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(Chest. 2004;125:135-142.)
© 2004 American College of Chest Physicians

Diffuse Cystic Lung Diseases*

Correlation Between Radiologic and Functional Status

Giuseppe Paciocco, MD, PhD, FCCP; Elisabetta Uslenghi, MD; Achille Bianchi, MD; Gennaro Mazzarella, MD; Giancarlo C. Roviaro, MD, FCCP; Giuseppe Vecchi, MD and Sergio Harari, MD

* From the Department of Cardio-Thoracic and Respiratory Science (Drs. Paciocco and Mazzarella), Second University of Naples, Naples, Italy; and the Departments of Chest Diseases (Drs. Bianchi and Harari), Radiology (Drs. Uslenghi and Vecchi), and General Surgery (Dr. Roviaro), San Giuseppe Hospital, AFAR, Milan, Italy.

Correspondence to: Sergio Harari, MD, Direttore Unità Operativa di Pneumologia, Ospedale San Giuseppe, 20123 Milan, Italy; e-mail: sharari{at}oh-fbf.it


   Abstract
TOP
 Abstract
Introduction
 Materials and Methods
 Results
 Discussion
 References
 
Background: High-resolution CT (HRCT) scanning plays an important role in the diagnosis of diffuse cystic lung diseases (DCLDs). However, its role in the clinical evaluation of patients affected by DCLD has not yet been well-clarified. At present, pulmonary function tests are the only methods available for the evaluation of lung impairment due to these diseases, but their sensitivity and reliability are still limited.

Purpose: The aim of this study was to correlate the quantitative score of cystic-aerial lesions obtained by a HRCT density mask (DM) software with pulmonary function data in DCLDs.

Methods: Spirometry, lung volumes, diffusion capacity, arterial blood gas (ABG) analysis, 6-min walking test (6-MWT), and HRCT with DM quantitative evaluation were performed in a cohort of 25 patients (lymphangioleiomyomatosis [LAM], 13 patients; Langerhans cells histiocytosis [LCH], 12 patients). Linear regression was used for the statistical analysis. The sum and mean of the air-trapping percentages at three different levels of DM study (ie, aortic arch, left lower lobe bronchus origin, and 2 cm from the diaphragmatic muscle), and various functional parameters and exercise performance values were matched for the analysis.

Results: An obstructive pattern was present in 13 patients (52%; LCH group, 8 patients; LAM group, 5 patients). A predominant restrictive pattern was detected only in three patients (12%; LCH group, two patients; LAM group, one patient). Nine patients (36%) walked < 350 m, and 14 of 23 patients (61%) had a significant decrease in arterial oxygen saturation during exercise (> 4 U). The results of DM quantitative study (sum and mean) significantly correlated with FVC (r = - 0.56; p < 0.001), FEV1/vital capacity (r = - 0.94; p < 0.002), midexpiratory phase of forced expiratory flow (r = - 0.84; p < 0.05), FEV1 (r = - 0.82; p < 0.05), and diffusing capacity of the lung for carbon monoxide (r = - 0.82; p < 0.05), bronchial airway resistance (r = 0.79; p < 0.05), and distance walked on the 6-MWT (r = - 0.53; p < 0.05). No significant correlation was found with the results of ABG analysis.

Conclusions: In DCLDs, HRCT scans with quantitative assessment performed by a DM software showed a very good correlation with functional parameters. Therefore, DM could be considered, in combination with a complete functional assessment, in the initial evaluation of patients affected by DCLDs. However, further studies are needed to assess its usefulness in the follow-up of these patients.

Key Words: density mask technique • diffuse cystic lung diseases • high-resolution CT • Langerhans cells histiocytosis • lymphangioleiomyomatosis • 6-minute walking test


   Introduction
TOP
 Abstract
 Introduction
Materials and Methods
 Results
 Discussion
 References
 
Pulmonary lymphangioleiomyomatosis (LAM) and Langerhans cells histiocytosis (LCH) are two different types of diffuse cystic lung diseases (DCLDs) with unknown etiology and origin.1 2 Both are rare, and show similar radiologic features (eg, multiple nodules and cysts in the lungs) and functional impairment.2 3 4 5 The prognosis of patients with these diseases is still unpredictable. Few sensitive and reliable clinical, functional, and radiologic indexes are available to evaluate lung impairment, prognosis, and disease progression.5 6 7 8

