SUZANA PINHEIRO PIMENTA
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article 37 Citação(ões) na Scopus Doxycycline use in patients with lymphangioleiomyomatosis: safety and efficacy in metalloproteinase blockade(2011) PIMENTA, Suzana Pinheiro; BALDI, Bruno Guedes; ACENCIO, Milena Marques Pagliarelli; KAIRALLA, Ronaldo Adib; CARVALHO, Carlos Roberto RibeiroObjective: Lymphangioleiomyomatosis (LAM) is characterized by lung cysts, whose development is associated with matrix metalloproteinase (MMP) hyperactivity, principally that of MMP-2 and MMP-9. Our objective was to compare LAM patients and controls in terms of the levels of these MMPs, as well as to determine the safety and efficacy of treatment with doxycycline, a potent MMP inhibitor. Methods: Prospective clinical study involving female LAM patients who received doxycycline (100 mg/day) for six months. Urine and blood samples were collected for the quantification of MMP-2 and MMP-9 before and after the treatment period. Samples from 10 healthy women were also collected. Results: Of the 41 LAM patients who started the treatment, 34 completed the protocol. Serum and urinary MMP-9 levels were significantly lower in the controls than in the LAM patients (p < 0.0001). Comparing pre- and post-treatment values, we found that the median level of MMP-9 in serum decreased from 919 ng/mL to 871 ng/mL (p = 0.05), whereas that of MMP-9 in urine decreased from 11,558 pg/mL to 7,315 pg/mL (p = 0.10). After treatment, the median level of MMP-2 in serum was significantly lower (p = 0.04) and urinary MMP-2 levels were undetectable. Nausea, diarrhea, and epigastric pain were the most prevalent adverse affects and were often self-limiting. There was only one case in which the patient discontinued the treatment because of side effects. Conclusions: We have demonstrated, for the first time, a decrease in serum and urine levels of MMPs in LAM patients treated with doxycycline, which proved to be a safe medication, with mild and well-tolerated side effects.conferenceObject Exercise in patients with lymphangioleiomyomatosis: Performance and the prevalence of dynamic hyperinflation(2012) ALBUQUERQUE, Andre; BALDI, Bruno; PIMENTA, Suzana; SALGE, Joao Marcos; KAIRALLA, Ronaldo; CARVALHO, CarlosconferenceObject Evaluation and correlations of the extension of pulmonary cysts in lymphangioleiomyomatosis(2013) ARAUJO, Mariana Sponholz; FREITAS, Carolina Salim Goncalves; BALDI, Bruno Guedes; TELES, Gustavo Borges da Silva; PIMENTA, Suzana Pinheiro; PEREIRA, Daniel Antunes Silva; DIAS, Olivia Meira; COSTA, Andre Nathan; KAWANO-DOURADO, Leticia; KAWASSAKI, Alexandre; KAIRALLA, Ronaldo Adib; CARVALHO, Carlos Roberto Ribeiro- Pulmonary arterial involvement leading to alveolar hemorrhage in lymphangioleiomyomatosis(2011) BALDI, Bruno Guedes; PIMENTA, Suzana Pinheiro; KAWASSAKI, Alexandre de Melo; BERNARDI, Fabiola Del Carlo; DOLHNIKOFF, Marisa; CARVALHO, Carlos Roberto Ribeiro
conferenceObject Characterization of 76 patients with lymphangioleiomyomatosis from a Brazilian reference center(2012) FREITAS, Carolina; ARAUJO, Mariana; BALDI, Bruno; PIMENTA, Suzana; KAIRALLA, Ronaldo; CARVALHO, Carlos Roberto- Exercise Performance and Dynamic Hyperinflation in Lymphangioleiomyomatosis(2012) BALDI, Bruno G.; ALBUQUERQUE, Andre L. P.; PIMENTA, Suzana P.; SALGE, Joao M.; KAIRALLA, Ronaldo A.; CARVALHO, Carlos R. R.Rationale: Lymphangioleiomyomatosis (LAM) is characterized by exercise performance impairment. Although airflow limitation is common, no previous studies have evaluated the prevalence and impact of dynamic hyperinflation (DH) in LAM. Objectives: To investigate the dynamic responses during maximal exercise and the prevalence, predictors, and repercussions of DH in LAM. Methods: Forty-two patients with LAM performed symptom-limited incremental cycle exercise and pulmonary functions tests (PFTs) and were compared with 10 age-matched healthy women. Dyspnea intensity, inspiratory capacity, oxygen saturation, and cardiac, metabolic, and respiratory variables were assessed during exercise. Patients with LAM also performed a 6-minute walk test (6MWT). Measurements and Main Results: Patients with LAM had higher baseline dyspnea, poorer quality of life, obstructive pattern, air trapping, and reduced diffusing capacity of carbon monoxide in PFTs. Although they had the same level of regular physical activity, their maximal exercise performance was reduced and was associated with ventilatory limitation, greater desaturation, and dyspnea. The prevalence of DH was high in LAM (55%), even in patients with mild spirometric abnormalities, and was correlated with airflow obstruction, air trapping, and diffusing capacity of carbon monoxide. Compared with the non-DH subgroup, the patients who developed DH had a ventilatory limitation contributing to exercise cessation on cycling and higher desaturation and dyspnea intensity during the 6MWT. Conclusions: Ventilatory limitation and gas exchange impairment are important causes of exercise limitation in LAM. DH is frequent in LAM, even in patients with mild spirometric abnormalities. DH was associated with the severity of disease, higher dyspnea, and lower oxygen saturation. In the 6MWT, desaturation and dyspnea were greater in patients with DH.
