主要なシーグレン病の表現形態としてのディサオトノミア: 6人の患者の症例シリーズ
Emilio Godínez-Lazarini1, Erik Cimé-Aké1, Mitzi Peña-Ojeda1
1Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Belisario Domínguez Sección XVI, Avenida Vasco de Quiroga 15, C.P. 14080, Tlalpan, Mexico City, Mexico.
Immunologic research
|September 5, 2025
まとめ
主要なSjögrenで発生する神経疾患であるディサウトノミア
科学分野:
- 神経学
- リウマトロジ
- 自律神経科学
背景:
- プライマリ・シェーグレン病 (pSjD) は慢性的な自己免疫疾患である.
- 自律神経系の機能不全によって特徴づけられるディサオトノミアは,自己免疫疾患の合併症である可能性があります.
研究 の 目的:
- pSjDに二次的なdysautonomiaを持つ患者の臨床的特徴とアウトカムを記述する.
- この珍しい神経学的症状に関する 既存の文献をレビューする
主な方法:
- 脱自律症とpSjDと診断された6人の患者の遡及症例シリーズ.
- 患者は,シーグレン病の2016年ACR/ EULAR基準を満たした.
- 自律機能はEwinのバッテリーテストで評価され,重症度はCASSとCOMPASS-31で評価された.
主要な成果:
- 6人の患者のうち5人にpSjDの診断が先行し,2~10年の間隔があった.
- 常見の症状には,正静性低血圧と昏睡が含まれていた.
- プレドニゾン,ヒドロキシクロロキン,DMARDs,リトキシマブを含む治療で,ディサウトノミア症状の部分的な改善が観察されました.
結論:
- ダイサウトノミアはpSjDの希少だが重要な神経学的合併症である.
- pSjD患者におけるディサオトノミアの早期発見と管理は極めて重要です.
- 治療は症状の部分的な改善につながるが,完全な寛解はまれである.
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