The diagnostic approach, clinical evolution, and treatment of a
patient with primary pulmonary lymphangioleiomyomatosis are reported.
This patient presented a restrictive respiratory syndrome resistant to
conventional glucocorticoid therapy. The diagnosis, based on clinical
and histologic examinations, was confirmed by immunohistochemical
localization of one of the desmins, the smooth muscle cell actin, and
HMB45 antigen. The patient received treatment with an anti-estrogenic
agent (tamoxifen citrate) and high doses of medroxyprogesterone
acetate, an antigonadotropic progestin. Respiratory function improved
rapidly with clinical relief.
Abbreviation: LLM = lymphangioleiomyomatosis