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Table of Contents
ORIGINAL ARTICLE
Year : 2018  |  Volume : 67  |  Issue : 1  |  Page : 50-55

Sonographic features of idiopathic pulmonary fibrosis


1 Chest Department, Faculty of Medicine, Menoufia University, Shebin El-Kom, Egypt
2 Chest Department, EL Zaton Specialized Hospital, Cairo, Egypt
3 Department of Radiology, Faculty of Medicine, Menoufia University, Shebin El-Kom, Egypt

Date of Submission21-Dec-2017
Date of Acceptance10-Jan-2018
Date of Web Publication21-Mar-2018

Correspondence Address:
Mohammed A Agha
Assistant Professor of Chest Diseases and Tuberculosis, Chest Department, Menoufia University, Shebin El-Kom, 32511
Egypt
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ejcdt.ejcdt_38_17

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  Abstract 

Background Idiopathic pulmonary fibrosis (IPF) is a specific form of idiopathic interstitial pneumonia (IIP) that occurs primarily in the elderly and is confined to the lungs. With the use of transthoracic ultrasound (TUS), diffuse parenchymal lung disease should be considered if multiple comet-tail artifacts distributed over the whole surface of the lung together with a thickened and irregular, fragmented pleural line are visible.
Objective The aim of this study was to evaluate lung ultrasound usefulness in the diagnosis of IPF.
Patients and methods This study was carried out on 40 patients diagnosed as having IPF. All cases were subjected to a full assessment of history, clinical examination, laboratory investigations, arterial blood gases, pulmonary function test, plain chest radiography, high-resolution computed tomography, and TUS.
Results Our results showed that B-lines in combination with thickened and irregular pleura are the TUS features of IPF. There was a statistically significant correlation among B-lines distance (mm), pleural line thickness (mm), pleural line irregularity, abolished lung sliding found by TUS, and severity of the disease.
Conclusion TUS is a useful tool in the diagnosis and assessment of the severity of IPF and can be used as a complementary method beside high-resolution computed tomography. The distance between two adjacent lines in combination with thickened and irregular pleura is a good feature in the follow-up of disease progression.

Keywords: high-resolution computed tomography, idiopathic pulmonary fibrosis, transthoracic ultrasound


How to cite this article:
Mansour OF, Agha MA, Al-Asdody AA, Mehana NS, Habib RM. Sonographic features of idiopathic pulmonary fibrosis. Egypt J Chest Dis Tuberc 2018;67:50-5

How to cite this URL:
Mansour OF, Agha MA, Al-Asdody AA, Mehana NS, Habib RM. Sonographic features of idiopathic pulmonary fibrosis. Egypt J Chest Dis Tuberc [serial online] 2018 [cited 2020 Apr 3];67:50-5. Available from: http://www.ejcdt.eg.net/text.asp?2018/67/1/50/228135


  Introduction Top


Idiopathic pulmonary fibrosis (IPF) is one of the commonest types of idiopathic interstitial pneumonia, which is the main type of diffuse interstitial lung diseases (DILD). To diagnose IPF in the absence of surgical lung biopsy, two conditions are required: first is the exclusion of other causes of diffuse parenchymal lung diseases (DPLD), and the second, which is the most important, is the characteristic pattern on high-resolution computed tomography (HRCT) which is the usual interstitial pneumonia (UIP) [1]. By monitoring increasing symptoms, deterioration of pulmonary function test (PFT) results, and increased fibrosis on HRCT, the severity of the disease can be assessed. Chest radiography does not have a big role in the diagnosis of IPF. The presence of bilateral, basal, and subpleuritic reticular pattern is the main feature of UIP [2]. Traction bronchiectasis and honeycombing are common features with basal and subpleural distribution. Usually in a less extensive way than the reticular pattern, ground glass opacities (GGO) may be present on HRCT in patients with IPF. Other pleural abnormalities such as pleural plaques, effusion, or calcifications make an alternative etiology for UIP pattern more common than IPF [3]. Moreover, in the presence of air trapping, nodules, or consolidations, other alternative diagnosis should be considered [4]. One of the main advantages of transthoracic ultrasound (TUS) is the absence of electromagnetic radiation [5]. The importance of TUS in the diagnosis of different respiratory disorders has been established in different studies [6],[7],[8]. Beside its diagnostic value, TUS has a big role in different lung interventional methods such as biopsies and thoracentesis [9]. Bilateral B-lines with pleural thickening and pleural irregularity are the main TUS features in DPLD [10]. B-lines occur owing to the thickening occurring in the subpleural interlobular septa. However, B-lines may occur in patients with other pulmonary disorders as chronic obstructive pulmonary disease, bronchiolitis obliterans with organizing pneumonia, pulmonary alveolar proteinosis, and pulmonary embolism [11],[12].

