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1.
Pneumologie ; 74(3): 159-172, 2020 Mar.
Artigo em Alemão | MEDLINE | ID: mdl-32052391

RESUMO

Exposure to granular or fibrous inorganic dusts of the alveolar dust fraction initiates inflammatory and fibrotic lung processes. Furthermore, such exposures are associated with an increased risk for lung cancer. By taking a detailed occupational history into consideration the diagnosis of relatively frequent pneumoconioses such as silicosis or asbestosis with dominating pictures, i. e. with its predominant rounded or irregular opacities located especially in the upper and lower lung fields, respectively, is mostly not difficult. However, rarely exposure to a single agent exists. Rather, mixed dust exposures occurring at the same time or in the follow-up have to be taken into consideration, e. g. quartz and carbon in hard coal mines, quartz, asbestos, various other components of cement and concrete dusts in the construction industry. It is also important that during the working life, changes of working processes and used raw materials as well as changes of jobs are associated with variations of type and intensity of exposures. This heterogenicity of exposures and of the associated intrapulmonary depositions result in variations of the pulmonary structural changes, i. e. more or less modifications of the pictures of pneumoconioses as described being typical in textbooks. This is associated with diagnostic difficulties, especially with regard to the differential diagnosis of idiopathic interstitial lung diseases. There is also evidence for genetic influence on disease susceptibility and on the degree of pathohistological changes.This publication includes 5 case reports; all of them were initially diagnosed as idiopathic pulmonary fibrosis, but a detailed workup of the author, mostly during social court litigations, showed that mixed dust pneumoconioses were the most likely diagnoses.


Assuntos
Pulmão/fisiopatologia , Pneumoconiose/diagnóstico , Adulto , Diagnóstico Diferencial , Poeira , Humanos , Fibrose Pulmonar Idiopática/diagnóstico
2.
Pathologe ; 41(1): 46-51, 2020 Feb.
Artigo em Alemão | MEDLINE | ID: mdl-31993696

RESUMO

Idiopathic pulmonary fibrosis (IPF) plays a special role within the group of interstitial lung diseases (ILDs) due to its inexorable progression and its specific medical treatment. With a median survival of only 2-3 years from the time of diagnosis, the prognosis is worse than many carcinomas.In contrast to other ILDs, IPF does not respond to anti-inflammatory treatment with corticosteroids but rather demands a specific medical therapy. Even though this cannot cure the disease, it can prolong survival. Lung transplantation is the only cure for progressive lung fibrosis. The clinical course is individual and difficult to predict. Acute exacerbations accelerate the clinical course and lead to high mortality.The underlying pathomechanisms of IPF, with its complex immunological and inflammatory processes and external impacts, have been the focus of recent research. Lifestyle and environmental influences are held responsible for much of its natural history. Smoking, pneumotoxic medications, and inhalation of dusts are well-known risk factors. Likewise, genetic and hereditary factors play a crucial role.This short review focuses on the peculiarities of IPF within the group of ILDs, especially in relation to its underlying mechanisms and clinical progression.


Assuntos
Fibrose Pulmonar Idiopática , Progressão da Doença , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/epidemiologia , Fibrose Pulmonar Idiopática/etiologia , Fibrose Pulmonar Idiopática/patologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/patologia , Prognóstico , Fatores de Risco
3.
Vet Clin North Am Small Anim Pract ; 50(2): 431-446, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31866093

RESUMO

Canine idiopathic pulmonary fibrosis (CIPF) is a chronic, progressive, interstitial lung disease (ILD) affecting older West Highland white terriers (WHWTs). According to one classification, CIPF is a familial fibrotic ILD in the group of idiopathic interstitial pneumonias. Etiology is unknown but likely arises from interplay between genetic and environmental factors. CIPF shares features with human idiopathic pulmonary fibrosis and human nonspecific interstitial pneumonia. This article describes clinical signs, findings in physical examination, arterial oxygenation, diagnostic imaging, bronchoscopy, bronchoalveolar lavage, histopathology, disease course, and outcome of WHWTs with CIPF; compares canine and human diseases; summarizes biomarker research; and gives an overview of potential treatment.


