米ポップシンガーで女優のセレーナ・ゴメス（２３）の難病告白により、注目されることになった『全身性エリテマトーデス』とはどんな病気なのか？
あまり聞きなれない病名ですが、著名人でいえば、安奈淳（宝塚出身女優）さんも患っていた病気です。
安奈淳、鳳蘭 宝塚星組最後の共演
どんな病気か
全身性エリテマトーデス(SLE)は、細胞の核成分に対する抗体を中心とした自己抗体（自分の体の成分と反応する抗体）が作られてしまうために、全身の諸臓器が侵されてしまう病気です。よくなったり悪くなったりを繰り返し、慢性に経過します。1万人に1人くらいが発病し、とくに20～30代の女性に多く、男女比は1対10です。
多くの臓器が侵されるため臨床所見も多彩で、関節症状、皮疹( 蝶形紅斑ちょうけいこうはん、 円板状えんばんじょう紅斑)、中枢神経病変、腎障害、心肺病変、血液異常などがみられます。とくに、中枢神経病変、腎障害があると命にかかわる危険性が高くなります。
原因は何か
SLEは、抗体を作るはたらきをしているBリンパ球が異常に活性化し、それに伴い産生された自己抗体によって、特有の臓器病変が生じると考えられています。
SLEの原因はまだよくわかっていませんが、動物モデルにみられるように、複数の遺伝的要因が関与することは確実だと思われます。これは、ヒトでも一卵性双生児でのSLE発症の一致率が約70％と高いことからも裏づけられます。
こうした遺伝的素因に、何らかの外因（感染症や紫外線など）が加わって発症するものと考えられています。また、女性に圧倒的に多いことから女性ホルモンが関与している可能性も示唆されています。
症状の現れ方
全身の症状として、発熱、全身倦怠感、 易い疲労感、食欲不振、体重減少などがみられます。また、皮膚や関節の症状はこの病気のほとんどの患者さんに現れます。
- 皮膚・粘膜の症状
蝶型紅斑（頬にできる赤い発疹で、蝶が羽を広げた形に似ている）が特徴的です。また、顔面、耳、首のまわりなどにできる円形の紅斑で、中心の色素が抜けてコインのようになるディスコイド疹もみられます。SLEでは日光過敏を認めることが多く、強い紫外線を受けたあとに、皮膚に発疹、水ぶくれができ、発熱を伴うこともあります。また、手のひら、手指、足の裏などにできる霜やけのような発疹も特有な症状です。その他、大量の脱毛や、口腔内や 鼻咽腔（びいんくう）に痛みのない浅い潰瘍ができたりします。
- 関節の症状
とくに、関節炎で発病する場合には、手指にはれや痛みがあるために関節リウマチと間違えられることもありますが、SLEでは関節リウマチと異なって骨の破壊を伴うことはほとんどありません。
- 臓器の症状
腎症状としては、急性期に蛋白尿がみられ、尿 沈渣ちんさでは赤血球、白血球、円柱などが多数出現するのが特徴です（テレスコープ沈渣）。 糸球体腎炎（しきゅうたいじんえん）（ループス腎炎）と呼ばれる腎臓の障害は約半数に現れ、放っておくと重篤となり、ネフローゼ症候群や腎不全に進行して透析が必要になったり、命にかかわったりすることがあります。
心臓や肺では、 漿膜炎（しょうまくえん）（ 心外膜炎しんがいまくえんや 胸膜炎きょうまくえん）の合併が約20％に起こります。 間質性肺炎（かんしつせいはいえん）、肺胞出血、 肺高血圧症（はいこうけつあつしょう）は頻度としては低いですが、難治性です。
腹痛や吐き気がみられる場合には、腸間膜の血管炎やループス 腹膜炎（ふくまくえん）、ループス 膀胱炎（ぼうこうえん）に注意が必要です。
- 中枢神経の症状
中枢神経症状（CNSループス）もループス腎炎と並んで、SLEの重篤な症状です。多彩な精神神経症状がみられますが、なかでも、うつ状態・ 失見当識しつけんとうしき・妄想などの精神症状とけいれん、脳血管障害が多くみられます。
- その他
貧血、白血球減少、リンパ球減少、血小板減少などの血液の異常もよくみられます。また、抗リン脂質抗体という抗体がある場合は、習慣性流早産、血栓症、血小板減少に基づく出血症状などの症状を伴い、抗リン脂質抗体症候群と呼ばれています。
検査と診断