Aim of the work

The aim of this work was to assess the usefulness of lung ultrasound (US) in the diagnosis of IPF.


  Patients and methods Top


A prospective cohort study was conducted on already diagnosed 40 patients with IPF (on the basis of exclusion of other known causes of DPLDs and the presence of UIP pattern on HRCT in the form of basilar, subpleural reticular opacities, and honeycombing with or without traction bronchiectasis) [1] who were admitted to the Chest Department, Menoufia University Hospitals, from September 2015 to September 2017. This study was approved by the ethical committee of the Menoufia University.

All patients were subjected to the following:
  1. History and clinical examination: it included symptoms and signs, comorbidities, occupations, special habits of medical importance, and if there was relevant drug history or relevant family history.
  2. Laboratory investigations: it included arterial blood gases, liver functions tests, kidney function tests, and complete blood count.
  3. Chest radiography: it was done in posteroanterior view and lateral if needed.
  4. PFTs: it was done to confirm the restrictive pattern of the disease and assess its severity for every patient.


Chest high-resolution computed tomography assessment

HRCT examination was performed for all patients, and the extent of each abnormality was estimated for each lobe.
  1. Transthoracic ultrasonography: All the patients underwent TUS examination using an US scanner (digital US imaging system model equipped with a 3.5-MHz convex probe and 7.5–10 MHz linear probe; Philips ultrasound Affiniti 50G, Philips, Germany). All patients had their examination in a sitting or supine position with arms raised above their head. Lung ultrasonography was performed in a series of scan lines along the chest wall with the transducer oriented either perpendicular or transverse to the chest wall.
  2. Transthoracic lung US interpretation: Assessment of the pleura was done regarding pleural thickness and pleural surface (smooth, irregular, or interrupted). Pleural thickenings are defined as focal or diffuse echogenic lesions arising from the visceral or parietal pleura that are greater than 3 mm in width with or without an irregular pleural surface [13]. Presence or absence of lung sliding (the ‘to-and-fro’ dynamic movement of the lung during respiration) was estimated; lung sliding was demonstrated as fleeting dots arising from the pleural line which moves with lung movement during breathing [14]. Recognition of B-lines, which were previously called comet-tail artifacts, appearing on the screen as laser-like vertical echogenic artifacts arising from the pleural line, spreading up without fading to the edge of the screen, and moving synchronous with lung sliding, generated from the thickened interlobular septa at the lung wall interface, was assessed [15],[16]. By parasternal, anterior, and posterior axillary lines, each hemithorax was divided into three regions, which were subdivided into upper and lower regions. Finally, there were six regions for each hemithorax. The presence of at least three B-lines in one region made it a positive region, and the presence of at least two positive regions bilaterally made it a positive examination result. Measurement in millimeters of the distance between two adjacent B-lines was done [15].


Statistical analysis

Data were coded and analyzed using SPSS 17. Data were expressed as median and percentiles for quantitative nonparametric measures in addition to both number and percentage for categorized data [16].


