Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry

被引：191

作者：

Behr, Juergen ^{[1
]}

Kreuter, Michael ^{[2
,3
]}

Hoeper, Marius M. ^{[4
,5
]}

Wirtz, Hubert ^{[6
]}

Klotsche, Jens ^{[7
]}

Koschel, Dirk ^{[8
]}

Andreas, Stefan ^{[9
,10
]}

Claussen, Martin ^{[11
]}

Grohe, Christian ^{[12
]}

Wilkens, Henrike ^{[13
]}

Randerath, Winfried ^{[13
]}

Skowasch, Dirk ^{[14
]}

Meyer, F. Joachim ^{[15
]}

Kirschner, Joachim ^{[16
]}

Glaeser, Sven ^{[17
]}

Herth, Felix J. F. ^{[18
,19
]}

Welte, Tobias ^{[20
,21
]}

Huber, Rudolf Maria ^{[22
]}

Neurohr, Claus ^{[22
]}

Schwaiblmair, Martin ^{[23
]}

Kohlhaeufl, Martin ^{[24
]}

Hoeffken, Gert ^{[25
]}

Held, Matthias ^{[26
]}

Koch, Andrea ^{[27
]}

Bahmer, Thomas ^{[28
]}

Pittrow, David ^{[29
]}

机构：

[1] LMU, Med Klin & Poliklin 5, Comprehens Pneumol Ctr Munich, Munich, Germany

[2] Univ Klinikum Heidelberg, Pneumol & Beatmungsmed, Heidelberg, Germany

[3] Mitglied Deutsch Zentrums Lungenforsch DZL, Translat Lung Res Ctr Heidelberg TLRC, Heidelberg, Germany

[4] Hannover Med Sch, Klin Pneumol, D-30623 Hannover, Germany

[5] Hannover Med Sch, German Ctr Lung Res, D-30623 Hannover, Germany

[6] Univ Klinikum Leipzig AoR, Dept Innere Med Neurol & Dermatol, Abt Pneumol, Leipzig, Germany

[7] Deutsch Rheuma Forschungsinst, Epidemiol, Berlin, Germany

[8] Fachkrankenhaus Coswig, Zentrum Pneumol, Coswig, Germany

[9] Univ Gottingen, Pneumol Lehrklin, Lungenfachklin Immenhausen, D-37073 Gottingen, Germany

[10] Univ Hosp Gottigen, D-37073 Gottingen, Germany

[11] Mitglied DZL, Akad Lehrkrankenhaus Univ Schleswig Holstein, LungenClin Grosshansdorf, Grosshansdorf, Germany

[12] Klin Pneumol ELK, Berlin, Germany

[13] Univ Klinikum Univ Kliniken Saarlandes, Klin Innere Med 5, Pneumol, Homburg, Germany

[14] Univ Klinikum Bonn, Med Klin & Poliklin 2, Bonn, Germany

[15] Stadt Klinikum Munchen GmbH, LZM Bogenhausen Harlaching, Lungenzentrum Munchen, Munich, Germany

[16] Ctr Internal Med Studies CIMS, Bamberg, Germany

[17] Univ Med, Klin & Poliklin Innere Med B, Forsch Bereich Pneumol & Pneumol Epidemiol, Greifswald, Germany

[18] Univ Klinikum Heidelberg, Pneumol & Beatmungsmed, Heidelberg, Germany

[19] Mitglied DZL, TLRC, Heidelberg, Germany

[20] Hannover Med Sch, Klin Pneumol, D-30623 Hannover, Germany

[21] Hannover Med Sch, German Ctr Lung Res, D-30623 Hannover, Germany

[22] LMU, Comprehens Pneumol Ctr Munich, Med Klin & Poliklin 5, Munich, Germany

[23] Klinikum Augsburg, Med Klin 1, Augsburg, Germany

[24] Robert Bosch Krankenhaus, Klin Schillerhohe, Gerlingen, Germany

[25] Fachkrankenhaus Coswig, Zentrum Pneumol, Coswig, Germany

[26] Missionsarztl Klin Wurzburg, Innere Med Abt, Wurzburg, Germany

[27] Ruhr Univ Bochum, Berufsgenossenschaftl Univ Klinikum Bergmannsheil, Med Klin & Poliklin 1, Bochum, Germany

[28] Mitglied DZL, Akad Lehrkrankenhaus Univ Schleswig Holstein, LungenClin Grosshansdorf, Grosshansdorf, Germany

[29] Tech Univ Dresden, Fak Med, Inst Klin Pharmakol, D-01062 Dresden, Germany

来源：

EUROPEAN RESPIRATORY JOURNAL | 2015年 / 46卷 / 01期

关键词：

DIAGNOSIS; GUIDELINES; ALVEOLITIS; THERAPY; SOCIETY; VALUES; TRIAL; SGRQ;

D O I：

10.1183/09031936.00217614

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

100201 [内科学];

摘要：

After introduction of the new international guidelines on idiopathic pulmonary fibrosis (IPF) in 2011, we investigated clinical management practices for patients with IPF according to physicians' diagnoses. A prospective, multicenter, noninterventional study with comprehensive quality measures including on-site source data verification was performed in Germany. 502 consecutive patients (171 newly diagnosed, 331 prevalent; mean+-/SD age 68.7+-/9.4 years, 77.9% males) with a mean disease duration of 2.3+-/3.5 years were enrolled. IPF diagnosis was based on clinical assessments and high-resolution computed tomography (HRCT) in 90.2%, and on surgical lung biopsy combined with histology in 34.1% (lavage in 61.8%). The median 6-min walk distance was 320 m (mean 268+-/200 m). The mean forced vital capacity was 72+-/20% pred and diffusing capacity of the lung for carbon monoxide was 35+-/15% pred. No drugs were administered in 17.9%, oral steroids in 23.7%, N-acetylcysteine in 33.7%, pirfenidone in 44.2% and other drugs in 4.6% of patients. Only 2.8% of the cohort was listed for lung transplantation. IPF patients were diagnosed in line with the new guidelines. They had more severe disease than those enrolled in recent randomised controlled trials. In addition to HRCT, the frequency of lung biopsies was surprisingly high. Treatment patterns varied substantially.

引用

页码：186 / 196

页数：11

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