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1.
Int J Rheum Dis ; 22(9): 1741-1745, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31328423

RESUMO

BACKGROUND: Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is associated with high mortality, but there is limited clinical data on AE of interstitial lung disease (ILD) in connective tissue disease-associated ILD (CTD ILD). The present study was conducted to provide prevalence and clinical features of AE, as well as various risk factors associated with mortality among AE CTD ILD patients. METHODS: Between May 2013 and April 2018, 15 patients who developed AE among 105 consecutive patients with CTD with chronic ILD were included. AE was defined using the criteria recently proposed by the IPF net, with slight modification for adaptation to CVD-IP6 (collagen vascular disease-associated interstitial pneumonia), and patients having CTD with AE met all the criteria. RESULTS: Fifteen patients with mean age of 45.8 ± 13.9 years developed AE; the most common subgroup (n = 5, 33%) was systemic sclerosis. The mean duration (months) between diagnosis of ILD and AE was 56.5 ± 38.0 with mean follow-up duration of 24 ± 18.1 months. The baseline arterial oxygen pressure (PaO2 ) was 81.7 ± 8.1 mm Hg and mean forced vital capacity (%) was 57.9 ± 8.9. Five patients requiring mechanical ventilation died. Patients with shorter duration (months) of disease between onset of ILD to AE had higher mortality, 40.4 ± 45.1 vs 64.6 ± 33.6. Those who had significantly lower baseline PaO2 (mean ± SD), 72.6 ± 3.4 vs 86.2 ± 5.3 mm Hg (P = .002) had higher mortality. CONCLUSIONS: In our study, the majority of patients with AE CTD ILD had systemic sclerosis. Patients with lower baseline PaO2 and those requiring mechanical ventilation had higher mortality.


Assuntos
Doenças do Tecido Conjuntivo/epidemiologia , Fibrose Pulmonar Idiopática/epidemiologia , Doenças Pulmonares Intersticiais/epidemiologia , Adulto , Doenças do Tecido Conjuntivo/diagnóstico , Doenças do Tecido Conjuntivo/mortalidade , Doenças do Tecido Conjuntivo/terapia , Progressão da Doença , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/terapia , Imunossupressores/uso terapêutico , Índia/epidemiologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/terapia , Masculino , Pessoa de Meia-Idade , Prevalência , Prognóstico , Estudos Prospectivos , Respiração Artificial , Medição de Risco , Fatores de Risco , Fatores de Tempo
2.
N Z Med J ; 132(1499): 36-42, 2019 07 26.
Artigo em Inglês | MEDLINE | ID: mdl-31352472

RESUMO

AIM: In light of new therapies and guidelines for the management of idiopathic pulmonary fibrosis (IPF), and in the absence of local epidemiological data, we sought to ascertain a current estimate of the prevalence of IPF in Canterbury and to audit local practices. METHODS: We performed a retrospective observational study of patients with IPF in Canterbury, New Zealand and the wider region. Patients were identified through a systematic search of hospital records and included if they were alive on 1 January 2017, had a histological or radiological diagnosis of usual interstitial pneumonia and clinical correlation consistent with a diagnosis of IPF. Clinical data was extracted from the clinical record. Follow up was complete until April 2018. RESULTS: Sixty-eight patients were included, median follow up 33 (14-49) months. Fifteen (22.1%) patients died during follow up, median survival 19 (6.5-54) months. Estimated prevalence of IPF was 6.53/100,000 persons. Six (8.8%) patients were discussed at the Interstitial lung disease multi-disciplinary meeting. Resting Sp02 and end-of-life discussions were documented in 44 (64.7%) and 19 (27.9%) patients respectively, while oxygen therapy was prescribed to 15 (22.7%). 20/36 (55.5%) patients eligible for pirfenidone were treated. Those treated were more likely to have undergone a six-minute walk test (5/20 vs 3/48, p<0.05) or have been hospitalised in the last 12 months (12/20 vs 3/48, p<0.05). 7/20 patients remained on treatment at the end of follow-up (eight discontinued, five deceased). CONCLUSION: In this study the estimated prevalence of IPF in the Canterbury region is 6.53/100,000 persons. Furthermore, we have identified limitations in local practice relevant for service development.