  Results Top


This study (prospective cohort study) was done on 40 patients diagnosed as having IPF. Their ages ranged from 47 to 65 years old. They were 24 male and 16 female patients, with 21 smokers. The most common presenting symptom was dyspnea followed by dry cough, and the most common comorbidity was gastroesophageal reflux (GERD) ([Table 1]). Regarding the TUS features found in the studied group, B-lines were found in all (100%) patients. Regarding pleural line features, 85% of patients with IPF had irregular pleural lines, with 77.5% of the 40 patients having pleural thickening. Overall, 82.5% of the studied patients had normal lung sliding, and in 92.5% of the patients, subpleural nodules were absent ([Table 2]). There was a statistically negative significant correlation between the distances between each of two adjacent B-lines and with both forced vital capacity and partial arterial oxygen pressure in the studied patients ([Table 3]). Regarding the correlation between TUS features and the degree of severity of IPF (measured by degree of restriction in PFTs), increased distance (in mm) between B-lines, pleural thickening and irregularity, and absence of normal lung sliding were associated with the severity of the disease ([Table 4]). The distance between two adjacent B-lines correlated with the degree of extension of reticular pattern in HRCT ([Figure 1],[Figure 2],[Figure 3]).
Table 1 Demographic data of the studied group

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Table 2 Thoracic ultrasound features in the studied group

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Table 3 Correlations between distance a mong B-lines and both forced vital capacity and PaO2

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Table 4 Correlations of ultrasonographic pleural features and severity of pulmonary function test

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Figure 1 Correlation between reticular patterns on high-resolution computed tomography and B-line distance in patients with idiopathic pulmonary fibrosis.

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Figure 2 A 55-year-old male patient presented with progressive dyspnea. high-resolution computed tomography shows bilateral basal reticular pattern, and transthoracic ultrasound shows the presence of B-lines with irregular pleural lines.

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Figure 3 A 61-year-old male patient with bilateral basal mild reticular pattern, confirmed with transthoracic ultrasound features (pleural thickening and B-lines).