In DCLDs, high-resolution CT (HRCT) scanning not only plays a key role in the qualitative description process of all detectable lesions, but is crucial in the diagnosis and monitoring of the progression of these diseases.6 7 9 The HRCT-related density mask (DM) evaluation has been commonly used in the quantitative evaluation of the respiratory impairment in patients affected by chronic obstructive lung diseases (eg, COPD and emphysema). This technique has been used only rarely for the clinical evaluation of patients with LAM and has never been used in the evaluation of patients with LCH.10 11 In the present study, a DM computed program at three different lung levels was used to evaluate the radiologic features of DCLDs (ie, LAM and LCH) and to quantify the air-trapping impairment. The aims of the study were the following: (1) to confirm the importance of the DM in the clinical evaluation of patients affected by LAM, as previously shown in literature7 ; (2) to assess the value of the DM in patients with LCH; and (3) to correlate a quantitative radiologic value with an extensive functional evaluation (pulmonary function tests [PFTs], arterial blood gas [ABG] analysis, and exercise tests).


   Materials and Methods
TOP
 Abstract
 Introduction
 Materials and Methods
Results
 Discussion
 References
 
Study Population
Between September 1999 and April 2001, 25 patients with a diagnosis of DCLDs (LAM, 13 patients; LCH, 12 patients) were consecutively enrolled in the study. The diagnosis of DCLD was based on clinical history, and the results of physical examinations, chest radiographs, HRCT scans, and PFTs. The diagnosis was confirmed by analysis of open lung biopsy specimens in 16 patients (LCH, 7 patients; and LAM, 9). BAL (CD-1a positivity, > 5%) allowed the diagnosis of LCH in four patients. One patient was affected by tuberous sclerosis with pulmonary LAM. A clinical-radiologic diagnosis was accepted only in five patients (LCH, one patient). Of these patients, three refused the videothoracoscopic-assisted biopsy, and the remaining two patients presented with an advanced stage of the disease with critical clinical conditions that did not allow an invasive procedure to be performed at time of our evaluation. Three of four LAM patients with a clinical-radiologic diagnosis showed renal angiomyolipomas on abdominal ultrasonography. One patient developed a lung adenocarcinoma during the advanced stage of LCH disease.

Pulmonary Function
All patients underwent PFTs and gas exchange evaluations. Lung volumes, flow rates, and diffusing capacity of the lung for carbon monoxide (DLCO) were measured in the sitting position using a plethysmographic technique (SensorMedics; Yorba Linda, CA), and corrected for temperature and barometric pressure, according to the American Thoracic Society recommendations.12 Values were expressed as a percentage of predicted normal values, using published standards.13 14 15

When an obstructive pattern was detected, FVC, FEV1, total lung capacity (TLC), and bronchial specific airway resistance (sRaw) were measured before and after the administration of 200 µg salbutamol via metered-dose inhaler. A positive response to therapy with bronchodilators was defined according to the results published by Taveira-DaSilva et al.16 Arterial blood samples were drawn from the radial artery, when patients had been at rest for at least 30 min, breathing room-air (Rapid Lab 348; Bayer; Leverkusen, Germany).

DLCO
The single-breath DLCO was measured according to the American Thoracic Society recommendations and was corrected for hemoglobin concentration.17 18 Results were expressed as a percentage of the predicted values, using the reference values of Crapo and Morris.19

6-Min Walking Test and Arterial Pulse Oximetry
The 6-min walking test (6-MWT) was performed at least 2 h after the PFTs by a trained technician who was unaware of the PFTs results, using a protocol similar to the one described by Sciurba and Slivka.20 Briefly, patients walked on level ground for a distance of 25 m using standardized instructions, including to walk as fast as possible, but not to run, for 6 min, and were gently encouraged using set phrases every 30 s.21 22

Percutaneous arterial saturation was assessed at baseline and during the test by a continuous pulse oximeter (Nellcor; Pleasanton, CA) using a finger or ear sensor. The test was stopped for safety purposes if the arterial oxygen saturation (SaO2 dropped to < 86% and was repeated with oxygen supplementation in patients with resting or exercise SaO2 levels of < 90% in order to determine the need and dosage for supplemental oxygen.23 24

The baseline percentage of SaO2 (ie, SaO2 measured at rest [SaO2-rest]), the minimal exercise SaO2 sustained for 10 s (ie, SaO2 measured after exercise [SaO2-exercise]), and the variation in SaO2 from rest to exercise (SaO2-rest - SaO2-exercise = {Delta}SaO2), reported as the percentage of saturation (1% = 1 U) during the test and the distance walked (in meters), were measured and recorded.