一般的な検査としては、 血沈けっちん（赤血球沈降速度）、尿、末梢血血液検査、胸部X線、心電図などが必要です。免疫血清検査では、免疫グロブリン、補体などの測定に加えて、抗核抗体・抗DNA抗体・抗Sm抗体・抗リン脂質抗体（抗カルジオリピン抗体、ループス抗凝血素、梅毒血清反応生物学的擬陽性）といった自己抗体の検査が重要です。
SLEそのものの診断は、1982年のアメリカリウマチ協会の「改訂基準」（1997年に改変）に照らして行われます。
SLEの活動性の指標としては、抗DNA抗体と補体が最も鋭敏で、活動性が高いと抗DNA抗体は上昇し、補体は低下します。一般的な炎症のマーカーである血清のC反応性蛋白は、SLEではあまり上昇しません。
治療の方法
治療の中心は、免疫のはたらきを抑えることと、炎症を止めることで、そのための第一選択薬は副腎皮質ステロイド薬（ステロイド）です。効果不十分の場合は、ステロイドのパルス療法や免疫抑制薬の併用が行われます。
こうした治療により、現在SLE全体としての5年生存率は90％を超えています。死因で多いのは、中枢神経障害、腎不全、感染症です。
治療に際しては、一人ひとりの重症度、疾患活動性を十分に吟味したうえで、薬の種類や量を決定します。一般的にステロイドは、重症の場合はプレドニゾロンを1日60mg、中等症～軽症の場合は1日20～40mgから開始します。
ステロイドによって症状が軽快し、検査データも改善したら減量を開始しますが、急激な減量は再燃を招く危険があるため、慎重にゆっくりと行います。目安としては、2～4週間ごとに投与量の10％を超えない範囲で減量します。最終的には、プレドニゾロン1日5～10mgを長期間にわたって使用し続ける必要があります。
初回投与量で効果不十分の場合、または減量中に再燃した場合はステロイドを増量します。これでも不十分な場合は、ステロイドのパルス療法や免疫抑制薬(シクロホスファミド1日50～100mg、アザチオプリン1日50～100mg)を併用します。とくに、WHO分類のIV型のループス腎炎に対しては、シクロホスファミドの点滴静注(シクロホスファミドパルス療法)が長期予後の面からも有用性が高いことが証明されていますが、副作用として無月経（生殖器障害）があるので、慎重に適応を考える必要があります。
ステロイドの副作用には、満月様 顔貌がんぼう、消化性潰瘍、糖尿病、感染症、 骨粗鬆症（こつそしょうしょう）などがあります。とくに、ステロイドの内服量が多い間は、ニューモシスチス肺炎などの 日和見ひよりみ感染に気をつける必要があり、ST合剤の内服やペンタミジンの吸入で予防します。骨粗鬆症はほぼ必発で、プレドニゾロン1日5mgであっても進行するため、活性型ビタミンD製剤やビスフォスホネート製剤などの予防内服が必要です。
生活指導
日光 暴露ばくろ、感染症、妊娠、外傷、手術、薬剤アレルギーなどのSLEの 増悪ぞうあく因子を極力避けるようにすることが大切です。SLEでは薬剤アレルギーを有することが多いので、原則として主治医以外からの投薬は受けないようにしてください。
また、感染症を起こした場合でも、決してステロイドを中止してはいけません。これは、長期間にわたるステロイドの内服のために副腎皮質のストレス反応が十分に起きにくくなっているため、中止すると副腎不全を起こしてショック状態になる危険があるからです。
プレドニゾロンを1日20mg以下でSLEの疾患活動性がコントロールされていれば、妊娠・出産が可能です。しかし、分娩後に増悪することが多いので、分娩時よりステロイドを一時的に増量します。手術が必要な場合も、分娩と同様にステロイドを一時的に増量します。
病気の悪化を招いたり、ショックになることがあるので、ステロイドは決して勝手に減らしたりやめたりしてはいけません。
緊急の災害時にはステロイドを一緒にもって避難できるように、普段から少し余分に持っておいたほうがいいでしょう。
《セレーナ・ゴメス》
セレーナ・マリー・ゴメスは、アメリカ合衆国の女優、歌手、ユニセフ親善大使。 ディズニー・チャンネルのオリジナルドラマ『ウェイバリー通りのウィザードたち』のアレックス・ルッソ役で最も知られ、全米ティーンには絶大的な人気を誇る。
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