ORIGINAL PAPER
Systemic sclerosis with central nervous system involvement: brain abnormalities revealed by magnetic resonance imaging
Online publication date: 2010-12-20
Reumatologia 2010;48(6):410-415
KEYWORDS
ABSTRACT
Background : Systemic sclerosis (SSc) is a progressive multisystem connective tissue disease characterised by obliterative microvascular abnormalities and visceral fibrosis. Primary involvement of the brain is thought to be uncommon. The aim of the study was to investigate the prevalence of the brain abnormalities in SSc patients by means of neurological examination and magnetic resonance imaging (MRI) and to correlate MRI images with neurological manifestations.
Material and methods : 21 female patients with SSc aged 23–71, with a disease duration of 1–11 years with neuropsychiatric symptoms and without other relevant systemic diseases were enrolled. All patients underwent brain MRI examinations.
Results : The most common findings in MRI were symptoms of cortical and subcortical atrophy (in 57% of all patients) and focal lesions (62% of patients) predominantly in white matter and < 2 mm in diameter. Among patients with depression, cortical and subcortical atrophy of moderate or significant degree was found in 8 individuals (66.7%), single focal lesions in 6 individuals (50%) and multiple focal lesions in 3 (25%).
Conclusions : These data suggest that brain involvement in patients with systemic sclerosis is not uncommon and magnetic resonance imaging is considered to be a sensitive tool for detection of the central nervous system involvement in patients with SSc.
Material and methods : 21 female patients with SSc aged 23–71, with a disease duration of 1–11 years with neuropsychiatric symptoms and without other relevant systemic diseases were enrolled. All patients underwent brain MRI examinations.
Results : The most common findings in MRI were symptoms of cortical and subcortical atrophy (in 57% of all patients) and focal lesions (62% of patients) predominantly in white matter and < 2 mm in diameter. Among patients with depression, cortical and subcortical atrophy of moderate or significant degree was found in 8 individuals (66.7%), single focal lesions in 6 individuals (50%) and multiple focal lesions in 3 (25%).
Conclusions : These data suggest that brain involvement in patients with systemic sclerosis is not uncommon and magnetic resonance imaging is considered to be a sensitive tool for detection of the central nervous system involvement in patients with SSc.
REFERENCES (25)
1.
Krieg T, Meurer M. Systemic scleroderma: clinical and pathophysiologic aspects. J Am Acad Dermatol 1988; 18: 457-481. .
2.
Seibold JR. Text book of rheumatology. In: Scleroderma, Kelley WN, Harris ED Jr, Ruddy S, Sledge CB (eds). 5th edn. Saunders, Philadelphia, 1133-1162. .
3.
Averbuch-Heller L, Steiner I, Abramsky O. Neurologic manifestations of progressive systemic sclerosis. Arch Neurol 1992; 49: 1292-1295. .
4.
Heron E, Fornes P, Rance A, et al. Brain involvement in scleroderma. Two autopsy cases. Stroke 1998; 29: 719-721. .
5.
Veale DJ, Collidge TA, Belch JJF. Increased prevalence of symptomatic macrovascular disease in systemic sclerosis. Ann Rheum Dis 1995; 54: 853-855. .
6.
Cutolo M, Nobili F, Sulli A, et al. Evidence of cerebral hypoperfusion in scleroderma patients. Rheumatology (Oxford) 2000; 39: 1366-1373. .
7.
Kahaleh B. Vascular disease in scleroderma: mechanisms of vascular injury. Rheum Dis Clin North Am 2008; 34: 57-71. .
8.
Guiducci S, Giacomelli R, Cerinic MM. Vascular complications of scleroderma. Autoimmun Rev 2007; 6: 520-523. .
9.
Kahaleh B. The microvascular endothelium in scleroderma. Rheumatology (Oxford) 2008; 47 Suppl 5: 14-15. .
10.
Blann AD, Illingworth K, Jayson MI. Mechanisms of endothelial cell damage in systemic sclerosis and Raynaud's phenomenon. J Rheumatol 1993; 20: 1325-1330. .