Radiographic Assessment
The first evaluation of all patients consisted of a complete radiologic assessment with digital posteroanterior and laterolateral chest radiograph views, and HRCT scans. For the chest radiograph, a computerized system (Thoravision; Philips; Amsterdam, the Netherlands) was used. Radiographs were performed during breatholding at the end of inspiration.

The HRCT scan was obtained (Tomoscan AV scanner; Philips); at 10-mm intervals using 10-mm collimation during breath-holding at the end of inspiration. A high spatial frequency algorithm was used in order to reconstruct each image. The images were obtained using 140 KV, 175 mA, a field of view of 320°, and a matrix of 512 x 512 pixels.

The quantitative HRCT analysis was performed using the DM software on images from three levels (ie, aortic arch, left lower lobe bronchus origin, and 2 cm above the diaphragm muscle levels) [Fig 1 ], which were obtained at the end of expiration.25 Each image was analyzed manually by drawing a region of interest around both lungs that excluded the trachea and the main bronchus. DM software highlighted pixels with attenuation values of < - 900 Hounsfield units (HU), which were highlighted to identify areas of air-trapping, as previously done by Crausman et al.10 Hard-copy images were photographed at 1,200-HU and - 900-HU window-width levels. The DM software automatically calculated the total area of highlighted pixels for each slice and expressed the percentage of emphysema in both lungs (Fig 2 ). The sum and the mean of the air-trapping percentages at three standardized levels (ie, aortic arch, left lower lobe bronchus origin, and 2 cm from the diaphragmatic muscle) were used for the statistical analysis of the pulmonary functional impairment.



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  		Figure 1. Levels used for the quantitative CT scan evaluation, underlined on a posteroanterior chest radiograph and obtained at end expiration time. Top: aortic arch. Middle: left lower lobe bronchus origin. Bottom: 2 cm above the diaphragm muscle levels.

 


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  		Figure 2. DM evaluation of a CT scan at three selected levels. Left: DM quantitative evaluation of air trapping areas. Right: corresponding image obtained with the HRCT technique.

 
Statistical Methods
Continuous variables were presented as the mean ± SD. Associations among radiologic parameters, pulmonary function, and 6-MWT variables were investigated using the Pearson correlation, partial correlation coefficients, and linear regression analysis. The statistical significance level was set at p < 0.05. A statistical software package (SPSS; SPSS Inc; Chicago, IL) was used for the analysis.


   Results
TOP
 Abstract
 Introduction
 Materials and Methods
 Results
Discussion
 References
 
Demographic and Clinical Parameters
In the LCH group, the male/female ratio was 1:2. All LAM patients were women. The mean ages were 44.3 ± 11.9 years (age range, 27 to 65 years) for the LCH group and 35.3 ± 8.2 years (age range, 23 to 52 years) for the LAM group. The most common symptom was dyspnea on exertion (88%). Other symptoms were cough (60%), chest pain (40%), hemoptysis (28%), asthenia (28%), and fever (4%). A spontaneous or traumatic pneumothorax was the first sign of the underlying disease in almost half of the patients (48%). Pneumothorax was more commonly present in the LAM group (eight patients; 62%) than in the LCH group (four patients; 33%). Six LAM patients (46%) presented with renal angiomyolipomas at time of presentation.

Pulmonary Function Testing and ABG Analysis
Spirometry:
The results of PFTs and ABG analysis are reported in Table 1 . An obstructive pattern was present in 13 patients (52%; LCH group, 8 patients; LAM group, 5 patients). A predominant restrictive pattern was detected only in three patients (LCH group, two patients; LAM group, one patient). Twenty-eight percent of patients (total, seven patients; LCH group, four patients [33%]; LAM group, three patients [23%]) had PFT values within the normal range. Residual volume (RV) was increased in 13 patients (52%; LCH group, 3 patients [25%]; LAM group, 10 patients [77%]). sRaw values were increased in 22 patients (88%; LCH group, 12 patients [100%]; LAM group, 10 patients [77%]). Among the 13 patients with obstruction, only 1 patient, who was affected by a severe form of LAM, had a significant response to inhaled salbutamol, 200 µg. DLCO was reduced in 21 patients (84%) in absolute value (LCH group, 11 patients [92%]; LAM group, 10 patients [77%]). However, DLCO was abnormal in only 11 patients (44%) if related to alveolar volume.