Assuntos
Fibrose Pulmonar Idiopática , Hospitalização/estatística & dados numéricos , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/epidemiologia , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/terapia , Nova Zelândia/epidemiologia , Prevalência , Estudos Retrospectivos
3.
Ther Adv Respir Dis ; 13: 1753466619847130, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31170875

RESUMO

BACKGROUND: Some patients with idiopathic pulmonary fibrosis (IPF) develop acute exacerbation (AE-IPF) leading to severe acute respiratory failure (ARF); despite conventional supportive therapy, the mortality rate remains extremely high. The aim of this study was to assess how a treatment algorithm incorporating high-flow nasal cannula (HFNC) oxygen therapy affects the short-term mortality of patients with AE-IPF who develop ARF. METHOD AND DESIGN: A retrospective cohort analysis was conducted. PATIENTS AND INTERVENTIONS: The study consisted of 17 patients with AE-IPF admitted to a respiratory intensive care unit (RICU) for ARF managed using a treatment algorithm incorporating HFNC. The outcome measure was mortality rate during their stay in the RICU. RESULTS: Implementation of the treatment algorithm led to a successful outcome in nine patients and to a negative one in eight patients (47.1%) who died within 39 days of being admitted to the RICU. The survival rate was 70.6% (±0.1 %) at 15 days, 52.9% (±0.1%) at 30 days, 35.3% (±0.1%) at 90 days, and 15.6% (±9.73 %) at 365 days. Overall, 4 out of 10 patients who did not respond to conventional oxygen therapy showed a satisfactory response to HFNC. CONCLUSIONS: Short-term mortality fell to below 50% when a treatment algorithm incorporating HFNC was implemented in a group of patients with AE-IPF admitted to a RICU for ARF. Patients not responding to conventional oxygen therapy seemed to benefit from HFNC. The reviews of this paper are available via the supplementary material section.


Assuntos
Algoritmos , Fibrose Pulmonar Idiopática/terapia , Oxigenoterapia/métodos , Insuficiência Respiratória/terapia , Doença Aguda , Idoso , Idoso de 80 Anos ou mais , Cânula , Estudos de Coortes , Feminino , Humanos , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/mortalidade , Unidades de Terapia Intensiva , Masculino , Pessoa de Meia-Idade , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/mortalidade , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento
4.
Medicine (Baltimore) ; 98(18): e15140, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31045757

RESUMO

RATIONALE: Idiopathic pulmonary fibrosis (IPF) is a special form of spontaneous, chronic, progressive interstitial fibrotic pneumonia of unknown cause, and treatments for IPF have shown a poor prognosis. This study reports a new treatment, traditional Chinese medicine (TCM) therapy, for tonifying Qi-deficiency of lung-kidney in a 64-year-old patient with IPF. PATIENT CONCERNS: A 64-year-old man, who complained of cough and asthma, was diagnosed as IPF with mild impairment in lung function by thoracic high-resolution computed tomography and pulmonary function test. He received an 18-month N-acetylcysteine monotherapy but had no improvement in lung function. DIAGNOSES: IPF with mild impairment in lung function was diagnosed. INTERVENTIONS: The Chinese herbal medicine composition was decocted in 300 ml water for oral administration with 150 ml decoction twice daily in June 2017. OUTCOMES: The pulmonary function test showed that diffusing capacity for carbon monoxide had increased to 81% of predicted back to normal after 2-month TCM monotherapy. And diffusing capacity for carbon monoxide had increased to 89% of predicted, and forced expiratory volume in 1 s/forced vital capacity ratio increased to 92% at 14-month follow-up. No adverse events occurred during the 14 months of therapy and observation. LESSONS: The treatment by TCM therapy of tonifying lung-kidney's Qi-deficiency for IPF can improve the pulmonary function and reverse disease progression; it may be considered as a complementary treatment for IPF with mild-to-moderate impairment. However, the insights provided in this case report require further exploration and verification.


