J Korean Med Sci.  2010 Aug;25(8):1182-1186. 10.3346/jkms.2010.25.8.1182.

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

• KMID: 1714040
• DOI: http://doi.org/10.3346/jkms.2010.25.8.1182

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 FEV1% 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.

Keyword

Lymphangioleiomyomatosis; Korea; Registries; Respiratory Function Tests

MeSH Terms

Adult
Aged
Early Diagnosis
Female
Humans
Lung Neoplasms/*diagnosis/mortality/radiography
Lymphangioleiomyomatosis/*diagnosis/mortality/radiography
Middle Aged
Republic of Korea
Respiratory Function Tests
Survival Rate
Tomography, X-Ray Computed

Figure

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  Fig. 1 Annual number of new patients diagnosed with LAM.

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  Fig. 2 Kaplan-Meier survival analysis of mortality of 63 patients with LAM. The probability of being alive was 84% at 5 yr and 65% at 8.5 yr after diagnosis.

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  Fig. 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.

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