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A nationwide survey of lymphangioleiomyomatosis in Korea: recent increase in newly diagnosed patients.

Park HY, Nam HS, Chung MP, Jeong SH, Kim YJ, Cha SI, Kim YW, Park JS, Uh ST, Park CS, Park MS, Moon JA, Jung KS, Jegal YJ, Kim DS, Song JW, Yum HK, Park YB - J. Korean Med. Sci. (2010)

Bottom Line: The higher proportion of patients diagnosed after 2004 showed the normal chest radiography, and they were detected by routine chest CT screening (P=0.016).This study showed that clinical features of Korean patients with LAM were not different from those reported elsewhere.It is concluded that the reason for the increase of newly diagnosed patients is the result of increase in detection of the early stage LAM by the widespread use of chest CT screening.

View Article: PubMed Central - PubMed

Affiliation: Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

ABSTRACT
In 2007, the Korean Interstitial Lung Disease Society had collected clinical data of patients who have diagnosed as Lymphangioleiomyomatosis (LAM) since 1990 through nationwide survey, which showed that LAM patients had increased sharply after 2004. The present study was performed to show the clinical features of Korean patients with LAM, and to establish the reason for the recent increase in the diagnosis. All 63 patients were women and the mean age at diagnosis was 36 yr. The most common presenting symptom was dyspnea and 8 patients had tuberous sclerosis complex. The survival rate at 5 yr after diagnosis was 84%. Compared with patients diagnosed after 2004 (n=34), the patients diagnosed before 2004 (n=29) complained with dyspnea more (P=0.016) and had lower FEV(1)% predicted (P=0.003), and DLco% predicted (P=0.042). The higher proportion of patients diagnosed after 2004 showed the normal chest radiography, and they were detected by routine chest CT screening (P=0.016). This study showed that clinical features of Korean patients with LAM were not different from those reported elsewhere. It is concluded that the reason for the increase of newly diagnosed patients is the result of increase in detection of the early stage LAM by the widespread use of chest CT screening.

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Radiographic features of a 46-yr old woman with LAM. (A) Chest radiography of 46-yr old woman with LAM. Chest radiography discloses no clue to the cystic lung disease. (B) High resolution CT scan of 46-yr old woman with LAM. High resolution CT scan at the level of lower lobar bronchus shows innumerable ovoid or round cystic lesions (arrows) in both lungs. The walls of these cysts are thin and even in thickness, which were considered as typical morphologic features of LAM.
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Figure 3: Radiographic features of a 46-yr old woman with LAM. (A) Chest radiography of 46-yr old woman with LAM. Chest radiography discloses no clue to the cystic lung disease. (B) High resolution CT scan of 46-yr old woman with LAM. High resolution CT scan at the level of lower lobar bronchus shows innumerable ovoid or round cystic lesions (arrows) in both lungs. The walls of these cysts are thin and even in thickness, which were considered as typical morphologic features of LAM.

Mentions: Nineteen patients were not available for pulmonary function tests because of dyspnea or pneumothorax at initial diagnosis, but the overall pulmonary function was better when compared to that in previous studies (2, , 13). In patients newly diagnosed with LAM after 2004, FEV1 predicted, FEV1/FVC, and DLco were significantly higher than those in patients diagnosed before 2004. Declines in FEV1 and the FEV1/FVC ratio, which represent progressive obstructive defects, and decreased DLco are sensitive factors for disease progression in LAM (8). Therefore, we suggest that patients diagnosed after 2004 were detected with mild lung impairment, which may mean that it was at an earlier stage compared to those diagnosed before 2004. This was consistent with the finding that clinical symptoms were significantly better, and normal radiographic findings were more frequent in recently diagnosed patients. This earlier detection of LAM in patients with mild symptoms and normal chest radiography might be explained by the widespread and liberal use of CT scanning, which is superior to chest radiography for the assessment of patients with LAM (14). In our study, among 10 patients with normal chest radiographic findings, seven were detected by CT screening (Fig. 3) and three without respiratory symptoms were diagnosed coincidentally for LAM, after undergoing CT scanning for breast cancer, thyroid cancer, or abdominal pain.