Assuntos
Doenças do Cão , Fibrose Pulmonar Idiopática/veterinária , Animais , Doenças do Cão/diagnóstico , Doenças do Cão/etiologia , Doenças do Cão/fisiopatologia , Doenças do Cão/terapia , Cães , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/etiologia , Fibrose Pulmonar Idiopática/fisiopatologia , Prognóstico
4.
Georgian Med News ; (294): 98-103, 2019 Sep.
Artigo em Russo | MEDLINE | ID: mdl-31687958

RESUMO

The article reveals the modern aspects of IPF pathogenesis in with an emphasis on the main proposed prognostic biomarkers. IPF remains the leader among diseases with unknown etiology, the diagnosis and management of which are not very successful, despite the obvious progress in molecular medicine. There is presented analysis of the significance of IPF potential biomarkers and their concentrations in the blood and bronchoalveolar lavage fluids (BAL): endothelin-1, CC-chemokine ligand 18, interleukin-1, surfactant protein SP-D in the review. The role of their changing levels in the blood and BAL for assessing the course of the IPF and its prognosis, as well as the prevailing importance of the polymorphism of the genes encoding them, is shown. Obviously, the progressive accumulation of fibroblast-myofibroblast cells in the lungs IPF patients worsens the prognosis of disease, forms its own environment with a set of cytokines, growth factors, collagen, fibronectin in the extracellular matrix of fibrous lungs. The insufficient amount of studies in the face of the rarity of the disease leaves a lot of controversial issues for solution in the future. Obviously, to assess the prognosis of IPF mortality, it is necessary to include a very large number of patients, to extend the observation period, which increases their cost and reduces the opportunities and desire of pharmaceutical companies to participate in these studies.


Assuntos
Biomarcadores/sangue , Líquido da Lavagem Broncoalveolar/química , Fibrose Pulmonar Idiopática/diagnóstico , Pulmão/metabolismo , Biomarcadores/análise , Quimiocinas CC , Endotelina-1 , Humanos , Fibrose Pulmonar Idiopática/sangue , Fibrose Pulmonar Idiopática/mortalidade , Interleucina-1 , Pulmão/fisiopatologia , Prognóstico , Proteína D Associada a Surfactante Pulmonar/análise , Proteína D Associada a Surfactante Pulmonar/sangue , Surfactantes Pulmonares/análise , Surfactantes Pulmonares/sangue
5.
Nat Commun ; 10(1): 4673, 2019 10 14.
Artigo em Inglês | MEDLINE | ID: mdl-31611594

RESUMO

Advances in precision molecular imaging promise to transform our ability to detect, diagnose and treat disease. Here, we describe the engineering and validation of a new cystine knot peptide (knottin) that selectively recognizes human integrin αvß6 with single-digit nanomolar affinity. We solve its 3D structure by NMR and x-ray crystallography and validate leads with 3 different radiolabels in pre-clinical models of cancer. We evaluate the lead tracer's safety, biodistribution and pharmacokinetics in healthy human volunteers, and show its ability to detect multiple cancers (pancreatic, cervical and lung) in patients at two study locations. Additionally, we demonstrate that the knottin PET tracers can also detect fibrotic lung disease in idiopathic pulmonary fibrosis patients. Our results indicate that these cystine knot PET tracers may have potential utility in multiple disease states that are associated with upregulation of integrin αvß6.


Assuntos
Antígenos de Neoplasias/metabolismo , Fibrose Pulmonar Idiopática/diagnóstico , Integrinas/metabolismo , Neoplasias/diagnóstico , Cristalografia por Raios X , Voluntários Saudáveis , Humanos , Imagem por Ressonância Magnética , Tomografia por Emissão de Pósitrons
6.
BMJ Case Rep ; 12(9)2019 Sep 06.
Artigo em Inglês | MEDLINE | ID: mdl-31494587

RESUMO

Interstitial pneumonia with autoimmune features (IPAF) is a recently proposed terminology for interstitial lung disease (ILD) with evidence of autoimmunity that does not meet the criteria for a defined connective tissue disease (CTD). Although ILD is well recognised in patients with established CTD, it is rarely the sole presenting feature of CTD. We report a case of 22-year-old male patient, who presented with progressive shortness of breath for 2 months and had features suggestive of platypnea-orthodeoxia syndrome (POS). Imaging revealed ILD with usual interstitial pneumonia pattern. Patient had features of autoimmune disorder but did not fulfil the criteria for any CTD and hence was labelled as IPAF. His POS was attributed predominantly to the lower lobe disease. The patient responded well to immunosuppressive treatment. A systematic review of literature of all cases with POS due to pulmonary parenchymal involvement has also been done.


