Usædvanlig sen debut af lymfangioleiomyomatose

The rare, severe lung disease lymphangioleiomyomatosis (LAM) usually presents with dyspnoea on exertion, cough and expectoration in females in the third decade and is worsened by pregnancy or hormone therapy. Lung transplant is often needed within ten years since no specific treatment is available. The patient in this case report was a mother of two, who had never received hormone therapy. Once, when she was 47-years-old, she had had a period of dyspnoea and pleural empyema and no diagnosis was made, but being 75-years-old she was admitted with moderate dyspnoea on exertion, and high resolution CT showed cysts of the lungs classical for LAM.