THE NERVOUS SYSTEM IN SYSTEMIC SCLEROSIS (SCLERODERMA) : Clinical Features and Pathogenetic Mechanisms - 11/09/11

Doi : 10.1016/S0889-857X(05)70306-9

Marco Matucci Cerinic, MD, PhD ^{^{*
From the Institute of Internal Medicine IV, University of Florence, Florence (MMC, SG, AP); and the Foundation “S. Maugeri,” Rehabilitation Center of Montescano, IRCCS, Montescano (RC), Italy}}, Sergio Generini, MD ^{^{*
From the Institute of Internal Medicine IV, University of Florence, Florence (MMC, SG, AP); and the Foundation “S. Maugeri,” Rehabilitation Center of Montescano, IRCCS, Montescano (RC), Italy}}, Alberto Pignone, MD, PhD ^{^{*
From the Institute of Internal Medicine IV, University of Florence, Florence (MMC, SG, AP); and the Foundation “S. Maugeri,” Rehabilitation Center of Montescano, IRCCS, Montescano (RC), Italy}}, Roberto Casale, MD ^{^{*
From the Institute of Internal Medicine IV, University of Florence, Florence (MMC, SG, AP); and the Foundation “S. Maugeri,” Rehabilitation Center of Montescano, IRCCS, Montescano (RC), Italy}}

Résumé

Systemic sclerosis (SSc) involves the microvascular system^{54 Matucci Cerinic M., Kahaleh B.M., LeRoy E.C. Pathogenesis: Vascular involvement Systemic Sclerosis. Baltimore: Williams & Wilkins (1995).
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Cliquez ici pour aller à la section Références} and leads to fibroblast activation and excessive production of collagen.^{74 Sollberg S., Mauch C., Eckes B. , et al. The fibroblast in systemic sclerosis Clin Dermatol 1995 ; 12 : 379

Cliquez ici pour aller à la section Références} The skin and the internal organs (lung, heart, kidney and gastrointestinal system) are the main targets of the disease. Very few reports, however, are available about the involvement of the nervous system, which has been almost always considered secondary to the disease process, and scarce interest has been devoted to the mechanisms of its pathogenesis.

At the beginning of this century, a case of localized scleroderma that developed after cranial nerve injury was described.^{44 Kingery L.B. Scleroderma following nerve injury Arch Dermatol Syphilol 1922 ; 5 : 579

Cliquez ici pour aller à la section Références} Since this observation, several papers have been published showing the involvement of the nervous system as a main event in localized linear scleroderma^{50 Littman B.H. Linear scleroderma: A response to neurologic injury? Report and literature review J Rheumatol 1989 ; 16 : 1135

Cliquez ici pour aller à la section Références} and in particular in Parry-Romberg syndrome.^{27 Fry A., Alvarellos A., Fink C.W. , et al. Intracranial findings in progressive facial hemiatrophy J Rheumatol 1992 ; 19 : 956

Cliquez ici pour aller à la section Références} A recent report describing the development of SSc only on the healthy side of a hemiplegic patient now puts the focus on the importance of the nervous system in the development and maintenance of the systemic form of scleroderma as well as on other rheumatic diseases.^{53 Matucci Cerinic M. Sensory neuropeptides and rheumatic diseases Rheum Dis Clin North Am 1993 ; 19 : 971

Cliquez ici pour aller à la section Références} SSc literature has devoted its attention to the nervous system mainly in two periods in this century: from the end of the 1940s to the mid-1960s and from the mid-1980s up to the present day. Recent publications^{37 Herrick A.L., Jayson M.I.V. Organ involvement: Nervous system Systemic Sclerosis. Baltimore: Williams & Wilkins (1995).
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Cliquez ici pour aller à la section Références} show the interest that the nervous involvement in SSc has raised today. Case reports and some works published on this subject may allow a collage of the clinical features of nervous involvement, although pathogenesis still remains controversial and intriguing.

This article reviews the literature on the involvement of the central (CNS) and peripheral nervous system (PNS) in SSc, analyzes the possible pathogenetic mechanisms, and analyzes the role of the nervous system in disease onset and evolution.

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