Limfangioleiomiomatoza (LAM) : trudności diagnostyczne

Bibliogr. s. 77

Background: LAM is a rare idiopathic disorder found almost exclusively in premenopausal women. It is characterized by a proliferation of abnormal smooth muscle cell in the pulmonary interstitium, in the lymphatic system of the thorax, retroperitoneum and by the formation of pulmonary parenchymal cysts. The most common clinical manifestations of LAM are pulmonary symptoms including progressive dyspnoea, pneumothorax and chylous effusion. Case report: The authors describe a case of a 43-year-old woman with lymphangiomyomatosis (LAM) with initial manifestation of pneumothorax and retroperitoneal cystic mass. Conclusions: HRCT is the best method for cyst detection and is essential for diagnosis. A radiologist is often the first physician to suggest the diagnosis of LAM. However, misdiagnosis is common and may result in inappropriate therapeutic procedures that can further complicate the treatment. There are characteristic abdominal findings in patients with LAM, that in conjunction with the HRCT findings of pulmonary cysts, prove to be useful in establishing this diagnosis.