Pulmonary artery hypertension in systemic lupus erythematous (Thai)
Main Article Content
Abstract
โรคเอสแอลอี (Systemic Lupus Erythematosus, SLE) หรือโรคลูปัส เป็นโรคที่มีการผลิตแอนติบอดีต่อเซลล์ร่างกายตนเองและทำให้เกิดการอักเสบของเนื้อเยื่อและอวัยวะในร่างกาย ส่งผลให้การทำงานของระบบต่าง ๆ ผิดปกติ เช่น ข้อต่อ ผิวหนัง ไต เซลล์เม็ดเลือด สมอง หัวใจและปอด เป็นโรคที่พบได้เพศหญิงวัยเจริญพันธุ์ และมีความชุกในคนที่มีเชื้อสายแอฟริกันอเมริกัน ฮิสแปนิกและเอเชีย ผู้ป่วยโรคเอสแอลอีมีอาการและอาการแสดงหลากหลาย ตั้งแต่ระดับความรุนแรงน้อย เช่น ผื่น ปวดข้อ ไปจนถึงอาการรุนแรงมากจนทำให้การทำงานของอวัยวะสำคัญผิดปกติและเสียชีวิตได้ เช่น ภาวะไตอักเสบ กล้ามเนื้อหัวใจหรือหลอดเลือดอักเสบ รวมถึงการทำงานของปอดและระบบทางเดินหายใจผิดปกติ1
อาการแสดงที่สัมพันธ์กับปอดและระบบทางเดินหายใจนั้น พบได้ประมาณร้อยละ 50-70 ของผู้ป่วยที่เป็นโรคเอสแอลอี ได้แก่ ภาวะปอดอินเตอร์สติเชียล (interstitial lung disease (ILD)) ปอดอักเสบเฉียบพลัน (acute pneumonitis) น้ำในเยื่อหุ้มปอด (pleural effusion) การมีเลือดออกในถุงลมปอด (diffuse alveolar hemorrhage) ภาวะความดันหลอดเลือดแดงปอดสูง (pulmonary artery hypertension) ภาวะหลอดเลือดในปอดอักเสบ (pulmonary vasculitis)2
สำหรับภาวะความดันหลอดเลือดปอดสูงในผู้ป่วยโรคเอสแอลอีเกิดได้หลายสาเหตุ ตามแนวทางของ European Society of Cardiology (ESC) และ European Respiratory Society (ERS) ตีพิมพ์ปี พ.ศ. 25653 จำแนกภาวะความดันหลอดเลือดปอดสูงออกเป็น 5 กลุ่ม ในผู้ป่วยโรคเอสแอลอี สามารถพบภาวะความดันหลอดเลือดปอดสูงได้ทุกกลุ่ม โดยเฉพาะ 4 กลุ่มหลัก สาเหตุสามารถเกิดจากการเปลี่ยนแปลงภายในหลอดเลือด (intravascular) เช่น การเกิดลิ่มเลือดอุดตัน (thrombus) ความผิดปกติของผนังหลอดเลือด (endothelium) หรือความผิดปกติของกล้ามเนื้อเรียบ (smooth muscle)4 ทำให้เกิดเป็นภาวะความดันหลอดเลือดปอดสูง การวินิจฉัยภาวะนี้ต้องอาศัยความตระหนักถึงของแพทย์ผู้รักษา อีกทั้งการรักษาภาวะความดันหลอดเลือดปอดสูงแต่ละประเภทนั้นมีความแตกต่างกัน บทความนี้จะกล่าวถึงพยาธิกำเนิด อาการและอาการแสดง การวินิจฉัย การรักษาและการดำเนินโรคของภาวะนี้ในผู้ป่วยโรคเอสแอลอี
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