- A pilot study assessing the effect of bronchodilator on dynamic hyperinflation in LAM(2013) BALDI, Bruno Guedes; ALBUQUERQUE, Andre Luis Pereira de; PIMENTA, Suzana Pinheiro; SALGE, Joao Marcos; KAIRALLA, Ronaldo Adib; CARVALHO, Carlos Roberto RibeiroIntroduction: Positive responses to bronchodilators (BDs) on spirometry can be found in up to 30% of patients with lymphangioleiomyomatosis (LAM). However, no previous studies have investigated the impact of BDs on exercise outcomes, including dynamic hyperinflation (DH). Methods: A randomised, double-blind, placebo-controlled, crossover trial was conducted on 38 patients with LAM, comparing inhaled placebo versus salbutamol. Pulmonary function tests and a cycle endurance test at 75% of the maximal work capacity, with evaluation of DH by serial measurement of inspiratory capacity (IC), which was the primary endpoint, were performed after each intervention. Results: Although salbutamol produced a slight improvement in airway obstruction, compared with placebo, there was no significant variation in resting IC or air trapping. A total of 18% of the patients met the criteria for a positive response to BD. During submaximal exercise, BD did not reduce DH or dyspnoea nor did it improve exercise tolerance in the entire population. In addition, BD produced only slight improvement in FEV1 and air trapping in patients who had DH during incremental cardiopulmonary exercise testing, without the beneficial effects on exercise outcomes. Conclusions: Although salbutamol produced a slight improvement in airway obstruction, it did not lead to a reduction in DH or increase in exercise tolerance in patients with LAM. Clinical trial registration: Brazilian Registry of Clinical Trials (ReBEC); www.ensaiosclinicos.gov.br; registration number: RBR-49sk2j.
conferenceObject Clinical, spirometric and radiological characteristics of Brazilian rheumatoid arthritis patients(2012) KAWASSAKI, Alexandre Melo; PIMENTA, Suzana Pinheiro; KAY, Fernando; VERCOSA-KAWASSAKI, Marianne Karel; LAURINDO, Ieda Maria Magalhaes; CARVALHO, Carlos Roberto Ribeiro; KAIRALLA, Ronaldo Adib- Birt-Hogg-Dube syndrome: metalloproteinase activity and response to doxycycline(2012) PIMENTA, Suzana Pinheiro; BALDI, Bruno Guedes; NASCIMENTO, Ellen Caroline Toledo do; MAUAD, Thais; KAIRALLA, Ronaldo Adib; CARVALHO, Carlos Roberto Ribeiro
- Sleep desaturation and its relationship to lung function, exercise and quality of life in LAM(2012) MEDEIROS JR., Pedro; LORENZI-FILHO, Geraldo; PIMENTA, Suzana P.; KAIRALLA, Ronaldo A.; CARVALHO, Carlos R. R.Background: Lymphangioleiomyomatosis (LAM) is characterised by progressive airway obstruction and hypoxaemia in young women. Although sleep may trigger hypoxaemia in patients with airway obstruction, it has not been previously investigated in patients with LAM. Methods: Consecutive women with lung biopsy proven LAM and absence of hypoxaemia while awake were evaluated with pulmonary function test, echocardiography, 6-min walk test, overnight full polysomnography, and Short Form 36 health-related quality-of-life questionnaire. Results: Twenty-five patients with (mean +/- SD) age 45 +/- 10 years, SpO(2) awake 95% +/- 2, forced expiratory volume in the first second (median-interquartile) FEV1 (% predicted) 77 (47-90) and carbonic monoxide diffusion capacity, DLCO (%) 55 (34-74) were evaluated. Six-minute walk test distance and minimum SpO(2) (median-interquartile) were, respectively, 447 m (411 -503) and 90% (82-94). Median interquartile apnoea-hypopnoea index was in the normal range 2 (1-5). Fourteen patients (56%) had nocturnal hypoxaemia (10% total sleep time with SpO(2) <90%), and the median sleep time spent with SpO(2) <90% was 136 (13-201) min. Sleep time spent with SpO(2) <90% correlated with the residual volume/total lung capacity ratio (r(s) = 0.5, p: 0.02), DLCO (r(s) = -0.7, p: 0.001), FEV1 (r(s) = -0.6, p: 0.002). Multivariate linear regression model showed that RV/TLC ratio was the most important functional variable related to sleep hypoxaemia. Conclusion: Significant hypoxaemia during sleep is common in LAM patients with normal SpO(2) while awake, especially among those with some degree of hyperinflation in lung function tests. (C) 2011 Published by Elsevier Ltd.