Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ORIGINAL ARTICLES
Clinical Features of Lymphangioleiomyomatosis Complicated by Renal Angiomyolipomas
Yoshiko MizushinaMasashi BandoTatsuya HosonoNaoko MatoTakakiyo NakayaYoshikazu IshiiHideaki YamasawaYukihiko Sugiyama
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JOURNALS OPEN ACCESS

2011 Volume 50 Issue 4 Pages 285-289

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Abstract

Objective Renal angiomyolipomas (R-AMLs) are major complications of lymphangioleiomyomatosis (LAM). The objective of this study was to better understand the influence of R-AMLs in patients with LAM on the prognosis and other clinical factors related to respiration, and to investigate the management of R-AMLs in patients with LAM.
Patients and Methods We retrospectively investigated the clinical features of 7 patients with LAM [4 were TSC (Tuberous sclerosis complex)-LAM and 3 were S (sporadic)-LAM] complicated by R-AMLs admitted to our hospital from 1997 to 2008.
Results All patients were females and the mean age at diagnosis of LAM was 40.7 years (31.7 years for TSC-LAM and 52.7 years for S-LAM). Although 5 patients had symptoms related to R-AMLs, only 1 patient experienced symptoms related to R-AMLs at the time of diagnosis. Five patients had bilateral and 2 patients had unilateral R-AMLs. R-AMLs ruptured in 4 cases (3 patients were TSC-LAM) including 2 patients in whom they ruptured bilaterally, and who underwent bilateral nephrectomy. In 1 case, unilateral R-AMLs grew larger and appeared on the other side during the follow-up period.
Conclusion Although only rare cases of LAM show symptoms related to R-AMLs initially, R-AMLs are a notable complication. To avoid nephrectomy, R-AMLs should be diagnosed when they are small and should be followed up carefully by periodic echograms or CT scans.

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© 2011 by The Japanese Society of Internal Medicine
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