosus within a defined Swedish population: increased morbidity but low mortality . Rheumatology 2002; 41: 1308 – 1312 .

16.

16 Tokano Y Morimoto S Amano H . The relationship between initial clinical manifestation and long-term prognosis of patients with systemic lupus erythematosus . Mod Rheumatol 2005; 15: 275 – 282 .