TY - JOUR T1 - Lymphangioleiomyomatosis – experience of a university hospital JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2021.PA2371 VL - 58 IS - suppl 65 SP - PA2371 AU - Mafalda Martins Baptista AU - Natália Melo AU - Patrícia Mota AU - Helder Novais E Bastos AU - Susana Guimarães AU - Conceição Souto-Moura AU - André Carvalho AU - José Miguel Jesus AU - Rui Cunha AU - António Morais Y1 - 2021/09/05 UR - http://erj.ersjournals.com/content/58/suppl_65/PA2371.abstract N2 - Introduction: Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that occurs sporadically (S-LAM) or linked to tuberous sclerosis (TSC-LAM), mainly in women at reproductive age. Diagnosis is supported by extrapulmonary features, VEGF-D or histology.Aim: To characterize and analyze approach of LAM.Methods: Retrospective study from an Interstitial Lung Diseases’ unit at a University Hospital, between 2001 and 2021.Results: 18 women were identified, 10 with S-LAM and 8 with TSC-LAM, mainly non-smokers (89%). Follow-up varied between 1 and 15 years. Most reported symptoms were dyspnea (39%) and cough (33%). Pneumothorax occurred in 22% and chylothorax in 6%.Mean age was higher in S-LAM group (55 versus 34 years-old, p 0,001), including 5 postmenopausal women, 2 with previous hormonal therapy. S-LAM was established by extrapulmonary features (30%), positive VEGF-D (20%), VATS biopsy (20%) and by multidisciplinary meeting in the remaining ones.All patients with TSC-LAM presented with renal angiomyolipomas, against only 1 with S-LAM (p<0,001). Other TSC features included neurologic (63%), cutaneous (50%), hepatic (38%) and bone (38%) involvement.44% had an obstructive pattern (mean FEV1 69% predicted) and low DLCO. Average annual decline was 1.4% in FEV1 and 2.4% in DLCO. 22% had hypoxemia and 22% had desaturation in 6MWT.61% received mTOR inhibitors, mainly Sirolimus, but 3 started Everolimus due to renal involvement.2 patients with S-LAM died, 1 of them few months after lung transplant.Conclusion: TSC-LAM patients were younger and had more extrapulmonary features, but mortality was higher in S-LAM group. Almost half the patients had lung function impairment, showing high morbidity of LAM.FootnotesCite this article as: European Respiratory Journal 2021; 58: Suppl. 65, PA2371.This abstract was presented at the 2021 ERS International Congress, in session “Prediction of exacerbations in patients with COPD”.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only). ER -