PURPOSE: The aim of this study is to characterize the demographic, clinical, physiologic, and radiological features of subjects with Lymphangioleiomyomatosis (LAM) in Israel.
METHODS: Retrospective clinical review of clinical data concerning patients that were diagnosed with LAM in Israel.
RESULTS: Fourteen women were diagnosed with LAM. One of them had tuberous sclerosis and the other had sporadic LAM. Their average age was 45.3, four of them were correctly diagnosed, in 10 patients there was a delay of 7.5 years from the time symptoms began until they were diagnosed. The most common misdiagnoses were asthma and anxiety. Four of the patients were past smokers. Eight women had children (2–4), ten patients were treated with different estrogen supplements with an average of 11.9±6.6 years. In 6 patients the presenting symptom of the disease was dyspnea, in 5-pneumothorax, and in 1-chylothorax. Nine patients had pneumothorax, with an average of 2.5±1.7 recurrences, 12 patients had dyspnea with an average MMRC score of 3±1, six of 14 patients had chylothorax, and one patient is asymptomatic. Eight patients were diagnosed with CT only, 1 patient underwent also transbronchial biopsy, two underwent open lung biopsies and two had another biopsy. Six patients had abdominal angiomyolipoma. Their average FEV1 is 49.4%. Four patients are being treated with long acting beta agonists, 5 underwent pleurodesis. One patient underwent right parietal pleurectomy and then left single lung transplantation with recurrence of chylothorax on the transplanted side that necessitated pleurodesis. Two patients died; one 7 months after lung transplantation and one while waiting on the candidate list.
CONCLUSION: Lymphangioleiomyomatosis (LAM) is a rare progressive disease, often unrecognized and misdiagnosed. The estimated prevalence of LAM in Israel is 2 per million.
CLINICAL IMPLICATIONS: In 2007 the Israeli LAM organization was founded in order to create better collaboration among Israeli patients and with between them and international institutes. Compared to the NHLBI LAM registry chylothorax is more frequent in Israeli patients (42.9% chylothorax vs 20.9 pleural effusion p=0.045).
DISCLOSURE: Anat Amital, No Financial Disclosure Information; No Product/Research Disclosure Information