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  		Table 1. PFT Results*

 
ABG Analysis:
Most of the patients had one or more abnormalities in resting ABG. At rest, hypoxemia (ie, PO2 < 70 mm Hg), was present in six patients (26%). One patient showed hypercapnia (ie, PCO2 > 45 mm Hg), and two other patients showed respiratory alkalosis (pH > 7.44).

6-MWT:
Twenty-three patients were able to perform a 6-MWT. The oximetry and the distance covered are summarized in Table 1 . Two patients were too sick to perform the test and were resting in bed. Nine patients (36%) walked < 350 m, and 14 of 23 patients (61%) had significant oxygen desaturation during exercise (SaO2 > 4 U).

Radiology
Chest Radiograph:
The digital radiographic assessment findings were negative in five patients (20%; LCH group, 4 patients [33%]; LAM group, 1 patient [8%]). A reticulonodular alteration was the most common radiologic pattern, which was found in 14 patients (56%). A cystic-bullous pattern was detected in only six subjects (24%).

HRCT:
In the LCH group, CT examination showed a cystic pattern with cysts of > 1 cm in diameter, which were commonly located in the upper and middle fields of the lungs. The cystic lesions were less present in the lower part, leading to a well-detected apex-base negative pattern. A microcyst pattern was present in only three patients (25%). Nodules of < 1 cm in diameter were visible in 80% of LCH patients at the time of evaluation. In the LAM group, HRCT scanning demonstrated a more diffuse lung involvement compared to that in the LCH group (no apex-base gradient in cysts distribution). Only three patients (23%) had nodules present on the scans.

Quantitative Chest CT Scan:
The quantitative DM evaluation at three different levels is shown in Table 2 . The quantitative DM index was significantly higher in the LAM group compared with the LCH group. Significant differences between the amount of abnormal lung in the third level was registered (p = 0.02), suggesting an apex-base gradient distribution of the thin-walled cysts throughout the lung (Table 2) .


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  		Table 2. DM Analysis of DCLD: Apex-Base Gradient Between LCH and LAM*

 
Statistical Results
The results are summarized in Table 3 . Using Pearson analysis, a significant statistical correlation was found between the DM values (sum and mean) and FVC (r = - 0.56; p < 0.001), FEV1 (r = - 0.82; p < 0.02), FEV1/vital capacity (VC) ratio (r = - 0.94; p < 0.002), midexpiratory phase of forced expiratory flow (FEF25–75) [r = - 0.84; p < 0.02], RV (r = 0.82; p < 0.008), DLCO (r = - 0.83; p = 0.04), and sRaw (r = 0.79; p = 0.02) [Fig 3 , top left, A, to bottom left, E]. Although it did not reach statistical significance, our data showed a correlation between ABG values and DM score. Among the data obtained while performing 6-MWT, the distance covered (in meters) while breathing room air was significantly correlated with DM score (r = - 0.54; p = 0.01) [Fig 3 , bottom right, F].


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  		Table 3. Statistical Correlations Among PFT, Gas Exchange, 6-MWT, and DM Quantitative Score*

 


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  		Figure 3. Scatterplot graphs of the correlation between the sum of DM values and important functional and exercise parameters. Top left, A: FEV1. Top right, B: FEF25–75. Middle left, C: FEV1/VC. Middle right, D: DLCO.Bottom left, E: RV. Bottom right, F: meters walked (MW).

 

   Discussion
TOP
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
References
 
LAM and LCH are both rare diseases characterized by cystic-aerial lung alterations, with reticular, nodular, reticulonodular, and honeycombing radiographic aspects combined in varying degrees. Both diseases show small cystic lesions on HRCT scans and may have a similar clinical presentation, with progressive dyspnea, hemoptysis, pneumothorax, and a variable course usually culminating in respiratory failure.

