限局型強皮症の臨床的意義のある間質性肺疾患

[改訂版]「強皮症における病因解明と根治的治療法の開発」重症度分類・治療指針試案(2007)医師向け[PDF]

全身性強皮症 (SSc) 病型分類　（ LeRoy と Medsgerによる, 一部改変）
diffuse cutaneous SSc(dSSc)

皮膚硬化:肘関節より近位皮膚硬化
進行:急速（皮膚硬化出現２年以内）
Raynaud 現象と皮膚硬化:皮膚硬化が先行するかほぼ同時
毛細管顕微鏡所見:毛細血管の脱落
爪上皮内出血点:進行期には消失
腱摩擦音:腱摩擦音 （ ＋ ） （但し日本人では少ない）
関節拘縮:高度
石灰沈着:まれ
主要臓器病変:肺，腎（日本人ではまれ），心，食道
主要抗核抗体:抗トポイソメラーゼＩ抗体
抗 RNAポリメラーゼ抗体

limited cutaneous SSc(lSSc)

皮膚硬化:肘 関節より遠位皮膚硬化
進行:緩徐（皮膚硬化出現５年以上）
Raynaud 現象と皮膚硬化:Raynaud 現象が先行
毛細管顕微鏡所見:毛細血管の蛇行，拡張
爪上皮内出血点:多数
腱摩擦音:腱摩擦音（ ）
関節拘縮:軽度
石灰沈着:多い
主要臓器病変:肺高血圧症（日本人でまれ），食道
主要抗核抗体:抗セントロメア抗体

Clinically Significant Interstitial Lung Disease in Limited Scleroderma
Histopathology, Clinical Features, and Survival
(Chest. 2008; 134:601-605)

All patients presented with significant respiratory symptoms.

Twenty-two of 27 subjects had surgical lung biopsy-proven ILD, and 5 subjects had miscellaneous non-ILD patterns.

Of those subjects with ILD, 64% (14 of 22 subjects) had a nonspecific interstitial pneumonia (NSIP) pathologic pattern (fibrotic NSIP, 13 subjects; cellular NSIP, 1 subject), and 36% (8 of 22 subjects) had the usual interstitial pneumonia (UIP) pattern.

Subjects with NSIP were younger (median age, 42 vs 58 years, respectively; p = 0.003), but no differences were noted in pulmonary physiology (FVC: NSIP group, 52% predicted; UIP group, 65% predicted; p = 0.22; diffusing capacity of the lung for carbon monoxide: NSIP group, 40% predicted; UIP group, 42% predicted; p = 1.0).

All patients had limited skin involvement. The Scl-70 antibody was absent among those assessed (NSIP group, 0 of 10 subjects; UIP group, 0 of 7 subjects).

All patients were treated with cytotoxic therapy. The median survival time for those with NSIP was 15.3 years (5,596 days) compared with 3 years (1,084 days) for those with UIP (p = 0.07 [log-rank test]).