Assuntos
Fibrose Pulmonar Idiopática/terapia , Rim/efeitos dos fármacos , Pulmão/efeitos dos fármacos , Medicina Tradicional Chinesa/métodos , Acetilcisteína/uso terapêutico , Monóxido de Carbono/metabolismo , Progressão da Doença , Expectorantes/uso terapêutico , Volume Expiratório Forçado/efeitos dos fármacos , Humanos , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/fisiopatologia , Rim/fisiopatologia , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Capacidade de Difusão Pulmonar/efeitos dos fármacos , Testes de Função Respiratória/métodos , Resultado do Tratamento , Capacidade Vital/efeitos dos fármacos , Deficiência da Energia Yin
6.
Rev Mal Respir ; 36(4): 508-518, 2019 Apr.
Artigo em Francês | MEDLINE | ID: mdl-31006579

RESUMO

INTRODUCTION: In 2015, the International Society for Heart and Lung Transplantation (ISHLT) published a consensus document for the selection of lung transplant candidates. In the absence of recent French recommendations, this guideline is useful in order to send lung transplant candidates to the transplantation centers and to list them for lung transplantation at the right time. BACKGROUND: The main indications for lung transplantation in adults are COPD and emphysema, idiopathic pulmonary fibrosis and interstitial diseases, cystic fibrosis and pulmonary arterial hypertension (PAH). The specific indications for each underlying disease as well as the general contraindications have been reviewed in 2015 by the ISHLT. For cystic fibrosis, the main factors are forced expiratory volume in one second, 6-MWD, PAH and clinical deterioration characterized by increased frequency of exacerbations; for emphysema progressive disease, the BODE score, hypercapnia and FEV1; for PAH progressive disease or the need of specific intravenous therapy and NYHA classification. Finally, the diagnosis of fibrosing interstitial lung disease is usually a sufficient indication for lung transplantation assessment. OUTLOOK AND CONCLUSION: These new recommendations, close to French practices, help clinicians to find the right time for referral of patients to transplantation centers. This is crucial for the prognosis of lung transplantation.


Assuntos
Transplante de Pulmão/métodos , Seleção de Pacientes , Adulto , Contraindicações , Fibrose Cística/epidemiologia , Fibrose Cística/terapia , França/epidemiologia , Transplante de Coração-Pulmão/efeitos adversos , Transplante de Coração-Pulmão/métodos , Transplante de Coração-Pulmão/normas , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/terapia , Fibrose Pulmonar Idiopática/epidemiologia , Fibrose Pulmonar Idiopática/terapia , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/terapia , Transplante de Pulmão/efeitos adversos , Transplante de Pulmão/normas , Transplante de Pulmão/estatística & dados numéricos , Guias de Prática Clínica como Assunto/normas , Doença Pulmonar Obstrutiva Crônica/epidemiologia , Doença Pulmonar Obstrutiva Crônica/terapia , Enfisema Pulmonar/epidemiologia , Enfisema Pulmonar/terapia
7.
Am J Med Sci ; 357(5): 359-369, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-31010461

RESUMO

The incidence and prevalence of idiopathic pulmonary fibrosis (IPF) is increasing worldwide. This, combined with its poor prognosis and unpredictable natural history, has amplified the importance of an accurate diagnosis and monitoring. A diagnosis of exclusion, IPF requires a comprehensive clinical evaluation. This results in a clinical context that provides the backdrop for interpretation of the chest imaging and histopathology. A confident or probable usual interstitial pneumonia chest imaging pattern on high-resolution computerized tomography may be diagnostic in the correct clinical context. Outcomes for IPF are unpredictable, ranging from rapid progression with death within months to prolonged stability. Disease activity is monitored by clinical and physiology measures with a declining forced vital capacity, a recognized measure of progression. The available treatments, pirfenidone and nintedanib, are effective at reducing the expected decline in forced vital capacity.


Assuntos
Fibrose Pulmonar Idiopática , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/epidemiologia , Fibrose Pulmonar Idiopática/terapia
8.
Am J Med Sci ; 357(5): 370-373, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-31010462

RESUMO

The last years have led to advances in the therapeutic management of idiopathic pulmonary fibrosis (IPF), mainly through the discovery of new pathological pathways and drugs and better design of clinical trials. The objective of this review is both to describe the current therapies approved for the treatment of IPF and the emerging therapeutic approaches. Currently, nintedanib and pirfenidone are the basis of IPF therapy, based on the results of large randomized clinical trials showing their safety and efficacy in reducing disease progression. Nonetheless, the ideal IPF therapy is still lacking and trials are underway to test new therapeutic targets. The near future could bring to clinicians and patients a combined therapeutic strategy, hitting the disease from several simultaneous pathways and hopefully leading to clinical stabilization or improvement.