A nationwide survey of lymphangioleiomyomatosis in Korea: recent increase in newly diagnosed patients.

Park HY, Nam HS, Chung MP, Jeong SH, Kim YJ, Cha SI, Kim YW, Park JS, Uh ST, Park CS, Park MS, Moon JA, Jung KS, Jegal YJ, Kim DS, Song JW, Yum HK, Park YB - J. Korean Med. Sci. (2010)

Radiographic features of a 46-yr old woman with LAM. (A) Chest radiography of 46-yr old woman with LAM. Chest radiography discloses no clue to the cystic lung disease. (B) High resolution CT scan of 46-yr old woman with LAM. High resolution CT scan at the level of lower lobar bronchus shows innumerable ovoid or round cystic lesions (arrows) in both lungs. The walls of these cysts are thin and even in thickness, which were considered as typical morphologic features of LAM.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2908788&req=5

Figure 3: Radiographic features of a 46-yr old woman with LAM. (A) Chest radiography of 46-yr old woman with LAM. Chest radiography discloses no clue to the cystic lung disease. (B) High resolution CT scan of 46-yr old woman with LAM. High resolution CT scan at the level of lower lobar bronchus shows innumerable ovoid or round cystic lesions (arrows) in both lungs. The walls of these cysts are thin and even in thickness, which were considered as typical morphologic features of LAM.
Mentions: Nineteen patients were not available for pulmonary function tests because of dyspnea or pneumothorax at initial diagnosis, but the overall pulmonary function was better when compared to that in previous studies (2, , 13). In patients newly diagnosed with LAM after 2004, FEV1 predicted, FEV1/FVC, and DLco were significantly higher than those in patients diagnosed before 2004. Declines in FEV1 and the FEV1/FVC ratio, which represent progressive obstructive defects, and decreased DLco are sensitive factors for disease progression in LAM (8). Therefore, we suggest that patients diagnosed after 2004 were detected with mild lung impairment, which may mean that it was at an earlier stage compared to those diagnosed before 2004. This was consistent with the finding that clinical symptoms were significantly better, and normal radiographic findings were more frequent in recently diagnosed patients. This earlier detection of LAM in patients with mild symptoms and normal chest radiography might be explained by the widespread and liberal use of CT scanning, which is superior to chest radiography for the assessment of patients with LAM (14). In our study, among 10 patients with normal chest radiographic findings, seven were detected by CT screening (Fig. 3) and three without respiratory symptoms were diagnosed coincidentally for LAM, after undergoing CT scanning for breast cancer, thyroid cancer, or abdominal pain.

Bottom Line: The higher proportion of patients diagnosed after 2004 showed the normal chest radiography, and they were detected by routine chest CT screening (P=0.016).This study showed that clinical features of Korean patients with LAM were not different from those reported elsewhere.It is concluded that the reason for the increase of newly diagnosed patients is the result of increase in detection of the early stage LAM by the widespread use of chest CT screening.

View Article: PubMed Central - PubMed

Affiliation: Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

ABSTRACT
In 2007, the Korean Interstitial Lung Disease Society had collected clinical data of patients who have diagnosed as Lymphangioleiomyomatosis (LAM) since 1990 through nationwide survey, which showed that LAM patients had increased sharply after 2004. The present study was performed to show the clinical features of Korean patients with LAM, and to establish the reason for the recent increase in the diagnosis. All 63 patients were women and the mean age at diagnosis was 36 yr. The most common presenting symptom was dyspnea and 8 patients had tuberous sclerosis complex. The survival rate at 5 yr after diagnosis was 84%. Compared with patients diagnosed after 2004 (n=34), the patients diagnosed before 2004 (n=29) complained with dyspnea more (P=0.016) and had lower FEV(1)% predicted (P=0.003), and DLco% predicted (P=0.042). The higher proportion of patients diagnosed after 2004 showed the normal chest radiography, and they were detected by routine chest CT screening (P=0.016). This study showed that clinical features of Korean patients with LAM were not different from those reported elsewhere. It is concluded that the reason for the increase of newly diagnosed patients is the result of increase in detection of the early stage LAM by the widespread use of chest CT screening.

Show MeSH
Related in: MedlinePlus