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Long-term survival in a patient with pulmonary lymphangioleiomyomatosis
JK Reid | H Rees | D Cockcroft
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.
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