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  Discussion Top


To diagnose IPF in the absence of surgical lung biopsy, two conditions are required: first the exclusion of other causes of DPLD, and the second one, which is the most important, is the characteristic pattern on HRCT, which is the UIP. The importance of TUS in the diagnosis of different respiratory disorders has been established in different studies [6],[7],[8]. The present study was designed to evaluate ultrasonographic features in patients with IPF; moreover, the results of the US examination were compared with PFTs, and with the arterial oxygen tension. This study was done on 40 patients diagnosed as having IPF. Their ages ranged from 47 to 65 years. In the present work, regarding the sex, there was a male predominance (24 male and 16 females), with 21 being smokers. The most common presenting symptom was dyspnea followed by dry cough, and the most common comorbidity was GERD ([Table 1]). The previous findings are in agreement with many studies that concluded that IPF should be considered in all adult patients with unexplained progressive dyspnea and commonly presents with cough, bibasilar inspiratory crackles, and finger clubbing [17],[18],[19]. Moreover, many studies illustrated the increasing incidence of the disease with patients in old ages, especially in the sixth and seventh decades of lift [20],[21],[22],[23]. Nadrous et al. [24] and Iwai et al. [25] concluded that the occurrence of IPF in patients younger than 50 years is rare, and in such patients, the incidence of pulmonary fibrosis secondary to connective tissue disorders is the commonest differential diagnosis. Moreover, the studies showing the increased incidence of IPF in men more than women and the increased incidence in smokers are present [26]. Hubbard et al. [27] and Steele et al. [28] concluded that smoking is strongly associated with IPF, particularly for individuals with a smoking history of more than 20 pack-years. Regarding GERD, several studies concluded that GERD is a risk factor for IPF. GERD is common in patients with IPF [23],[29],[30]. GERD is frequent in the normal population as well as in patients with other advanced lung diseases such as lung fibrosis associated with scleroderma [31]. In the present study, all the 40 (100%) cases had bilateral diffuse B-lines, three (7.5%) cases had subpleural lesions, 34 (85%) cases had irregular pleural line, and 31 (77.5%) cases had pleural thickening. Moreover, in seven (17.5%) cases, lung sliding was absent ([Table 2]). [Table 3] shows that increased distance between two adjacent B-lines was associated with severity of restriction of lung functions and severity of the degree of hypoxemia. In addition, [Table 4] shows that features of IPF detected with TUS, such as distance between B-lines, irregularity of pleural lines, thickened pleural lines, and absence of lung sliding, were associated with the severity of the degree of restrictive pulmonary functions. In the present work, the degree of reticulation in HRCT was associated with the severity of the conditions ([Figure 1]). Many studies were done to assess the value of TUS in patients with interstitial lung diseases including IPF, and all studies illustrated the importance of B-lines in the diagnosis DPLD. Targhetta et al. [32] studied TUS in 12 patients with pulmonary sarcoidosis and found that all patients had both an irregular pleural surface and B-lines. Moreover, Gargani et al. [33] found that 51% of the patients with systemic sclerosis (SSc) had B-lines as a hallmark of interstitial pulmonary fibrosis. Suzan et al. [34] found that the most predominant artifact type among patients with ILD was B-lines (73.8%). Hasan and Makhlouf [35] found that all their 61 patients with DILD had diffuse bilateral B-lines. Comparable results were obtained by Reissig and Kroegel [14] who found that 84.9% of their patients with DPLDs had thickened pleural surface and 98.1% had irregular pleura. Moreover, Sperandeo et al. [36] studied TUS features of pulmonary fibrosis in patients with different disease severity and found that 100% of the patients had irregular and fragmented pleural lines, with thickened pleura in 92.8% and reduction or absence of normal lung sliding sign in 28% of cases. In a study done by Reissig and Kroegel [14], at least six B-lines per scan were found with an irregular pleural line. It also was similar to the results obtained by Moazedi-Fuerst et al. [37] who studied TUS findings in 25 patients with SSc and 25 healthy individuals as control group and found that 44% of patients with SSc had B lines with pleural thickening. Buda et al. [38] correlated the US findings of IPF with the HRCT findings, and found that features of IPF were the presence of B-lines, irregular with thickened pleural lines. Moazedi-Fuerst et al. [37] studied the role of TUS in 55 patients with different connective tissue disorders and 40 healthy control group. B-lines, subpleural nodes, and irregularities of the pleura were assessed with TUS. These features assessed with TUS were significantly more frequent in IPF group. In the present study, the distance between two adjacent B lines negatively correlated with forced vital capacity and PaO2 ([Table 3]) and positively correlated with the reticular pattern in HRCT ([Figure 1]). The previous findings are in accordance with the results of Hasan and Makhlouf [35] and Assayag et al. [39] who stated that the distance between two adjacent B-lines is positively correlatedwith the severity and extension of the fibrosis on using the Warrick scoring system on HRCT, and this also agreed with Suzan et al. [34] who studied the correlation between distance between two adjacent B-lines and found positive correlations with reticular pattern and negative correlation with GGO. The same findings were supported by Bouhemad et al. [40] as they concluded that multiple B-lines are associated with reticular pattern, whereas B-lines 3 mm or less apart are associated with GGO. Moreover, Gargani et al. [33], Hasan and Makhlouf [34], and Suzan et al. [35] proved the correlation between distance between B-line and PFTs and found that there was deterioration of the disease with increased distance between B-lines. Regarding the data obtained from [Table 4], there was a significant difference in median value of pleural thickness (mm) between all groups involved. In addition, there was a significant difference in percentage of patients with irregular pleura and absent or reduced lung sliding between mild, moderate, and severe groups. Moreover, all severe cases showed irregular pleural line over whole lung (100%), thickened pleura, and nearly absent lung sliding. In this study, different TUS features for IPF were found, including thickened pleura, irregular pleural lines, absent lung sliding, and the presence of B-lines ([Table 2]). This is in agreement with Sperandeo et al. [36] who studied 84 consecutive patients. They found that features of IPF in TUS were fragmented, irregular thickening pleural line, subpleural cysts, and reduction or absence of the sliding sign.


  Conclusion Top


Chest US can be used as a complementary method for the diagnosis of IPF beside HRCT. TUS is a good method for follow-up patients with IPF as chest US is an effective noninvasive modality that requires neither ionizing radiation nor a contrast medium. Bilateral B-lines in combination with thickened and irregular pleura are a good TUS features for assessment of the severity of IPF. Increased B-lines distance can be used as a marker of pulmonary function deterioration and for the presence of increased reticular pattern on HRCT.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
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    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4]


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