Introduction Lymphangioleiomyomatosis(LAM)isararedis- ease that mainly affects women of reproductive age, thatischaracterisedbyproliferationofatypicalmuscle cells (LAM cells) around the airways, blood vessels and lymphatic vessels, which can result in cystic de- structionofthelungsandpulmonaryfunctionimpair- ment.LAMmayoccurinsporadicformorinassocia- tionwithtuberoussclerosiscomplex(TSC)(1,2). LAM is clinically characterised by recurrent spontaneous pneumothorax, progressive dyspnoea, haemoptysis, and chylothorax (1-5). Extrapul- monary manifestations include renal angiomyolipo- mas, abdominal and pelvic masses along the axial lymphatics (lymphangiomyomas), and chylous as- cites (1, 2-4). The most common abnormalities foundinpulmonaryfunctiontests(PFTs)includean obstructive pattern, air trapping, and a reduction in the diffusion capacity of the lungs for carbon monoxide(DL CO )(4,6-8). Clinical course and characterisation of lymphangioleiomyomatosis in a Brazilian reference centre B.GuedesBaldi,C.SalimG.Freitas,M.SponholzAraujo,O.MeiraDias,D.A.SilvaPereira,S. PinheiroPimenta,R.A.Kairalla,C.R.RibeiroCarvalho PulmonaryDivision,HeartInstitute(InCor),HospitaldasClínicas,UniversityofSaoPauloMedicalSchool,SaoPaulo,Brazil Abstract. Background and objective: Lymphangioleiomyomatosis(LAM)isararediseasethatpromotespulmonary cysticdestructionandimpairspulmonaryfunction.WeaimtodescribefeaturesandclinicalcourseofLAMpatients from Brazil. Methods: We described the clinical and functional features, performance in six minute walk test (6MWT),managementdetails,survivalandclinicalcourseof84LAMpatientsfollowedinaBrazilianreferencecen- tre. Results: Allsubjectswerewomen,theaverageageatonsetofsymptomswas38years,andtheaverageatdiagno- siswas42years.Themajorsymptomsduringthecourseofthediseaseweredyspnoeaandpneumothorax.Thepa- tientsexperiencedimpairedqualityoflife,withworsescoresinthephysicalandemotionaldomains.Themostcom- monabnormalitiesinpulmonaryfunctiontestswereanobstructivepatternandreduceddiffusioncapacity,whereas aquarterofthepatientshadnormalspirometricresults.Inthe6MWT,althoughpatientshadpreservedexerciseca- pacity,morethanhalfofthepatientshadsignificantdesaturation.Hormonalblockageanddoxycyclinewerethemost commontreatmentmodalitiesemployedinourpatients.Thesurvivalprobabilityfromdiagnosiswas90%at5years, whereasthemeanannualrateofdeclineinFEV1was60±78mL. Conclusions: Clinicalandfunctionalfeaturesof theLAMpatientsfromourcentrearesimilartothosefromothercountries.Oursampleshowedpreservedexercise capacity,withdesaturationinthe6MWT,andimpairedqualityoflife.Survivalwassimilar,whereastheannualrate ofdeclineofFEV1wasslightlylowerthaninrecentstudies. (Sarcoidosis Vasc Diffuse Lung Dis 2014; 31: 129-135) Key words: Angiomyolipoma;Brazil;lymphangioleiomyomatosis;Respiratoryfunctiontests;Survival Received:15August2013 Correspondence:BrunoGuedesBaldi,M.D., Dr.EnéasdeCarvalho AguiarAvenue,44,Fifthfloor,PostalCode05403-900, SãoPaulo,Brazil. Telephone:55112661-5695;Fax:55112661-5695 E-mail:bruno.guedes2@terra.com.br Original article: Clinical Research SARCOIDOSISVASCULITISANDDIFFUSELUNGDISEASES2014;31129-135 ©Mattioli1885