Assuntos
Anti-Inflamatórios/uso terapêutico , Fibrose Pulmonar Idiopática/terapia , Indóis/uso terapêutico , Piridonas/uso terapêutico , Progressão da Doença , Humanos
9.
Am J Med Sci ; 357(5): 374-378, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-31010463

RESUMO

Idiopathic pulmonary fibrosis is a disease of older adults leading to progressive dyspnea and reduced exercise capacity, typically resulting in death within 3-5years of diagnosis. Underlying genetic susceptibility combined with environmental insults is proposed to trigger a chronic wound repair response, leading to activation of the fibrotic cascade. Perturbations in several molecular pathways mediate vulnerability of the alveolar epithelium to injurious agents, including the unfolded protein response, autophagy, mitophagy, and cellular senescence. These cellular responses are intricately intertwined and link genetic susceptibility to the progressive fibrotic phenotype. Ongoing studies investigating these pathways in type II alveolar epithelial cells show promise for identifying new targeted interventions that could prevent or halt the progression of IPF.


Assuntos
Células Epiteliais Alveolares/fisiologia , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/terapia , Idoso , Humanos , Pessoa de Meia-Idade
10.
Am J Med Sci ; 357(5): 390-393, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-31010466

RESUMO

With the combination of ideologic beliefs and the will to survive, fraught patients and determined clinicians seek alternative therapies for treatment of terminal conditions, such as idiopathic pulmonary fibrosis. Unfortunately, the need for treatment has supported the growth of unapproved stem cell therapy over the years spanning across many countries. The reality, however, is that the science behind this therapy is lagging. While there have been promising results from phase I trials, there remain multiple reasons that "stem cells" are not ready for clinical application, starting from a gap in understanding at the bench research level, all the way to optimal clinical application in order to provide effective therapy.


Assuntos
Fibrose Pulmonar Idiopática/terapia , Transplante de Células-Tronco Mesenquimais , Células-Tronco Mesenquimais/fisiologia , Ensaios Clínicos Fase I como Assunto
11.
Zhongguo Zhen Jiu ; 39(3): 241-5, 2019 Mar 12.
Artigo em Chinês | MEDLINE | ID: mdl-30942008

RESUMO

OBJECTIVE: To evaluate the clinical efficacy of umbilical moxibustion in the treatment of patients with idiopathic pulmonary interstitial fibrosis complicated with gastroesophageal reflux of lung-spleen qi deficiency. METHODS: A total of 116 patients with idiopathic pulmonary fibrosis complicated with gastroesophageal reflux of lung spleen-deficiency were randomized into an observation group (58 cases) and a control group (58 cases). In the control group, the routine treatment was applied; in the observation group, on the base of the treatment as the control group, the umbilical moxibustion was used. The treatment was given 2 times a week, 2 h each time for 12 weeks, and followed up for 12 weeks after treatment. The composite physiological indicators (CPI), TCM syndrome scores, the St. George respiratory questionnaire score (SGRQ) and 6-minute walk test (6MWT) were compared before and after treatment in the two groups. RESULTS: The improvement of the TCM syndrome scores (cough, post-activity shortness of breath, fatigue, heartburn, acid reflux), CPI score and 6MWT in the observation group was better than those in the control group (all P<0.05). There was no significant difference in the scores of SGRQ scale between the two groups after treatment (P>0.05). There was no significant difference in the scores of CPI scores and syndromes (cough and fatigue) between the two groups at 12 weeks after treatment (all P>0.05). The 6MWT, SGRQ scale scores and syndrome scores (post-activity shortness of breath, acid reflux, heartburn) were better than those in the control group in the follow up (all P<0.05). CONCLUSION: Umbilical moxibustion can improve the ventilation and diffusion function of lung in patients with idiopathic pulmonary fibrosis and gastroesophageal reflux of lung-spleen qi deficiency, reduce clinical symptoms and improve patients' quality of life and activity endurance. And it has long-term effect.


Assuntos
Refluxo Gastroesofágico , Fibrose Pulmonar Idiopática , Moxibustão , Humanos , Fibrose Pulmonar Idiopática/terapia , Qi , Qualidade de Vida , Baço
12.
Subcell Biochem ; 91: 45-74, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30888649

RESUMO

Chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis are regarded as a diseases of accelerated lung ageing and show all of the hallmarks of ageing, including telomere shortening, cellular senescence, activation of PI3 kinase-mTOR signaling, impaired autophagy, mitochondrial dysfunction, stem cell exhaustion, epigenetic changes, abnormal microRNA profiles, immunosenescence and a low grade chronic inflammation due to senescence-associated secretory phenotype (SASP). Many of these ageing mechanisms are driven by exogenous and endogenous oxidative stress. There is also a reduction in anti-ageing molecules, such as sirtuins and Klotho, which further accelerate the ageing process. Understanding these molecular mechanisms has identified several novel therapeutic targets and several drugs and dietary interventions are now in development to treat chronic lung disease.


