Interstitial lung disease in systemic sclerosis: current and future treatment

被引：71

作者：

Giacomelli, Roberto ^{[1
]}

Liakouli, Vasiliki ^{[1
]}

Berardicurti, Onorina ^{[1
]}

Ruscitti, Piero ^{[1
]}

Di Benedetto, Paola ^{[1
]}

Carubbi, Francesco ^{[1
]}

Guggino, Giuliana ^{[2
]}

Di Bartolomeo, Salvatore ^{[1
]}

Ciccia, Francesco ^{[2
]}

Triolo, Giovanni ^{[2
]}

Cipriani, Paola ^{[1
]}

机构：

[1] Univ Aquila, Sch Med, Rheumatol Unit, Dept Biotechnol & Appl Clin Sci, Delta 6 Bldg,Via Osped, I-67100 Laquila, Italy

[2] Univ Palermo, Div Rheumatol, Dept Internal Med, Palermo, Italy

来源：

RHEUMATOLOGY INTERNATIONAL | 2017年 / 37卷 / 06期

关键词：

Systemic sclerosis; Scleroderma; Interstitial lung disease; Fibrosis; Treatment; STEM-CELL TRANSPLANTATION; PLACEBO-CONTROLLED TRIAL; ENDOTHELIN TYPE-A; PULMONARY-FIBROSIS; OPEN-LABEL; PULSE CYCLOPHOSPHAMIDE; T-CELL; BRONCHOALVEOLAR LAVAGE; MYCOPHENOLATE-MOFETIL; MOLECULAR-MECHANISMS;

D O I：

10.1007/s00296-016-3636-7

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Systemic sclerosis (SSc) has the highest fatality rate among connective tissue diseases and is characterized by vascular damage, inflammation and fibrosis of the skin and various internal organs. Interstitial lung disease (ILD) frequently complicates SSc and can be a debilitating disorder with a poor prognosis. ILD is the most frequent cause of death in SSc, and the management of SSc-ILD patients is a great challenge. Early detection of pulmonary involvement based on a recent decline of lung function tests and on the extent of lung involvement at high-resolution computed tomography is critical for the best management of these patients. This article summarizes classification, pathogenesis, diagnosis, prognosis, survival and finally current and future treatment options in SSc-ILD.

引用

页码：853 / 863

页数：11

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