11.
Pearson JD. The endothelium: its role in scleroderma. Ann Rheum Dis 1991; 50 Suppl 4: 866-871. .
12.
Sakkas LI. New developments in the pathogenesis of systemic sclerosis. Autoimmunity 2005; 38: 113-116. .
13.
Worda M, Sgonc R, Dietrich H, et al. In vivo analysis of the apoptosis-inducing effect of anti-endothelial cell antibodies in systemic sclerosis by the chorionallantoic membrane assay. Arthritis Rheum 2003; 48: 2605-2614. .
14.
Waters CE, Shi-Wen X, Denton CP, et al. Signaling pathways regulating intercellular adhesion molecule 1 expression by endothelin 1: comparison with interleukin-1beta in normal and scleroderma dermal fibroblasts. Arthritis Rheum 2006; 54: 649-660. .
15.
Shi-Wen X, Denton CP, Dashwood MR, et al. Fibroblast matrix gene expression and connective tissue remodeling. Role of endotelin-1. J Invest Dermatol 2001; 116: 417-425. .
16.
Guiducci S, Fatini C, Rogai V, et al. Angiotensin-converting enzyme in systemic sclerosis: from endothelial injury to a genetic polymorphism. Ann N Y Acad Sci 2006; 1069: 10-19. .
17.
Bartoli F, Angotti C, Fatini C, et al. Angiotensin – converting enzyme I/D polymorphism and macrovascular disease in systemic sclerosis. Rheumatology (Oxford) 2007; 46: 772-775. .
18.
Pathak R, Gabor AJ. Scleroderma and central nervous vasculitis. Stroke 1991; 22: 410-413. .
19.
Sardanelli F, Iozzelli A, Cotticelli B, et al. White matter hyperintensities on brain magnetic resonance in systemic sclerosis. Ann Rheum Dis 2005; 64: 777-779. .
20.
Subcommittee for Scleroderma Criteria of the American Rheumatism Association in Diagnostic and Therapeutic Criteria Committee (1980). Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980; 23: 581-590. .
21.
Cerinic MM, Generini S, Pignone A, et al. The nervous system in systemic sclerosis (scleroderma). Clinical features and pathogenetic mechanisms. Rheum Dis Clin North Am 1996; 22: 879-892. .
22.
Mohammed RH, Sabry YY, Nasef AA. Brain MRI screening showing evidences of early central nervous system involvement in patients with systemic sclerosis. Rheumatol Int 2010; Jan 13 (on line). .
23.
Argyropoulou MI, Tsifetaki N, Zikou AK, et al. Systemic sclerosis: brain abnormalities revealed by conventional magnetic resonance imaging and magnetization transfer imaging. Arthritis Rheum 2006; 54: 1350-1352. .
24.
Terrier B, Charbonneau F, Touze E, et al. Cerebral vasculopathy is associated with severe vascular manifestations in systemic sclerosis. J Rheumatol 2009; 36: 1486-1494. .
25.
Walecka I, Sicińska J, Furmanek M, et al. Value of computed tomography and magnetic resonance imaging in diagnosis of central nervous system involvement in systemic sclerosis. Pol J Radiol 2006; 71: 7-12.
Copyright: © Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. This is an Open Access journal, all articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (https://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
Share
RELATED ARTICLE
| eISSN: | 2084-9834 |
| ISSN: | 0034-6233 |
We process personal data collected when visiting the website. The function of obtaining information about users and their behavior is carried out by voluntarily entered information in forms and saving cookies in end devices. Data, including cookies, are used to provide services, improve the user experience and to analyze the traffic in accordance with the Privacy policy. Data are also collected and processed by Google Analytics tool (more).
You can change cookies settings in your browser. Restricted use of cookies in the browser configuration may affect some functionalities of the website.
You can change cookies settings in your browser. Restricted use of cookies in the browser configuration may affect some functionalities of the website.