Assuntos
Doenças Autoimunes/diagnóstico , Fibrose Pulmonar Idiopática/diagnóstico , Imunossupressores/uso terapêutico , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/imunologia , Doenças Autoimunes/fisiopatologia , Dispneia/imunologia , Humanos , Fibrose Pulmonar Idiopática/tratamento farmacológico , Fibrose Pulmonar Idiopática/imunologia , Fibrose Pulmonar Idiopática/fisiopatologia , Masculino , Síndrome , Resultado do Tratamento , Adulto Jovem
7.
Zhonghua Jie He He Hu Xi Za Zhi ; 42(9): 700-704, 2019 Sep 12.
Artigo em Chinês | MEDLINE | ID: mdl-31484245

RESUMO

Objective: To explore the effect of pirfenidone in fibrotic interstitial pneumonia with autoimmune features (IPAF) after treatment with corticosteroids and immunosuppressants. Methods: We conducted a retrospective analysis of 2 adult patients with IPAF in the Peking Union Medical College Hospital. As their fibrotic interstitial lung disease failed to improve with further treatment with corticosteroids and immunosuppressants, they were treated with pirfenidone based on corticosteroids and immunosuppressants. Their clinical, chest radiological data and prognosis were collected and relevant literatures were reviewed. Results: One patient was a 43 year old female, the other was a 53 year old male. IPAF was diagnosed with their classic clinical, serological and radiological features. They were partially responded to corticosteroids and immunosuppressants at the initial period. Pirfenidone was suggested for them as their lung fibrosis was not improved further with immunosuppressive therapy. After 4-5 months treatment with pirfenidone, based on corticosteroids and immunosuppressant administration, their clinical and radiological manifestations improved significantly. Conclusions: Pirfenidone might be a good add-on choice for fibrotic IPAF when the disease did not respond well to corticosteroids and immunosuppressants.


Assuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Doenças Autoimunes/diagnóstico , Fibrose Pulmonar Idiopática/tratamento farmacológico , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Piridonas/uso terapêutico , Corticosteroides/uso terapêutico , Adulto , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento
8.
Respir Res ; 20(1): 182, 2019 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-31409344

RESUMO

Previous studies have shown that the co-existence of bone marrow failure and pulmonary fibrosis in a single patient or in a family is suggestive of telomere related genes (TRG) germline mutations. This study presents the genetic background, clinical characteristics, and outcome of a group of five Greek patients co-affected with IPF and MDS. Four out of five patients developed an IPF acute exacerbation that was not reversible. We failed to detect any mutation in the TERT, TERC, DKC1, TINF2, RTEL1, PARN, NAF1, ACD, NHP2 and NOP10 genes in any patient. Moreover, telomere length was normal in the two patients tested. This could suggest that although the co-occurence of IPF and MDS are suggestive of TRG mutation in patients < 65 years old, in the elderly it may occur without germline mutations and could negatively affect prognosis. Physicians should be aware for possible IPF deterioration and therapeutic options for MDS should be wisely considered.


Assuntos
Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/diagnóstico , Síndromes Mielodisplásicas/complicações , Síndromes Mielodisplásicas/diagnóstico , Exacerbação dos Sintomas , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Fibrose Pulmonar Idiopática/genética , Masculino , Síndromes Mielodisplásicas/genética
9.
BMC Pulm Med ; 19(1): 149, 2019 Aug 14.
Artigo em Inglês | MEDLINE | ID: mdl-31412851

RESUMO

BACKGROUND: Lung cancer is a common comorbidity of idiopathic pulmonary fibrosis (IPF) and has poor outcomes. The incidence and clinical factors related to development of lung cancer in idiopathic pulmonary fibrosis (IPF) are unclear. The aim of this study was to elucidate the cumulative incidence, risk factors, and clinical characteristics of lung cancer in IPF. METHODS: In this retrospective study, we analyzed clinical data for 938 patients who were diagnosed with IPF without lung cancer between 1998 and 2013. Demographic, physiologic, radiographic, and histologic characteristics were reviewed. Cumulative incidence of lung cancer and survival were estimated by the Kaplan-Meier method. Risk factors of lung cancer development were determined by Cox proportional hazard analysis. RESULTS: Among 938 IPF patients without lung cancer at initial diagnosis, lung cancer developed in 135 (14.5%) during the follow-up period. The cumulative incidences of lung cancer were 1.1% at 1 year, 8.7% at 3, 15.9% at 5, and 31.1% at 10 years. Risk factors of lung cancer were male gender, current smoking at IPF diagnosis, and rapid annual decline of 10% or more in forced vital capacity (FVC). Patients who developed lung cancer were mostly elderly men with smoking history. Squamous cell carcinoma followed by adenocarcinoma was the most common histologic type. Lung cancer was frequently located in areas abutting or within fibrosis. Survival was significantly worse in patients with lung cancer compared to patients with IPF alone. CONCLUSION: Lung cancer frequently developed in patients with IPF and was common in current-smoking men with rapid decline of FVC.


