Lymphangioleiomyomatosis (LAM) is a rare disease affecting women, classically involving the lungs. However, extrapulmonary manifestations also occur, including renal angiomyolipomas, retroperitoneal lymphangioleiomyomas and extrathoracic lymphadenopathy. The lung disease is hormone-responsive, but no information exists regarding sex hormone responsiveness of abdominal LAM. Here, we report two women with LAM whose abdominal lymphangioleiomyomas increased in size with hormonal changes of the menstrual cycle. This is the first description of abdominal lymphangioleiomyomas exhibiting hormone responsiveness in LAM. We describe these cases and summarize the literature on abdominal LAM. Menstrual cycle variation should be taken into account when assessing response to therapy, both clinically and in research studies.
The present article reports the case of a 22-yr-old female with new onset Crohn's colitis, anterior uveitis and multiple pulmonary nodules which, on histological examination, were necrobiotic nodules. This is a rare but recognised pulmonary extraintestinal manifestation of Crohn's disease and only the fourth reported case. The present case report is followed by a brief review of the relevant literature.