Long term survival in a patient with pulmonary lymphangioleiomyomatosis.
A patient with pulmonary lymphangioleiomyomatosis was diagnosed more than 22 years after the onset of symptoms by a thoracoscopic lung biopsy, after a high resolution computerized tomogram of the chest was highly suggestive of the disease. After nearly 30 years since the onset of her symptoms, the patient leads a relatively normal life with only mildly abnormal lung function and has minimal reduction in her exercise tolerance. There have been few reports of patients surviving for such a long time after the onset of this disease; the literature suggests that most patients die within 15 years of symptom onset.
Reid
John K
JK
Department of Medicine, Royal University Hospital, University of Saskatchewan, Saskatoon, Canada.
Rees
Henrike
H
Cockcroft
Donald
D
D017079:Exercise Tolerance; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D018192:Lymphangioleiomyomatosis / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D008875:Middle Aged; D011379:Prognosis; D012129:Respiratory Function Tests; D013997:Time Factors