Assuntos
Adenocarcinoma/mortalidade , Carcinoma de Células Escamosas/mortalidade , Fibrose Pulmonar Idiopática/complicações , Neoplasias Pulmonares/mortalidade , Adenocarcinoma/fisiopatologia , Idoso , Carcinoma de Células Escamosas/fisiopatologia , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Incidência , Neoplasias Pulmonares/fisiopatologia , Masculino , Pessoa de Meia-Idade , República da Coreia/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Análise de Sobrevida , Centros de Atenção Terciária , Tomografia Computadorizada por Raios X , Capacidade Vital
10.
Respir Res ; 20(1): 148, 2019 Jul 12.
Artigo em Inglês | MEDLINE | ID: mdl-31299951

RESUMO

Idiopathic pulmonary fibrosis (IPF) is characterised by excessive extracellular matrix (ECM) deposition and remodelling. Measuring this activity provides an opportunity to develop tools capable of identifying individuals at-risk of progression. Longitudinal change in markers of ECM synthesis was assessed in 145 newly-diagnosed individuals with IPF.Serum levels of collagen synthesis neoepitopes, PRO-C3 and PRO-C6 (collagen type 3 and 6), were elevated in IPF compared with controls at baseline, and progressive disease versus stable disease during follow up, (PRO-C3 p < 0.001; PRO-C6 p = 0.029). Assessment of rate of change in neoepitope levels from baseline to 3 months (defined as 'slope to month 3': HIGH slope, slope > 0 vs. LOW slope, slope < =0) demonstrated no relationship with mortality for these markers (PRO-C3 (HR 1.62, p = 0.080); PINP (HR 0.76, p = 0.309); PRO-C6 (HR 1.14, p = 0.628)). As previously reported, rising concentrations of collagen degradation markers C1M, C3M, C6M and CRPM were associated with an increased risk of overall mortality (HR = 1.84, CI 1.03-3.27, p = 0.038, HR = 2.44, CI 1.39-4.31, p = 0.002; HR = 2.19, CI 1.25-3.82, p = 0.006; HR = 2.13 CI 1.21-3.75, p = 0.009 respectively).Elevated levels of PRO-C3 and PRO-C6 associate with IPF disease progression. Collagen synthesis and degradation biomarkers have the potential to enhance clinical trials in IPF and may inform prognostic assessment and therapeutic decision making in the clinic.


Assuntos
Colágeno/biossíntese , Colágeno/sangue , Progressão da Doença , Fibrose Pulmonar Idiopática/sangue , Fibrose Pulmonar Idiopática/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/sangue , Estudos de Coortes , Humanos , Estudos Longitudinais , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Biossíntese de Proteínas/fisiologia
11.
Eur Respir Rev ; 28(152)2019 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-31285290

RESUMO

Idiopathic pulmonary fibrosis (IPF) arises in genetically susceptible individuals as a result of an aberrant wound-healing response following repetitive alveolar injury. The clinical course of the disease remains both variable and unpredictable with periods of more rapid decline, termed acute exacerbation of IPF (AE-IPF), often punctuating the disease trajectory. Exacerbations carry a significant morbidity and mortality, and their exact pathogenesis remains unclear. Given the emerging evidence that disruption and alteration in the lung microbiome plays a role in the pathogenesis and progression of IPF, this review discusses the current knowledge of the contribution of infection and the respiratory microbiome to AE-IPF.


Assuntos
Bactérias/patogenicidade , Fibrose Pulmonar Idiopática/microbiologia , Pulmão/microbiologia , Microbiota , Infecções Respiratórias/microbiologia , Animais , Progressão da Doença , Disbiose , Interações Hospedeiro-Patógeno , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Infecções Respiratórias/diagnóstico
12.
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
13.
BMC Pulm Med ; 19(1): 130, 2019 Jul 18.
Artigo em Inglês | MEDLINE | ID: mdl-31319833

