Summary
In order to better understand the clinical manifestation of systemic lupus erythematosus (SLE) with intracranial hypertension syndrome (IHS), we analyzed the clinical features and treatment of a typical SLE patient with IHS. SLE is one of the most unpredictable autoimmune diseases involving multiple organ systems that is defined clinically and associated with antibodies directed against cell nuclei. IHS is an uncommon manifestation of neuropsychiatric SLE (NPSLE) and is characterized by an elevated intracranial pressure, papilledema, and headache with occasional abducens nerve paresis, absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. IHS has been reported in a few sporadic cases in patients with SLE worldwide, but rarely has been reported in China. In this study, a 34-year-old female SLE patient with IHS was reported and pertinent literature reviewed. The clinical presentation, image logical features, and investigatory findings were discussed.
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References
Castellino G, Govoni M, Giacuzzo S, et al. Optimizing clinical monitoring of central nervous system involvement in SLE. Autoimmun Rev, 2008, 7(4):297–304
Stojanovich L, Zandman-Goddard G, Pavlovich S, et al. Psychiatric manifestations in systemic lupus erythematosus. Autoimmun Rev, 2007, 6(6):421–426
Ogawa M, Ishimaru K, Shiroto T, et al. A case of benign intracranial hypertension associated with systemic lupus erythematosus (SLE) showing diffuse white matter lesions on MRI. Rinsho Shinkeigaku, 1994, 34(6):577–581
Amit M, Molad Y, Levy O, et al. Headache in systemic lupus erythematosus and its relation to other disease manifestations. Clin Exp Rheumatol, 1999, 17:467–470
Edworthy SM. Clinical Manifestations of Systemic Lupus Erythematosus. In: Harris E D et al, Eds. Kelley’s Textbook of Rheumatology, 7th Edition. USA: Saunders, 2005. 1201–1224
Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum, 1982, 25(11):1271
Hanly JG, Urowitz MB, Sanchez-Guerrero J, et al. Neuropsychiatric events at the time of diagnosis of systemic lupus erythematosus: an international inception cohort study. Nat Clin Pract Rheumatol, 2007, 3(8):428–429
Sanna G, Piga M, Pelty MT, et al. Central nervous involvement in systemic lupus erythematosus: cerebral imaging and serological profile in patients with and without overt neuropsychiatric manifestations. Lupus, 2000, 9(8): 573–583
Lee HS, Bae SC, Uhm WS, et al. Red ear(s) syndrome associated with child neuropsychiatric lupus erythemaytosus. Lupus, 2000, 9(4):301–303
Sibbitt WL, Saibbit RR, Brooks WM, et al. Neuroimaging in neuropsychiatric systemic lupus erythematosus. Arthritis Rheum, 1999, 42(10):2026–2038
Steens S C A, Bosma G P, Cate R T et al. A neuroimaging follow up study of a patient with juvenile central nervous system lupus erythematosus. Ann Rheu Dis, 2003, 52(6):583–586
Kuyucu S, Argin A, Kuyucu N, et al. Systemic lupus erythematosus presenting with pseudotumor cerebri: a rare association. Turk J Pediatr, 2007, 49(1):98–101
ACR Ad Hoc Committee on neuropsychiatric lupus nomenclature. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum, 1992, 42:599–608
Zhou H, Zhang F, Tian X, et al. Clinical features and outcome of neuropsychiatric lupus in Chinese: analysis of 240 hospitalized patients. Lupus, 2008, 17(2):93–99
Govoni M, Castellino G, Padovan M, et al. Recent advances and future perspective in neuroimaging in neuropsychiatric systemic lupus erythematosus. Lupus, 2004, 13(3):149–158
Trysberg E, Blennow K, Zachrisson O, et al. Intrathecal levels of matrix metalloproteinases in systemic lupus erythematosus with central nervous system engagement. Arthritis Res Ther, 2004, 6:R551–R556
Ni J, Gao S, Wu QJ, et al. Myelopathy associated with systemic lupus erythematosus in 6 cases. Chin J Neuroimmunol Neurol (Chinese), 2006, 13(5):294–298
Sanna G, Bertolaccini ML, Mathieu A. Central nervous system lupus: a clinical approach to therapy. Lupus, 2003, 12(12):935–942
Traynor AE, Schroeder J, Rosa RM, et al. Treatment of severe systemic lupus erythematosus with high-dose chemotherapy and hematopoietic stem-cell transplantation: a phase I study. Lancet, 2000, 356:701–707
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Xue, Z., Wang, X., Liu, F. et al. Intracranial hypertension syndrome in systemic lupus erythematosus: Clinical analysis and review of the literature. J. Huazhong Univ. Sci. Technol. [Med. Sci.] 29, 107–111 (2009). https://doi.org/10.1007/s11596-009-0123-3
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DOI: https://doi.org/10.1007/s11596-009-0123-3