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Neurologic involvement in scleroderma: a systematic review.

Tiago Nardi Amaral1, Fernando Augusto Peres, Aline Tamires Lapa

  • 1Rheumatology Division, Faculty of Medical Science, State University of Campinas, Campinas, Brazil; Rheumatology Lab, Faculty of Medical Science, State University of Campinas, Campinas, Brazil.

Seminars in Arthritis and Rheumatism
|July 6, 2013
PubMed
Summary
This summary is machine-generated.

Neurologic involvement in scleroderma, including Systemic Sclerosis (SSc) and Localized Scleroderma (LS), is increasingly recognized. Seizures and headaches are common in LS, while SSc often involves peripheral and autonomic nervous systems.

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Area of Science:

  • Neurology
  • Rheumatology
  • Dermatology

Background:

  • Neurologic involvement in scleroderma, encompassing Systemic Sclerosis (SSc) and Localized Scleroderma (LS), is a significant clinical concern.
  • Understanding the spectrum of neurological manifestations is crucial for timely diagnosis and management.

Purpose of the Study:

  • To systematically review and synthesize existing literature on neurologic involvement in SSc and LS.
  • To describe the clinical features, neuroimaging findings, and treatment strategies for nervous system complications in these conditions.

Main Methods:

  • Comprehensive literature search of PubMed using extensive MeSH terms related to scleroderma and neurological conditions.
  • Inclusion criteria focused on SSc and LS, excluding other connective tissue diseases with known neurological impact.
  • Analysis of identified case reports and studies to extract data on patient demographics, symptoms, and treatments.

Main Results:

  • 182 studies on SSc (9506 patients) and 50 on LS (224 patients) were analyzed.
  • In LS, seizures (41.58%) and headache (18.81%) were predominant. In SSc, central nervous system involvement included headache (23.73%), seizures (13.56%), and cognitive impairment (8.47%).
  • Peripheral nervous system involvement in SSc commonly included myopathy (51.8%), trigeminal neuropathy (16.52%), and sensorimotor polyneuropathy (14.25%). Autonomic neuropathy was also frequently reported. Depression and anxiety were highly prevalent (73.15% and 23.95%).

Conclusions:

  • Neurologic manifestations in scleroderma are more common than previously thought.
  • LS primarily presents with seizures and headaches, whereas SSc frequently involves peripheral and autonomic nervous systems.
  • Emerging evidence highlights white matter lesions in asymptomatic SSc patients, indicating potential microvascular involvement.