Successful Treatment of Pulmonary Lymphangioleiomyomatosis With Progestins

Denoo, Xavier; Hermans, Ginette; Degives, Raoul; Foidart, Jean‐Michel

doi:10.1378/chest.115.1.276

Cited by 15 publications

(6 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Reports of success, based on few patients, or without objective data, are matched by reports [25][26][27][28][29][30][31][32][33][34][35] of therapeutic failures. In one study 36 of premenopausal patients, the yearly decline in Dlco was less in 16 patients treated with progesterone than in 13 untreated patients.…”

Section: Discussionmentioning

confidence: 99%

Decline in Lung Function in Patients With Lymphangioleiomyomatosis Treated With or Without Progesterone

Taveira-DaSilva

Stylianou

Hedin

et al. 2004

Chest

211

185

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 99%

Decline in Lung Function in Patients With Lymphangioleiomyomatosis Treated With or Without Progesterone

Taveira-DaSilva

Stylianou

Hedin

et al. 2004

Chest

211

185

View full text Add to dashboard Cite

“…Oopherectomy, medroxyprogesterone, and tamoxifen use have been advocated, but therapeutic benefit is unclear. [98][99][100] Lung transplantation is reserved for patients with end-stage LAM. 101,102 Cardiovascular manifestations are often the earliest diagnostic finding in patients with TSC.…”

Section: Other Manifestations Of Tscmentioning

confidence: 99%

Tuberous sclerosis complex: Advances in diagnosis, genetics, and management

Schwartz¹,

Fernández²,

Kotulska³

et al. 2007

Journal of the American Academy of Dermatology

345

304

View full text Add to dashboard Cite

“…Até à data não existe nenhum tratamento eficaz para a LAM ou ET, à excepção do transplante pulmonar na fase terminal da doença. Devido à maior prevalência da LAM na mulher em idade reprodutiva, pensa -se que os estrogénios possam ter um papel na progressão da doença, pelo que alguns doentes recebem tratamento hormonal (progesterona), apesar de não ter sido demonstrado de forma conclusiva o seu benefício 13,14 . Novas terapêuticas, como a rapamicina, apresentam já os primeiros resultados positivos.…”

Section: Discussionunclassified

Esclerose tuberosa com envolvimento pulmonar

Ferreira¹,

Nogueira²,

Ferreira

et al. 2010

Revista Portuguesa de Pneumologia (English Edition)

View full text Add to dashboard Cite

ResumoA esclerose tuberosa (ET) é uma doença rara, esporádica ou transmitida de forma autossómica dominante. Caracteriza -se pela tríade convulsões, atraso mental e angiofibromas faciais. O envolvimento pulmonar é raro e, quando ocorre, é mais frequente no sexo feminino. Os autores apresentam o caso de uma doente de 52 anos, não fumadora, com antecedentes conhecidos de epilepsia na infância e angiomiolipomas renais. Assintomática e sem alterações ao exame objectivo. Em tomografia do tórax realizada para avaliação da doença, foram detectadas formações microquísticas dispersas em ambos os campos pulmonares. Exame funcional respiratório normal. A ressonância magnética cerebral evidenciou tuberosidades corticais e nódulos subependimários calcificados. Concluiu -se AbstractTuberous sclerosis (TS) is a rare, sporadic or autosomal dominant disease characterized by the triad of seizures, mental retardation and angiofibromas. Lungs are rarely involved in TS, and pulmonary involvement is almost always found in females. We report the case of a 52 year -old female, nonsmoker, with a history of seizures in childhood and renal angiomyolipomas. She presented no complaints and her physical exam was normal. Chest CT performed for the evaluation of the disease detected thin-walled pulmonary cysts in both lungs. Lung function tests were normal. Cortical tubers and calcified subependymal nodules were seen in cerebral magnetic resonance. Tuberous sclerosis was diagnosed (lymphangioleiomyomatosis, cortical tubers, calcified subependymal nodules and angiomyio- Esclerose tuberosa com envolvimento pulmonar Tuberous sclerosis with pulmonary involvmentRecebido para publicação/

show abstract

Successful Treatment of Pulmonary Lymphangioleiomyomatosis With Progestins

Cited by 15 publications

References 18 publications

Decline in Lung Function in Patients With Lymphangioleiomyomatosis Treated With or Without Progesterone

Decline in Lung Function in Patients With Lymphangioleiomyomatosis Treated With or Without Progesterone

Tuberous sclerosis complex: Advances in diagnosis, genetics, and management

Esclerose tuberosa com envolvimento pulmonar

Contact Info

Product

Resources

About