Scleroderma - Scleroderma
What is the disease? Scleroderma is a relatively rare disease whose main symptom is hardening of the skin. There are two types of scleroderma: localized scleroderma, in which hardening is limited to certain parts of the skin, and generalized scleroderma, in which not only the skin but also blood vessels and internal organs are affected at the same time. Systemic sclerosis is characterized by not only hardening of the skin, but also frequent peripheral circulatory disorders and autoantibodies. It can be seen in men and women of all ages, from infants to the elderly, but is particularly prevalent in women in their 30s to 50s. What is the cause? The cause has not yet been identified, but it is believed to be triggered by certain environmental factors in addition to a congenital predisposition. However, it is not a genetic or infectious disease. Environmental factors include exposure to certain chemicals such as organic solvents and drugs. How symptoms manifestThe initial symptom is often Raynaud's phenomenon, but it may also begin with joint pain, tightness of the skin, or swelling. Raynaud's phenomenon is a phenomenon in which the fingertips turn white in response to cold stimulation or mental stress, and then change color to purple and red, reflecting circulatory disorders (Figure 2B). When the skin becomes hard, it becomes difficult to pinch the skin, it turns black even without sunburn (pigmentation), and the fingers become deformed ( The hardening of the skin slowly spreads from the fingers, back of the hand, forearm and down the center of the body. Testing and diagnosisIt is easy to diagnose when there is obvious hardening of the skin. However, in mild cases or when the disease has only recently developed, diagnosis can be difficult, and in such cases the diagnostic criteria (Table 3) are helpful. As the speed, degree, and scope of the progression of the hardening of the skin vary, it is classified into two disease types (Table 4). Blood tests for autoantibodies such as anti-topoisomerase I (Scl70) antibodies and anticentromere antibodies are useful for diagnosis and disease classification. Systemic sclerosis can cause a variety of visceral lesions, as shown in Table 5, and various tests are performed as necessary to check for the presence or absence of these and their severity. Treatment methods Depending on the type of disease and the extent of internal organ involvement, whether or not treatment is necessary and, if so, what type of treatment is required will be decided. Treatment methods also vary depending on the disease itself, and the type of treatment may change the natural course of the disease. The number of patients who require disease-modifying therapy (mainly diffuse type) is less than half, which is not a large number. Unfortunately, there is currently no treatment that has been proven to be effective as disease-modifying therapy, but corticosteroids (prednisone) and immunosuppressants (endoxan) are used. Symptomatic treatments include vasodilators and prostaglandin preparations for circulatory disorders, proton pump inhibitors for esophageal lesions, prostacyclin preparations for pulmonary hypertension, endothelin receptor antagonists, and PDE5 inhibitors. What to do if you notice an illnessIf you experience symptoms related to systemic sclerosis, such as Raynaud's phenomenon or skin hardening, please visit a medical institution and receive a medical examination. The medical specialty to be examined should be internal medicine (department that deals with rheumatism and collagen diseases) or dermatology, but there is no need to be particular about the medical specialty as long as the doctor has the expertise and experience. Symptoms vary from patient to patient, so treatment and attention to daily life according to the condition are required. For this reason, regular visits to a medical institution are essential. Masataka Kuwana "> Figure 2. Main hand symptoms seen in scleroderma "> Table 3 Diagnostic criteria for scleroderma "> Table 4. Classification of scleroderma types "> Table 5 Visceral lesions seen in scleroderma Source: Houken “Sixth Edition Family Medicine Encyclopedia” Information about the Sixth Edition Family Medicine Encyclopedia |
どんな病気か 強皮症は皮膚が硬くなることを主症状とする比較的まれな病気です。強皮症のなかには、硬化が皮膚の一部に限られる限局性強皮症と、皮膚だけでなく血管や内臓も同時に侵され、 全身性強皮症では皮膚の硬化だけでなく、末梢循環障害と自己抗体を高頻度に伴うことが特徴です。幼児からお年寄りまですべての年齢の男女にみられますが、とくに30～50代の女性に好発します。 原因は何か 今のところ原因は特定されていませんが、生まれながらにもっている素因に加えて、ある種の環境要因が引き金になって起こると考えられています。ただし、遺伝病や伝染病ではありません。環境要因として、有機溶媒や薬剤など特定の化学物質への 症状の現れ方初発症状の多くはレイノー現象で、そのほかに関節痛、皮膚のつっぱり感やむくみ感で始まる場合もあります。レイノー現象とは寒冷刺激や精神的な緊張がきっかけで指先が白くなり、引き続いて紫、赤と色調が変化する現象で、循環障害を反映しています（図2B）。 皮膚が硬くなると、皮膚をつまみづらい、日焼けしていないのに黒くなる（色素沈着）、手指の変形（ 皮膚の硬化は手指から手背、前腕と体の中心に向かってゆっくり広がっていきます。 検査と診断はっきりした皮膚の硬化が存在する場合の診断は容易です。ただし、軽症の例や発症して間もないと診断が難しい場合もあり、その際には診断基準（表3）が参考になります。皮膚硬化の進行の速さや程度、範囲はさまざまなことから、2つの病型に分類されています（表4）。 診断や病型分類に際しては抗トポイソメラーゼI（Scl70）抗体や抗セントロメア抗体などの自己抗体の血液検査が参考になります。 全身性強皮症では表5に示すような多様な内臓病変を来し、これらの有無と重症度を検索するため、必要に応じてさまざまな検査が行われます。 治療の方法 病型と内臓病変の程度によって、治療が必要かどうか、必要であればどのような治療を行うのかを決めます。また、治療法も、全身性強皮症の病気そのものの自然経過を変える疾患 疾患修飾療法が必要になる患者さん（主にびまん型）は半数以下で、決して多くありません。残念ながら現時点で疾患修飾療法としての有効性が証明された治療法はありませんが、副腎皮質ステロイド薬（プレドニン）、免疫抑制薬（エンドキサン）などが用いられます。 対症療法薬としては循環障害に対する血管拡張薬やプロスタグランジン製剤、食道病変に対するプロトンポンプ阻害薬、肺高血圧に対するプロスタサイクリン製剤、エンドセリン受容体拮抗薬、PDE5阻害薬などがあります。 病気に気づいたらどうするレイノー現象や皮膚硬化など全身性強皮症と関連する症状があれば医療機関を受診し、診察を受けてください。診療科としては内科（リウマチや膠原病を扱っている科）あるいは皮膚科ですが、専門知識と経験をもつ医師であれば診療科にこだわる必要はありません。 患者さんごとに症状は多様ですので、病状に応じた治療と日常生活の注意が必要になります。そのためには、定期的な医療機関への受診が不可欠です。 桑名 正隆 "> 図2 強皮症にみられる主な手の症状 "> 表3 強皮症の診断基準 "> 表4 強皮症の病型分類 "> 表5 強皮症にみられる内臓病変 出典 法研「六訂版 家庭医学大全科」六訂版 家庭医学大全科について 情報 |
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