Turkish Thoracic Journal
Original Article

Evaluation of Patients with Fibrotic Interstitial Lung Disease: Preliminary results from the Turk-UIP Study

1.

Department of Chest Diseases, Istanbul University-Cerrahpasa, Istanbul, Turkey

2.

Department of Chest Diseases, Ege University Medical Faculty, Izmir, Turkey

3.

Department of Chest Diseases, Ondokuz Mayis University Medical Faculty, Samsun, Turkey

4.

Sureyyapaşa Chest Diseases and Thoracic Surgery Training and Research Hospital, University of Health Science, Istanbul, Turkey

5.

Department of Chest Diseases, Gazi University Medical Faculty, Ankara, Turkey

6.

Department of Chest Diseases, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey

7.

Department of Chest Diseases, Akdeniz University Medical Faculty, Antalya, Turkey

8.

Department of Chest Diseases, Necmettin Erbakan University Meram Medical Faculty, Konya, Turkey

9.

Department of Chest Diseases, Duzce University Medical Faculty, Duzce, Turkey

10.

Atatürk Chest Diseases and Thoracic Surgery Training and Research Hospital, University of Health Science, Ankara, Turkey

11.

Dıskapi Yildirim Beyazit Training and Research Hospital, University of Health Science, Ankara, Turkey

12.

Department of Chest Diseases, Dokuz Eylul University Medical Faculty, Izmir, Turkey

13.

American Hospital, Vehbi Koc Foundation

14.

Department of Chest Diseases, Aydin Adnan Menderes University Faculty of Medicine, Aydin, Turkey

15.

Private Sultan Hospital, Diyarbakir, Turkey

16.

Dr. Lütfi Kırdar Kartal Training and Research Hospital, University of Health Science, Istanbul, Turkey

17.

Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital, University of Health Science, Istanbul, Turkey

18.

Department of Chest Diseases, Recep Tayyip Erdoğan University Medical Faculty, Rize, Turkey

19.

Department of Chest Diseases, Kirikkale University Medical Faculty, Kirikkale, Turkey

20.

Department of Chest Diseases, Koç University Medical Faculty, Istanbul, Turkey

21.

Department of Chest Diseases, Karadeniz Technical University Medical Faculty, Trabzon, Turkey

22.

Department of Chest Diseases, Dicle University Medical Faculty, Diyarbakir, Turkey

23.

Department of Chest Diseases, Kocaeli University Medical Faculty, Kocaeli, Turkey

24.

Department of Chest Diseases, Atatürk University Medical Faculty, Erzurum, Turkey

25.

Department of Chest Diseases, Uludağ University Medical Faculty, Bursa, Turkey

26.

Department of Chest Diseases, Pamukkale University Medical Faculty, Denizli, Turkey

27.

Department of Radiodiagnostics, Istanbul University İstanbul Medical Faculty, Istanbul, Turkey

28.

Department of Radiology, Ege University Medical Faculty, Izmir, Turkey

29.

Department of Radiology, Atatürk University Medical Faculty, Erzurum, Turkey

Turk Thorac J 2021; 22: 102-109
DOI: 10.5152/TurkThoracJ.2021.20028
Read: 27 Downloads: 22 Published: 06 April 2021

OBJECTIVE: Differential diagnosis of idiopathic pulmonary fibrosis (IPF) is important among fibrotic interstitial lung diseases (ILD). This study aimed to evaluate the rate of IPF in patients with fibrotic ILD and to determine the clinical-laboratory features of patients with and without IPF that would provide the differential diagnosis of IPF.

MATERIAL AND METHODS: The study included the patients with the usual interstitial pneumonia (UIP) pattern or possible UIP pattern on thorax high-resolution computed tomography, and/or UIP pattern, probable UIP or possible UIP pattern at lung biopsy according to the 2011 ATS/ERS/JRS/ALAT guidelines. Demographics and clinical and radiological data of the patients were recorded. All data recorded by researchers was evaluated by radiology and the clinical decision board. 

RESULTS: A total of 336 patients (253 men, 83 women, age 65.8±9.0 years) were evaluated. Of the patients with sufficient data for diagnosis (n=300), the diagnosis was IPF in 121 (40.3%), unclassified idiopathic interstitial pneumonia in 50 (16.7%), combined pulmonary fibrosis and emphysema (CPFE) in 40 (13.3%), and lung involvement of connective tissue disease (CTD) in 16 (5.3%). When 29 patients with definite IPF features were added to the patients with CPFE, the total number of IPF patients reached 150 (50%). Rate of male sex (p<0.001), smoking history (p<0.001), and the presence of clubbing (p=0.001) were significantly high in patients with IPF. None of the women <50 years and none of the men <50 years of age without a smoking history were diagnosed with IPF. Presence of at least 1 of the symptoms suggestive of CTD, erythrocyte sedimentation rate (ESR), and antinuclear antibody (FANA) positivity rates were significantly higher in the non-IPF group (p<0.001, p=0.029, p=0.009, respectively).

CONCLUSION: The rate of IPF among patients with fibrotic ILD was 50%. In the differential diagnosis of IPF, sex, smoking habits, and the presence of clubbing are important. The presence of symptoms related to CTD, ESR elevation, and FANA positivity reduce the likelihood of IPF.

Cite this article as: Musellim B, Mogulkoc N, Uzun O, et al. Evaluation of Patients with Fibrotic Interstitial Lung Disease: Preliminary results from the Turk-UIP Study. Turk Thorac J 2021; 22(2): 102-9.

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