MARIANA SPONHOLZ ARAUJO

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7
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Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina - Médico

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Agora exibindo 1 - 10 de 23
  • conferenceObject
    Evaluation Of The Functional Impact Of The Use Of Doxycycline For Three Years In Patients With Lymphangioleiomyomatosis
    (2014) BALDI, B. G.; PIMENTA, S. P.; ARAUJO, M. S.; FREITAS, C. S. G.; KAIRALLA, R. A.; CARVALHO, C. R. R.
  • bookPart
    Fibrose Pumonar Idiopática
    (2016) ARAUJO, Mariana Sponholz; DOURADO, Leticia Kawano; BALDI, Bruno Guedes; KAIRALLA, Ronaldo Adib; CARVALHO, Carlos Roberto Ribeiro de
  • conferenceObject
    Rapamycin As Treatment Of Lymphangioleiomyomatosis: Favorable Response In Different Clinical Manifestations
    (2014) FREITAS, C. S. G.; ARAUJO, M. S.; BALDI, B. G.; KAIRALLA, R. A.; CARVALHO, C. R. R.
  • article 14 Citação(ões) na Scopus
    Colagenose pulmão dominante em pacientes com doença pulmonar intersticial: prevalência, estabilidade funcional e manifestações extratorácicas comuns
    (2015) PEREIRA, Daniel Antunes Silva; DIAS, Olivia Meira; ALMEIDA, Guilherme Eler de; ARAUJO, Mariana Sponholz; KAWANO-DOURADO, Leticia Barbosa; BALDI, Bruno Guedes; KAIRALLA, Ronaldo Adib; CARVALHO, Carlos Roberto Ribeiro
    Objective: To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD). Methods: This was a retrospective study of patients with interstitial lung disease (ILD), positive antinuclear antibody (ANA) results (>= 1/320), with or without specific autoantibodies, and at least one clinical feature suggestive of connective tissue disease (CO). Results: Of the 1,998 patients screened, 52 initially met the criteria for a diagnosis of LD-CTD: 37% were male; the mean age at diagnosis was 56 years; and the median follow-up period was 48 months. During follow-up, 8 patients met the criteria for a definitive diagnosis of a CTD. The remaining 44 patients comprised the LD-CTD group, in which the most prevalent extrathoracic features were arthralgia, gastroesophageal reflux disease, and Raynaud's phenomenon. The most prevalent autoantibodies in this group were ANA (89%) and anti-SSA (anti-Ro, 27%). The mean baseline and final FVC was 69.5% and 74.0% of the predicted values, respectively (p > 0.05). Nonspecific interstitial pneumonia and usual interstitial pneumonia patterns were found in 45% and 9% of HRCT scans, respectively; 36% of the scans were unclassifiable. A similar prevalence was noted in histological samples. Diffuse esophageal dilatation was identified in 52% of HRCT scans. Nailfold capillaroscopy was performed in 22 patients; 17 showed a scleroderma pattern. Conclusions: In our LD-CTD group, there was predominance of females and the patients showed mild spirometric abnormalities at diagnosis, with differing underlying ILD patterns that were mostly unclassifiable on HRCT and by histology. We found functional stability on follow-up. Esophageal dilatation on HRCT and scleroderma pattern on nailfold capillaroscopy were frequent findings and might come to serve as diagnostic criteria.
  • article 25 Citação(ões) na Scopus
    CLINICAL COURSE AND CHARACTERISATION OF LYMPHANGIOLEIOMYOMATOSIS IN A BRAZILIAN REFERENCE CENTRE
    (2014) BALDI, B. Guedes; SALIM, C.; FREITAS, G.; ARAUJO, M. Sponholz; DIAS, O. Meira; PEREIRA, D. A. Silva; PIMENTA, S. Pinheiro; KAIRALLA, R. A.; CARVALHO, C. R. Ribeiro
    Background and objective: Lymphangioleiomyomatosis (LAM) is a rare disease that promotes pulmonary cystic destruction and impairs pulmonary function. We aim to describe features and clinical course of LAM patients from Brazil. Methods: We described the clinical and functional features, performance in six minute walk test (6MWT), management details, survival and clinical course of 84 LAM patients followed in a Brazilian reference centre. Results: All subjects were women, the average age at onset of symptoms was 38 years, and the average at diagnosis was 42 years. The major symptoms during the course of the disease were dyspnoea and pneumothorax. The patients experienced impaired quality of life, with worse scores in the physical and emotional domains. The most common abnormalities in pulmonary function tests were an obstructive pattern and reduced diffusion capacity, whereas a quarter of the patients had normal spirometric results. In the 6MWT, although patients had preserved exercise capacity, more than half of the patients had significant desaturation. Hormonal blockage and doxycycline were the most common treatment modalities employed in our patients. The survival probability from diagnosis was 90% at 5 years, whereas the mean annual rate of decline in FEV1 was 60 +/- 78 mL. Conclusions: Clinical and functional features of the LAM patients from our centre are similar to those from other countries. Our sample showed preserved exercise capacity, with desaturation in the 6MWT, and impaired quality of life. Survival was similar, whereas the annual rate of decline of FEV1 was slightly lower than in recent studies.