Alterations of the respiratory function in patients affected by DCLDs vary according to the stage and extension of the disease.26 27 28 29 In the initial phase, the only significant alteration is often a decrease in CO transfer factor (ie, DLCO), which is attributed more to the thickening of the alveolocapillary membrane, to the vascular involvement, and to the subsequent alteration of the ventilation-perfusion ratio rather than to the destruction of the alveolocapillary membrane.30 For these reasons, a restrictive pattern is more common in the initial phase than an obstructive pattern, which is present in more advanced stages of LCH and LAM.26 27 28

Exercise tests (eg, maximum oxygen uptake study and 6-MWT) are often used to quantify the cardiorespiratory impairment in a large cohort of patients. They are useful in discovering a latent state of cardiorespiratory impairment and are used in the literature to evaluate the functional impairment of LAM patients.10 28 We used the 6-MWT in all patients enrolled, evaluating the distance covered and the desaturation degree during exercise. Its usefulness is underlined by the higher percentage of hypoxemia found in patients after comparing results obtained after exercise with those obtained at rest (60% vs 24%, respectively).

To date, only a few studies published in literature evaluated the importance of PFTs in the prognosis of DCLDs, and the correlation between the severity of radiologic and clinical findings with pulmonary dysfunction (ie, FVC, FEV1, and TLC) rarely has been described.4 28

HRCT scanning has been demonstrated to be an important test for the initial evaluation and prognosis of patients affected by interstitial lung diseases. Several authors performed different qualitative and quantitative CT measurements of the extent of lung impairment in patients with LAM, correlating them with PFT results.6 7 10 11 However, this technique has never been used to evaluate the clinical status of patients affected by LCH.

Quantitative thin-section CT scans have been performed in a cohort of LAM subjects by Crausman et al,10 underlining the good correlation between the quantitative CT index and maximum oxygen uptake (r = - 0.79; p = 0.01), FEV1/FVC (r = - 0.92; p = 0.002), and FEV1 (r = - 0.81; p = 0.02). Such a relationship between radiologic appearance and pulmonary function abnormality is of particular importance, due to the recognized prognostic value of the FEV1/FVC ratio and TLC in predicting mortality rates between 2 and 5 years, as reported by Kitaichi et al29 in his clinicopathologic study of 46 patients with LAM.28 Recently, Avila et al11 correlated qualitative and quantitative CT scan findings with pulmonary dysfunction over a wide range of disease severity in patients with LAM, using a 1.0-mm-thick section at 3-cm intervals. The absence or presence of pleurodesis had no significant effect on the relationship between the ratio of normal lung volume to total lung volume and FEV1.11 Moreover, quantitative CT scans showed a good reproducibility and significant correlation among FEV1, DLCO, and the percent predicted ratio of RV to TLC.11

In our study, we confirmed the data reported in the articles by Crausman et al28 and Avila et al7 11 with only three DM reconstruction images at predefined levels. The correlation between the functional parameters and DM also was found in LCH patients, showing its importance in both DCLD types. Moreover, we demonstrated a high correlation of the DM score not only with FEV1 and DLCO, but also with other important functional parameters (ie, FEF25–75, sRaw, and RV). The correlation of the DM calculated air-trapping areas with sRaw values in both diseases is crucial, showing that the bronchial impairment plays a fundamental role in the functional impairment and the radiologic appearance of DCLDs. Moreover, we described the importance of the 6-MWT in the initial evaluation of DCLDs, which is the result of the relationship between the calculated distance covered and the DM score.

LAM and LCH, even if different in terms of pathogenesis, risk factors, and prognosis, showed a similar functional impairment that correlated strongly with the amount of destroyed lung. This value may be calculated easily with the DM score, thus expressing the illness severity with a numeric score. Therefore, due to the statistical correlation with pathophysiologic parameters, DM could be used in combination with a complete functional assessment in the initial evaluation of patients affected by DCLDs.


   Footnotes
 
Abbreviations: ABG = arterial blood gas; DCLD = diffuse cystic lung disease; DLCO = diffusing capacity of the lung for carbon monoxide; DM = density mask; FEF25–75 = midexpiratory phase of forced expiratory flow; HRCT = high-resolution CT; HU = Hounsfield units; LAM = lymphangioleiomyomatosis; LCH = Langerhans cells histiocytosis; PFT = pulmonary function test; RV = residual volume; SaO2 = arterial oxygen saturation; SaO2-exercise = arterial oxygen saturation measured after exercise; SaO2-rest = arterial oxygen saturation measured at rest; 6-MWT = 6-min walking test; sRaw = specific airway resistance; TLC = total lung capacity

Received for publication December 11, 2002. Accepted for publication August 8, 2003.


   References
TOP
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 References
 

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