Assuntos
Envelhecimento/patologia , Pneumopatias/patologia , Senescência Celular , Humanos , Fibrose Pulmonar Idiopática/patologia , Fibrose Pulmonar Idiopática/terapia , Pulmão/efeitos dos fármacos , Pulmão/patologia , Pneumopatias/terapia , Estresse Oxidativo , Doença Pulmonar Obstrutiva Crônica/patologia , Doença Pulmonar Obstrutiva Crônica/terapia
13.
Rev Med Suisse ; 15(641): 536-541, 2019 Mar 06.
Artigo em Francês | MEDLINE | ID: mdl-30860324

RESUMO

Diffuse interstitial lung disease (ILD) is one of the most frequent extra-articular manifestation of rheumatoid arthritis (RA) and is an important factor of morbidity and mortality. However, the physiopathological mechanisms underlying RA-associated ILD remain poorly understood, and disease management is difficult in the absence of effective treatments and international guidelines. The recent identification of genetic variants and mutations similar to those observed in idiopathic pulmonary fibrosis (IPF), a disease affecting exclusively the lung, provides new insights into the understanding of RA-associated ILD. Furthermore, new antifibrotic drugs approved for the treatment of IPF, including pirfenidone and nintedanib, could also prove to be effective for RA-associated ILD. Studies are ongoing to confirm this hypothesis.


Assuntos
Artrite Reumatoide , Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Artrite Reumatoide/complicações , Artrite Reumatoide/genética , Artrite Reumatoide/terapia , Humanos , Fibrose Pulmonar Idiopática/genética , Fibrose Pulmonar Idiopática/terapia , Doenças Pulmonares Intersticiais/genética , Doenças Pulmonares Intersticiais/terapia , Resultado do Tratamento
14.
BMJ Case Rep ; 12(2)2019 Feb 11.
Artigo em Inglês | MEDLINE | ID: mdl-30755425

RESUMO

Our case series describes two siblings with complex fibrosing lung diseases. The first patient was initially given a diagnosis of sarcoidosis based on imaging and exclusion of alternative diagnoses. A number of years after diagnosis, he had rapid deterioration of his disease and following surgical lung biopsy, his lung fibrosis was re-classified as chronic hypersensitivity pneumonitis (cHP) with a usual interstitial pneumonia pattern. He subsequently underwent successful lung transplantation. The second patient presented with rapidly progressing exertional dyspnoea. His bloods, imaging, bronchoalveolar lavage and histology were discussed at our multidisciplinary team meeting. His histology was most in keeping with subacute on cHP with overlapping imaging features between the two siblings. He was treated accordingly but unfortunately succumbed to his illness shortly after diagnosis. These cases highlight the difficulties differentiating between the various interstitial lung disease (ILD) subtypes and the challenges in management while also increasing awareness of familial ILD.


Assuntos
Alveolite Alérgica Extrínseca/diagnóstico , Fibrose Pulmonar Idiopática/diagnóstico , Pulmão/patologia , Idoso , Alveolite Alérgica Extrínseca/fisiopatologia , Alveolite Alérgica Extrínseca/cirurgia , Doença Crônica , Diagnóstico Diferencial , Erros de Diagnóstico , Dispneia/etiologia , Humanos , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/terapia , Transplante de Pulmão , Masculino , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Tomografia Computadorizada por Raios X
15.
Transl Res ; 209: 1-13, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30768925

RESUMO

Pulmonary fibrosis refers to the development of diffuse parenchymal abnormalities in the lung that cause dyspnea, cough, hypoxemia, and impair gas exchange, ultimately leading to respiratory failure. Though pulmonary fibrosis can be caused by a variety of underlying etiologies, ranging from genetic defects to autoimmune diseases to environmental exposures, once fibrosis develops it is irreversible and most often progressive, such that fibrosis of the lung is one of the leading indications for lung transplantation. This review aims to provide a concise summary of the recent advances in our understanding of the genetics and genomics of pulmonary fibrosis, idiopathic pulmonary fibrosis in particular, and how these recent discoveries may be changing the clinical approach to diagnosing and treating patients with fibrotic interstitial lung disease.


