To the Editors:
In the paper by Johnson 1 on lymphangioleiomyomatosis (LAM), he describes the typical patient being female and of child-bearing age. Although patients outside this age range have been described, the oldest recorded in the recently reported National Heart, Lung and Blood Institute registry was aged 76 yrs, the mean age of onset being 38.9 yrs 2.
We wish to report a case of LAM in an 86 yr-old-female who presented with acute breathlessness on a background of increasing dyspnoea over a 3-yr period. She had been told that she had chronic obstructive pulmonary disease 2 yrs years prior to this presentation, despite having never smoked. At the time of admission she was taking regular nebulised salbutamol but no other respiratory medications. She had previously undergone resective surgery for breast carcinoma and was on continuing treatment with anastrazole. Several years previously, she had also undergone a hysterectomy and bilateral oopherectomy, but was unclear as to why. There was no other significant medical history. On examination, she was comfortable at rest, but tachypnoeic and hypoxic on minimal exertion (oxygen saturation of 88% on room air). Apart from hyperinflation, the rest of the chest examination was unremarkable. Blood investigations including α1-antitrypsin assays were within normal limits. A computed tomography pulmonary angiogram was arranged as there was concern that she had suffered a pulmonary embolus. No emboli were noted, however, diffuse bilateral thin-walled cysts were identified. Two chest radiologists each reviewed the scans independently and concluded that these appearances were most consistent with the diagnosis of LAM (fig. 1⇓). We speculate that the patient's previous oophorectomy and use of the aromatase inhibitor anastrazole had ameliorated the condition, which led to its late presentation. Although a rare condition, we suggest LAM be considered in the differential diagnosis of dyspnoea in elderly females who demonstrate atypical features of airflow limitation in their presentation.
Computed tomography scan of the chest revealing multiple thin-walled cysts, typical of lymphangioleiomyomatosis.
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