  • article 25 Citação(ões) na Scopus
    Use of sirolimus in the treatment of lymphangioleiomyomatosis: favorable responses in patients with different extrapulmonary manifestations
    (2015) FREITAS, Carolina Salim Gonçalves; BALDI, Bruno Guedes; ARAÚJO, Mariana Sponholz; HEIDEN, Glaucia Itamaro; KAIRALLA, Ronaldo Adib; CARVALHO, Carlos Roberto Ribeiro
    OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a rare disease that is currently considered a low-grade neoplasm with metastatic potential and variable progression. Mammalian target of rapamycin (mTOR) inhibitors, such as sirolimus and everolimus, have recently become a treatment option for LAM patients, especially those with extrapulmonary manifestations. The objective of the present study was to describe a case series of four patients with LAM in Brazil who showed significant improvement, particularly in their extrapulmonary manifestations, after treatment with sirolimus (at 1-4 mg/day). METHODS: We describe four cases of LAM patients with different extrapulmonary manifestations who were treated with sirolimus. RESULTS: After treatment with sirolimus for 12 months, one patient presented resolution of severe chylothorax; one had a significant reduction in renal angiomyolipoma volume; and one showed significant regression of retroperitoneal lymphangioleiomyomas and abdominal lymph node enlargement. After treatment with sirolimus for 6 months, the remaining patient had a significant reduction in the volume of a massive retroperitoneal lymphangioleiomyoma. CONCLUSIONS: Our findings confirm that mTOR inhibitors are beneficial for patients with LAM, especially those with extrapulmonary manifestations, such as renal angiomyolipoma, lymphangioleiomyomas, and chylous effusions. However, certain aspects, such as the optimal dose, duration of treatment, and long-term adverse effects, have yet to be sufficiently clarified for mTOR inhibitors to be incorporated into LAM management protocols.
  • conferenceObject
    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
  • article 22 Citação(ões) na Scopus
    Pulmonary rehabilitation in lymphangioleiomyomatosis: a controlled clinical trial
    (2016) ARAUJO, Mariana S.; BALDI, Bruno G.; FREITAS, Carolina S. G.; ALBUQUERQUE, Andre L. P.; SILVA, Cibele C. B. Marques da; KAIRALLA, Ronaldo A.; CARVALHO, Celso R. F.; CARVALHO, Carlos R. R.
    Lymphangioleiomyomatosis (LAM) is a cystic lung disease frequently associated with reduced exercise capacity. The aim of this study was to assess safety and efficacy of pulmonary rehabilitation in LAM. This controlled clinical trial included 40 patients with LAM and a low physical activity level. The pulmonary rehabilitation programme comprised 24 aerobic and muscle strength training sessions and education. The primary outcome was exercise capacity (endurance time during a constant work rate exercise test). Secondary outcomes included health-related quality of life (St George's Respiratory Questionnaire (SGRQ)), 6-min walking distance (6MWD), dyspnoea, peak oxygen consumption (V'O-2), daily physical activity (pedometer), symptoms of anxiety and depression, lung function and peripheral muscle strength (one-repetition maximum). The baseline characteristics were well balanced between the groups. The pulmonary rehabilitation group exhibited improvements in the following outcomes versus controls: endurance time (median (interquartile range) 169 (2-303) s versus -33 (-129-39) s; p=0.001), SGRQ (median (interquartile range) -8 (-16-2) versus 2 (-4-5); p=0.002) and 6MWD (median (interquartile range) 59 (13-81) m versus 20 (-12-30) m; p=0.002). Dyspnoea, peak V'O-2, daily physical activity and muscle strength also improved significantly. No serious adverse events were observed. Pulmonary rehabilitation is a safe intervention and improves exercise capacity, dyspnoea, daily physical activity, quality of life and muscle strength in LAM.
  • article 10 Citação(ões) na Scopus
    Pneumomediastinum, subcutaneous emphysema, and pneumothorax after a pulmonary function testing in a patient with bleomycin-induced interstitial pneumonitis
    (2013) ARAUJO, Mariana Sponholz; FERNANDES, Frederic Leon Arrabal; KAY, Fernando Uliana; CARVALHO, Carlos Roberto Ribeiro
    Spontaneous pneumomediastinum is an uncommon event, the clinical picture of which includes retrosternal chest pain, subcutaneous emphysema, dyspnea, and dysphonia. The pathophysiological mechanism involved is the emergence of a pressure gradient between the alveoli and surrounding structures, causing alveolar rupture with subsequent dissection of the peribronchovascular sheath and infiltration of the mediastinum and subcutaneous tissue with air. Known triggers include acute exacerbations of asthma and situations that require the Valsalva maneuver. We described and documented with HRCT scans the occurrence of pneumomediastinum after a patient with bleomycin-induced interstitial lung disease underwent pulmonary function testing. Although uncommon, the association between pulmonary function testing and air leak syndromes has been increasingly reported in the literature, and lung diseases, such as interstitial lung diseases, include structural changes that facilitate the occurrence of this complication.
  • 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