Assuntos
Fibrose Pulmonar Idiopática/terapia , Pesquisa Médica Translacional , Diagnóstico Precoce , Predisposição Genética para Doença , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/etiologia , Fibrose Pulmonar Idiopática/genética , Fenótipo , Telômero/metabolismo
16.
Perm J ; 23: 18-074, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30624191

RESUMO

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a lethal, chronic, degenerative disease most commonly affecting the elderly population. The pathologic finding of this restrictive lung condition is excessive deposition of fibrotic tissue in the alveolar interstitium. Conventional therapies minimally alter the course of disease, and mean survival time from diagnosis is only 2 to 4 years. CASE PRESENTATION: A 65-year-old man, diagnosed with IPF in 2007, was being monitored by a conventional pulmonologist while being treated with weekly acupuncture targeting a Chinese medicine diagnosis of spleen dampness and lung qi weakness and with botanical medicine targeting the stimulation of adrenal dehydroepiandrosterone secretion. He survived for 10 years after diagnosis. DISCUSSION: Studies exist linking dehydroepiandrosterone with IPF disease activity as well as acupuncture with antifibrotic activity. Larger clinical studies are needed to substantiate the link between acupuncture and/or dehydroepiandrosterone-stimulating herbs for the treatment of IPF.


Assuntos
Terapia por Acupuntura/métodos , Desidroepiandrosterona/biossíntese , Fibrose Pulmonar Idiopática/terapia , Fitoterapia/métodos , Idoso , Humanos , Masculino , Índice de Gravidade de Doença
17.
Annu Rev Med ; 70: 211-224, 2019 01 27.
Artigo em Inglês | MEDLINE | ID: mdl-30691371

RESUMO

This is a time of substantial progress in the evaluation and care of patients with idiopathic pulmonary fibrosis (IPF). In addition to the approval and widespread availability of the first IPF-specific therapies, there have been improvements in imaging interpretation and lung biopsy methods to enable more expeditious and more accurate diagnosis. Recent advances in identifying genetic factors that underlie susceptibility to IPF and affect prognosis have raised the possibility of personalized therapeutic approaches in the future. Further, evolving work is elucidating novel mechanisms influencing epithelial, mesenchymal, and inflammatory cell responses during the injury-repair process, thus advancing understanding of disease pathogenesis. As analytic approaches mature, the field is now poised to harness the power of rapidly advancing "omics" technologies to further accelerate progress.


Assuntos
Gerenciamento Clínico , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/terapia , Indóis/administração & dosagem , Piridonas/administração & dosagem , Idoso , Biópsia por Agulha , Doença Crônica , Compreensão , Progressão da Doença , Feminino , Humanos , Fibrose Pulmonar Idiopática/epidemiologia , Imuno-Histoquímica , Transplante de Pulmão/métodos , Masculino , Pessoa de Meia-Idade , Medicina de Precisão/tendências , Prognóstico , Testes de Função Respiratória , Medição de Risco , Índice de Gravidade de Doença , Resultado do Tratamento
18.
Respir Investig ; 57(2): 165-171, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30600175

RESUMO

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronically progressive lung disease with exceptionally poor prognosis. While lung transplantation (LTx) is considered the last-resort therapeutic option, dismal waitlist mortality still hampers the salvage of patients with IPF. Pirfenidone, originally designed for IPF treatment, has increasingly been utilized. This study aimed to evaluate whether Pirfenidone could influence outcomes of patients with IPF on the Japanese LTx waitlist. METHODS: This retrospective single-center cohort study included 25 consecutive patients with IPF who were registered as LTx candidates at our institution between July 1999 and August 2016. Patients with a history of pretransplant Pirfenidone therapy (Pirfenidone group) were compared with those with no history (non-Pirfenidone group). RESULTS: In total, 6 (24%) patients received Pirfenidone as pretransplant therapy for 45.2 (range, 18.6-66.8) months. During the treatment period, the Pirfenidone group achieved a significant reduction in the decline rate of the forced vital capacity (-6.2% vs. -0.3%, p = 0.04) and a lower lung allocation score (31 vs. 41, p = 0.013) compared with the non-Pirfenidone group. The Pirfenidone group exhibited 100% waitlist survival three years after registration that was comparable to other indications, and 66% of the patients were still alive at the time of organ availability. No patient in the Pirfenidone group developed Pirfenidone-related surgical complications postoperatively. CONCLUSIONS: Patients with IPF successfully managed with long-term Pirfenidone therapy achieved favorable outcomes after LTx registration, comparable to other patients with LTx indications. The tolerability to antifibrotic therapy can be a predictor of waitlist survival.


