Lung-dominant connective tissue disease (LD-CTD)

44症例の後顧的解析

Lung-dominant connective tissue disease (LD-CTD) は間質性肺炎の疾患概念

その臨床的、レントゲン的、組織学的報告


Lung-Dominant Connective Tissue Disease Clinical, Radiologic, and Histologic Features
Norihito Omote, et. al.
CHEST 2015; 148(6):1438-1446

44例の連続血清学的確認LD-CTD手術的肺生検施行患者 

組織所見
UIP　25、　NSIP 13
2つ以上の組織学的特性を持つLD-CTDは、h-UIP 15、 h-NSIP 11
h-UIP　15例　はHRCT上　inconsistent UIPパターン
MDD後、h-UIP 18例は unclassifiable IIP


 h-UIP患者では有意に年時％予測肺活量変化改善し、その症例はh-UIPより生存率良好 (p=0.02)

生存率はHRCTパターンと相関せず

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ABBREVIATIONS:
ANA antinuclear antibody
anti-Jo1 anti-tRNA synthetase
CS  corticosteroid
CTD  connective tissue disease
h-NSIP  histologic nonspeci c interstitial pneumonia
HRCT high- resolution CT
h-UIP  histologic usual interstitial pneumonia
IIP idiopathic interstitial pneumonia
ILD interstitial lung disease
IPF idiopathic pulmonary  brosis
LD-CTD  lung-dominant connective tissue disease
MDD multidisciplinary discussion
NSIP  nonspeci c interstitial pneumonia
OPorganizing pneumonia
%FVCpercent predicted FVC
PFT  pulmonary function test
SLB surgical lung biopsy
UIP  usual interstitial pneumonia