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

Taveira-DaSilva, Angelo M.; Stylianou, Mario; Hedin, Carolyn J.; Hathaway, Olanda; Moss, Joel

doi:10.1378/chest.126.6.1867

Cited by 211 publications

(222 citation statements)

References 44 publications

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“…(9) In another study, in which 275 patients treated with progesterone were evaluated, the mean annual decline in FEV 1 was 75 mL (1.7% of predicted), whereas, in a nonrandomized prospective study of 10 patients treated with triptorelin, there was a mean annual decline of 156 mL (4.9% of predicted). (12,14) In the present study, there was a mean annual increase in FEV 1 of 80 mL (2.5% of predicted) in LAM patients treated with goserelin. However, this difference was not significant, probably because of the small size of the patient sample, which is a wellrecognized limitation of studies evaluating rare diseases.…”

supporting

confidence: 48%

“…Oophorectomy, as well as the administration of tamoxifen, progesterone, gonadotropinreleasing hormone (GnRH) agonists, and GnRH analogues, has been tested, producing controversial results, because there have been no randomized clinical trials of these treatment modalities. (9)(10)(11)(12)(13)(14) In a retrospective study of 35 LAM patients receiving various hormone therapy regimens, 10-year survival was 90%. (11) However, other studies have shown a decrease in FEV 1 even after hormonal manipulation.…”

mentioning

confidence: 99%

“…(11) However, other studies have shown a decrease in FEV 1 even after hormonal manipulation. (12,14) In one retrospective study, treatment with goserelin, compared with the use of progesterone, showed a trend toward slowing the decline in FEV 1 over time. (15) To date, because of the rarity of the disease and the lack of standard treatment protocols, there have been no studies comparing treatment options in an appropriate manner.…”

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confidence: 99%

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Evolução da função pulmonar após tratamento com goserelina em pacientes com linfangioleiomiomatose

et al. 2011

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In the atypical smooth muscle cells that are characteristic of lymphangioleiomyomatosis (LAM), there are estrogen and progesterone receptors. Therefore, anti-hormonal therapy, despite having produced controversial results, can be considered a treatment option. The objective of this retrospective study was to evaluate hormonal and spirometric data for nine women with LAM after one year of treatment with goserelin. The mean increase in FEV 1 and FVC was 80 mL and 130 mL, respectively. There was effective blockage of the hormonal axis. It is still not possible to exclude a potential beneficial effect of the use of gonadotropin-releasing hormone analogues in LAM patients, which underscores the need for randomized trials.Keywords: Lymphangioleiomyomatosis; Spirometry; Goserelin. ResumoNas células musculares lisas atípicas características da linfangioleiomiomatose (LAM) encontram-se receptores de estrogênio e progesterona, de modo que o tratamento anti-hormonal pode ser considerado uma opção, mas ainda com resultados controversos. O objetivo deste trabalho foi avaliar retrospectivamente parâmetros hormonais e espirométricos em nove mulheres com LAM após o tratamento com goserelina por um ano. Houve um aumento médio de 80 mL e 130 mL, respectivamente, em VEF 1 e CVF, assim como bloqueio hormonal efetivo. Ainda não se pode excluir um potencial efeito favorável da utilização de análogos de hormônio liberador de gonadotrofina em pacientes com LAM, reforçando a necessidade de ensaios randomizados.

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Evolução da função pulmonar após tratamento com goserelina em pacientes com linfangioleiomiomatose

et al. 2011

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“…Patients should be suspected of having LAM when they present with typical HRCT changes, pneumothorax or chylothorax in order to avoid misdiagnosis (5). Pulmonary function tests can show an obstructive or restrictive pattern, and the condition is often accompanied with hypoxemia (15). The lung function and blood gas analysis are in line with these changes.…”

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confidence: 79%

Sporadic lymphangioleiomyomatosis with bloody sputum as an initial symptom: A case report and review of the literature

2015

Experimental and Therapeutic Medicine

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Abstract. Lymphangioleiomyomatosis (LAM) is an extremely rare lung disease affecting females of a childbearing age. The condition occurs sporadically or in association with tuberous sclerosis complex. The diagnosis of LAM is often delayed since the clinical symptoms and signs are non-specific. The present study reports the a case of a patient with LAM, in which bloody sputum was presented as the initial symptom. The 43-year-old female had experienced a small amount of bloody sputum over the previous five years. The patient was admitted to the Department of Respiratory Medicine at Taizhou People's Hospital (Taizhou, China) in 2012, reporting symptoms of a cough, shortness of breath and bloody sputum over the previous 10 days. A high-resolution computed tomography scan revealed multiple small circular thin-walled translucent areas in both lung fields. The initial diagnosis of the patient was LAM. A biopsy was performed using a video-assisted thoracoscopy. In conclusion, increased awareness and early diagnosis and treatment were determined to be key factors in ensuring a satisfactory prognosis.

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“…15 Univariate and multivariate regression analyses were performed to identify factors related to the pulmonary function rate of decline. 15 Analysis of variance and regression methods were used to compare lung function, lung involvement by CT scan, bronchodilator response, and functional decline. The bronchodilator response was considered both as a continuous measurement and as a categorical variable (never, response in Ͻ 50% of visits, or response in Ն 50% of the visits).…”

Section: Discussionmentioning

confidence: 99%

Reversible Airflow Obstruction in Lymphangioleiomyomatosis

Taveira-DaSilva

Steagall

Rabel

et al. 2009

Chest

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Background:We previously reported that approximately one-fourth of patients with lymphangioleiomyomatosis (LAM) may respond to therapy with bronchodilators. However, the validity of those observations has been questioned. The aims of the present study were to determine the prevalence of reversible airflow obstruction in patients with LAM and to identify associated clinical and physiologic parameters. Methods: First, the clinical and physiologic characteristics of 235 patients were analyzed to determine the frequency of the response to albuterol during a total of 2,307 visits. Second, we prospectively evaluated the response to albuterol (2.5 mg) and ipratropium (500 g) in 130 patients, and correlated their responses with their clinical and physiologic characteristics. Results: In the retrospective study, 51% of the patients responded at least once to bronchodilators; of these, 12% responded > 50% of the time. A higher frequency of positive bronchodilator responses was associated with greater rates of decline in FEV 1 and diffusing capacity of the lung for carbon monoxide (DLCO). In the prospective study, 39 patients (30%) responded to bronchodilators, including 12 to ipratropium, 9 to albuterol, and 18 to both. The prevalence of asthma and smoking in the 39 responders was not different from that seen in the 91 nonresponders. Patients who responded to ipratropium, albuterol, or both had significantly (p < 0.02) lower FEV 1 and DLCO, and a greater rate of FEV 1 decline (p ‫؍‬ 0.044) and DLCO decline (p ‫؍‬ 0.039) than patients who did not respond to these bronchodilators. After adjusting for FEV 1 /FVC ratio, DLCO decline also was greater in responders than in nonresponders (p ‫؍‬ 0.009). Conclusions

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

Cited by 211 publications

References 44 publications

Evolução da função pulmonar após tratamento com goserelina em pacientes com linfangioleiomiomatose

Evolução da função pulmonar após tratamento com goserelina em pacientes com linfangioleiomiomatose

Sporadic lymphangioleiomyomatosis with bloody sputum as an initial symptom: A case report and review of the literature

Reversible Airflow Obstruction in Lymphangioleiomyomatosis

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