Assuntos
Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/terapia , Transplante de Pulmão , Piridonas/administração & dosagem , Listas de Espera/mortalidade , Adolescente , Adulto , Criança , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento , Adulto Jovem
19.
Lung ; 197(1): 81-88, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30600393

RESUMO

PURPOSE: To assess the prognostic value of the 8-foot-up-and-go test (8-FUGT) in pilot cohort of patients with idiopathic pulmonary fibrosis (IPF). METHODS: Thirty-four patients with IPF (68 ± 8 years) underwent 8-FUGT at baseline and were followed for up to 40 months. Receiver operating characteristics and age-adjusted Cox hazard ratios (HR) were analyzed for 8-FUGT, hospitalizations, and mortality. Correlation coefficients were determined between 8-FUGT and other exercise tests. RESULTS: 8-FUGT ≥ 6.9 s was found to be associated with hospitalization (sensitivity = 77%, specificity = 76%, p = 0.03) and mortality (sensitivity = 91%, specificity = 70% p = 0.008) in patients with IPF. Categorical models demonstrated that 8-FUGT ≥ 6.9 s was associated with 14.1- (p < 0.001) and 55.4-fold (p = 0.001) increased risks for hospitalization and mortality, respectively. In continuous models, for every 1-s slower performance in the 8-FUGT there were 54% [HR = 1.54, 95% CI (1.11-2.15) p = 0.01] increased risk for hospitalization and 94% [HR = 1.94, 95% CI (1.26-2.99) p = 0.003] increased risk for mortality. 8-FUGT was inversely related to 6-min walk distance (r = - 0.61), peak oxygen consumption (r = - 0.58), and peak work rate (r = - 0.72), all p < 0.001. CONCLUSIONS: The 8-FUGT was strongly associated with hospitalizations and mortality in patients with IPF, as well as correlated with established prognostic markers. These novel findings suggest a prognostic value of the 8-FUGT for risk stratification, referral to pulmonary rehabilitation, and considering listing for lung transplantation. 8-FUGT is an inexpensive and practical tool that has prospective for implementation in clinical and research settings in IPF. Future prospective studies should evaluate the effect of changes in 8-FUGT on clinical outcomes. TRIAL REGISTRATION: NCT01499745, Clinicaltrials.gov.


Assuntos
Teste de Esforço/métodos , Avaliação Geriátrica/métodos , Hospitalização , Fibrose Pulmonar Idiopática/diagnóstico , Idoso , Feminino , Humanos , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/terapia , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo
20.
Expert Opin Ther Targets ; 23(1): 69-81, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30468628

RESUMO

INTRODUCTION: Fibrosis is an irreversible pathological endpoint in many chronic diseases, including pulmonary fibrosis. Idiopathic pulmonary fibrosis (IPF) is a progressive and often fatal condition characterized by (myo)fibroblast proliferation and transformation in the lung, expansion of the extracellular matrix, and extensive remodeling of the lung parenchyma. Recent evidence indicates that IPF prevalence and mortality rates are growing in the United States and elsewhere. Despite decades of research on the pathogenic mechanisms of pulmonary fibrosis, few therapeutics have succeeded in the clinic, and they have failed to improve IPF patient survival. Areas covered: Based on a literature search and our own results, we discuss the key cellular and molecular responses that contribute to (myo)fibroblast actions and pulmonary fibrosis pathogenesis; this includes signaling pathways in various cells that aberrantly and persistently activate (myo)fibroblasts in fibrotic lesions and promote scar tissue formation in the lung. Expert opinion: Lessons learned from recent failures and successes with new therapeutics point toward approaches that can target multiple pro-fibrotic processes in IPF. Advances in preclinical modeling and single-cell genomics will also accelerate novel discoveries for effective treatment of IPF.


Assuntos
Fibrose Pulmonar Idiopática/terapia , Pulmão/fisiopatologia , Terapia de Alvo Molecular , Animais , Proliferação de Células/fisiologia , Progressão da Doença , Matriz Extracelular/metabolismo , Fibroblastos/citologia , Humanos , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/fisiopatologia , Miofibroblastos/citologia , Sobrevida
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