Immune-Mediated Necrotizing Myopathy (Thai)
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Abstract
Immune-mediated necrotizing myopathy (IMNM) หรือ necrotizing autoimmune myopathy (NAM) เป็นโรคหนึ่งในกลุ่ม idiopathic inflammatory myopathies (IIM) พบร้อยละ 20-38 ของ IIM ทั้งหมด มีการดำเนินโรครุนแรงและรวดเร็ว อาการสำคัญคือ กล้ามเนื้อต้นขาและต้นแขนอ่อนแรง (proximal muscle weakness) อย่างรุนแรงและเฉียบพลันภายใน 6 สัปดาห์ หรือรองเฉียบพลันภายใน 6 เดือน พร้อมกับมีเอนไซม์กล้ามเนื้อในซีรัม (serum creatine kinase, CK) สูง ผู้ป่วยสองในสามมี autoantibodies จำเพาะต่อโรคได้แก่ anti-signal recognition particle (SRP) และ anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) autoantibodies พยาธิสภาพของกล้ามเนื้อที่เป็นมาตรฐานสำคัญสำหรับวินิจฉัยโรคคือ มีใยกล้ามเนื้อตาย (myofiber necrosis) อย่างรุนแรงเป็นวงกว้าง โดยพบการอักเสบน้อยมากหรือไม่พบเลย (scanty or no inflammatory cell infiltration) การวินิจฉัยโรคและการรักษาอย่างถูกต้องตั้งแต่ระยะแรกจะทำให้ผู้ป่วยรอดชีวิตและมีคุณภาพชีวิตที่ดีเหมือนคนทั่วไป
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