IVIg の基本と pitfall Fig. 1 Mechanism of action of IVIG Table 1 Japan インフルエンザ脳炎 Bickerstaff 脳幹脳炎 急性散在性脳 Treatment selection and adaptation disease of IVI
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1 2 2 pitfall* ** Key Words : intravenous immunoglobulin, Guillain Barre syndrome, chronic inflammatory demyelinating polyneuropathy, myasthenia gravis, side effect IVIg IVIg IVIg I IVIg 400mg/kg 8ml1 5 IVIg ml/kg/ 0.03ml/kg/ IVIg IVIg II IVIg IVIg Fig. 1 IVIg Fc B T 1 3 III IVIg IVIg 4 Guillain Barré Guillain Barré syndrome GBS chronic inflammato ry demyelinating polyneuropathy CIDP myathenia gravis MG polymyositis PM dermatomyositis DMChurg Strauss CSS Table 1 1. GBS CIDP GBS CIDP multifocal motor neuropathy MMN 2. MG PM DM CSS MG MG PM DM CSS 3. IVIg The Foundations and Pitfall of Intravenous Immunoglobulin in Treatment of Neurological Diseases. Kyoichi NOMUR : Department of Neurology, Saitama Medical Center, Saitama Medical University 183
2 IVIg の基本と pitfall Fig. 1 Mechanism of action of IVIG Table 1 Japan インフルエンザ脳炎 Bickerstaff 脳幹脳炎 急性散在性脳 Treatment selection and adaptation disease of IVIG in 脊髄炎などがあり その他に視神経脊髄炎 小児難治性てん Neurological Diseases かんなどがある 5 Level of Evidence IV IVIg の追加 再投与 First-line Therapy Guillain-Barre syndrome GBS chronic inflammatory demyelinating polyneruropathy CIDP 1. GBS GBS において初回の IVIg を施行しても 神経症状がさら に進行 あるいは明らかな改善を認めない症例では IVIg を 追加投与する さらに 神経症状の改善後の回復期において Second-line Therapy Myasthenia gravis MG acute exacerbation Churg-Strauss syndrome CSS B Polymyositis & Dermatomyositis PM DM B 症状の悪化 治療関連性変動 再燃 を認める症例などでは 再度の IVIg を考慮する 6 本邦市販後調査の結果では初回投 与の有効率は 71.2 に対し 再投与では 54.9 で低下した しかし 再投与開始時期による有効性の検討では 初回投与 から 4 週以内 5 週以降の比較では 5 週以降の有効率 37.5 に対して 4 週以内では 76.9 であり 早期からの再投与を 考慮する結果であった 7 Fig. 2 神経疾患として 筋疾患では封入体筋炎 末梢神経 筋関連 疾 患 で は 傍 腫 瘍 性 末 梢 性 神 経 障 害 stiff-person 症 候 群 2. CIDP CIDP において IVIg は有効であるが 一部の症例では 1 回 Isaacs 症候群 Lambert-Eaton 症候群などがある 脳炎関 目の投与では効果が認めらない場合がある この場合では 連疾患では非ヘルペス性辺縁系脳炎 抗 MND 受容体脳炎 神経治療 Vol. 31 No
3 Fig. 2 Effective rate due to the timing of re administration of IVIG 2 IVIg 8 IVIg 1 IVIg IVIg MG PM DM CSS MG PM DM CSS IVIg IVIg4 V IVIg 1. IVIg GBS IVIg IVIg 11, 12 CIDP 3IVIg IVIg 13 MG IVIg 2. IVIg GBS kg IVIg 3. IVIg 15 VI IVIg Ig 16 VII IVIg
4 Table 2 The side effects in the course of IVIG treatment, and Treatment Table 3 The frequency of occurrence of side effects of IVIG in nu eroimmunological diseases Course of treatment & Side effects Symptoms appearing at the start of treatment in 30 min. headache, chilling, muscle pain, general malaise, fever, nausea Symptoms appearing during treatment and after treatment aseptic meningitis rash pompholyx renal tubular necrosis thromboembolisim brain, lung hyponatremia granulocytopenia Symptomatic treatment & Prognosis slowing in the rate of infusion disappear in 1 2 days recover in a few days lasting about a month, then disappear note in cases with elderly, DM, CKD note in cases with DM, hyperlipidemia pseudoneutropenia Disorders Frequency of LT Globulin No occurrence of abnormal preparations cases side effects % % CIDP Glovenin I GBS Venilon I MG Venoglobulin IH PM/DM Venoglobulin IH CSS Venilon I Pemphigus Glovenin I time the insurance has been approved 3. IVIg IVIg 30 IVIg Na 17 Table 2 4. Table 3 LT IVIg LT IVIg VIII 3 6 IVIg COI 1 Dwyer JM : N Engl J Med 326 : , Chapel H : Q J Med 89 : , Marinose C et al : Neurology 51 suppl 5 : S2 S8, Imbach P et al : Lancet : , Members of the Task Force : Eur J Neurol 15 : , p : , p Joint Task Force of the EFNS and the PNS : J Periher Nerv Syst 15 : 1 9, Chan LY et al : cta Obstet Gynecol Scand 83 : , : , p p p : , p
5 The Foundations and Pitfall of Intravenous Immunoglobulin in Treatment of Neurological Diseases Kyoichi NOMUR Department of Neurology, Saitama Medical Center, Saitama Medical University Intravenous immunoglobulin IVIG has been success fully used to treat a number of immune mediated diseases of central and peripheral nervous system. The mechanism of action of IVIG have not been fully explained. IVIG has been used as a first line therapy in Guillain Barre syn drome GBS, chronic inflammatory demyelinating poly neuropathy CIDP. IVIG is recommended as a second line treatment in combination with prednisone of severe myasthenia gravis MG, Churg Strauss syndrome CSS and inflammatory myopathy in the insurance adaptation of Japan. IVIG seems to have a favourable effect also in paraneoplastic neurological disease, stiff person syn drome, some acute demyelinating encephalopathy. Patients with GBS or CIDP who improve after IVIG and then re lapse should preferentially be retreated with a second course of IVIG. IVIG treatment of patient who had accom panied pregnancy or high body weight or high age was dis cussed. The side effects in the course of treatment of IVIG, symptoms appearing at the start of treatment in 30 min utes, symptoms appearing during and after IVIG treat ment have been illustrated. The frequency of side effects of IVIG may differ by individual disease. This review focuses on the foundations and pitfall of IVIG treatment in neuro logical diseases. 187
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