Pulmonary Manifestations of Systemic Sclerosis and Mixed Connective Tissue Disease

被引：35

作者：

Perelas, Apostolos ^{[1
]}

Arrossi, Andrea, V ^{[2
]}

Highland, Kristin B. ^{[1
]}

机构：

[1] Cleveland Clin Fdn, Resp Inst, 9500 Euclid Ave, Cleveland, OH 44195 USA

[2] Cleveland Clin Fdn, Pathol & Lab Med Inst, 9500 Euclid Ave, Cleveland, OH 44195 USA

来源：

CLINICS IN CHEST MEDICINE | 2019年 / 40卷 / 03期

关键词：

Systemic sclerosis; Scleroderma; Interstitial lung disease; Pulmonary hypertension; Mixed connective tissue disease; INTERSTITIAL LUNG-DISEASE; STEM-CELL TRANSPLANTATION; ARTERIAL-HYPERTENSION; VENOOCCLUSIVE DISEASE; RISK-FACTORS; CLINICAL CHARACTERISTICS; SPONTANEOUS PNEUMOTHORAX; PULSE CYCLOPHOSPHAMIDE; FUNCTION TESTS; CT FEATURES;

D O I：

10.1016/j.ccm.2019.05.001

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

Systemic sclerosis (SSc) is a rare disease characterized by widespread collagen deposition resulting in fibrosis. Although skin involvement is the most common manifestation and also the one that determines the classification of disease, mortality in SSc is usually a result of respiratory compromise in the form of interstitial lung disease (ILD) or pulmonary hypertension (PH). Clinically significant ILD is seen in up to 40% of patients and PH in up to 20%. Treatment with either cyclophosphamide or mycophenolate has been shown to delay disease progression, whereas rituximab and lung transplantation are reserved for refractory cases.

引用

页码：501 / +

页数：19

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