RESUMO

BACKGROUND: The aim of this study was to analyze the relative frequency, clinical characteristics, disease onset and progression in f-IPF vs. sporadic IPF (s-IPF). METHODS: Familial IPF index patients and their family members were recruited into the European IPF registry/biobank (eurIPFreg) at the Universities of Giessen and Marburg (UGMLC). Initially, we employed wide range criteria of f-IPF (e.g. relatives who presumably died of some kind of parenchymal lung disease). After narrowing down the search to occurrence of idiopathic interstitial pneumonia (IIP) in at least one first grade relative, 28 index patients were finally identified, prospectively interviewed and examined. Their family members were phenotyped with establishment of pedigree charts. RESULTS: Within the 28 IPF families, overall 79 patients with f-IPF were identified. In the same observation period, 286 f-IIP and s-IIP patients were recruited into the eurIPFreg at our UGMLC sites, corresponding to a familial versus s-IPF of 9.8%. The both groups showed no difference in demographics (61 vs. 79% males), smoking history, and exposure to any environmental triggers known to cause lung fibrosis. The f-IPF group differed by an earlier age at the onset of the disease (55.4 vs. 63.2 years; p < 0.001). On average, the f-IPF patients presented a significantly milder extent of functional impairment at the time point of inclusion vs. the s-IPF group (FVC 75% pred. vs. FVC 62% pred., p = 0.011). In contrast, the decline in FVC was found to be faster in the f-IPF vs. the s-IPF group (4.94% decline in 6 months in f-IPF vs. 2.48% in s-IPF, p = 0.12). The average age of death in f-IPF group was 67 years vs. 71.8 years in s-IPF group (p = 0.059). The f-IIP group displayed diverse inheritance patterns, mostly autosomal-dominant with variable penetrance. In the f-IPF, the younger generations showed a tendency for earlier manifestation of IPF vs. the older generation (58 vs. 66 years, p = 0.013). CONCLUSIONS: The 28 f-IPF index patients presented an earlier onset and more aggressive natural course of the disease. The disease seems to affect consecutive generations at a younger age. TRIAL REGISTRATION: Nr. NCT02951416 http://www.www.clinicaltrials.gov.


Assuntos
Pneumonias Intersticiais Idiopáticas/diagnóstico , Fibrose Pulmonar Idiopática/diagnóstico , Sistema de Registros , Idoso , Estudos Transversais , Progressão da Doença , Europa (Continente)/epidemiologia , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/mortalidade , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/fisiopatologia , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Taxa de Sobrevida
14.
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
15.
BMC Pulm Med ; 19(1): 113, 2019 Jun 25.
Artigo em Inglês | MEDLINE | ID: mdl-31238929

RESUMO

BACKGROUND AND OBJECTIVE: Here, we present real-world data on the incidence and risk factors of acute exacerbation (AE) in patients with chronic fibrotic interstitial pneumonia (CFIP) treated with antifibrotic agents, which has been previously poorly documented. METHODS: We retrospectively examined clinical characteristics, incidence and risk factors of AE in a cohort of 100 patients with CFIP (n = 75, idiopathic pulmonary fibrosis [IPF]; n = 25, other conditions), all of whom received antifibrotic agents in a real-world setting. RESULTS: The median follow-up was 17.4 months (interquartile range [IQR], 6.6 to 26.7 months). During the follow-up periods, 21 patients experienced AE after starting antifibrotic agents. The estimated 1-, 2-, and 3-year AE incidence rates were 11.4% (95% confidence interval [95%CI], 6.2-20.3%), 32% (95%CI, 20.7-47.4%), and 36.3% (95%CI 23.5-53.1%), respectively. Decreased baseline lung function (forced vital capacity and carbon monoxide diffusing capacity of the lung), existence of pulmonary hypertension estimated from an echocardiogram, higher Interstitial Lung Disease-Gender, Age, and Physiology (ILD-GAP) score, supplementary oxygen, and concomitant corticosteroid and proton-pump inhibitor (PPI) use upon starting the antifibrotic agent were risk factors of AE. Concomitant corticosteroid and PPI use and corticosteroid dose were risk factor of AE in a multivariate Cox regression hazard model adjusting for ILD-GAP score. CONCLUSION: AE of CFIP is more common in patients with physiologically and functionally advanced disease under antifibrotic agents. Prudent use of corticosteroids and PPIs when initiating antifibrotic agents may be recommended. Further studies are warranted.


Assuntos
Progressão da Doença , Fibrose Pulmonar Idiopática/diagnóstico , Pulmão/fisiopatologia , Capacidade Vital , Doença Aguda , Corticosteroides/uso terapêutico , Idoso , Feminino , Humanos , Fibrose Pulmonar Idiopática/tratamento farmacológico , Fibrose Pulmonar Idiopática/fisiopatologia , Incidência , Pulmão/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Prognóstico , Inibidores da Bomba de Prótons/uso terapêutico , Estudos Retrospectivos , Fatores de Risco , Tomografia Computadorizada por Raios X
16.
Respir Res ; 20(1): 127, 2019 Jun 17.
Artigo em Inglês | MEDLINE | ID: mdl-31208406

RESUMO

BACKGROUND: Little is known on the characteristics of patients diagnosed with idiopathic pulmonary fibrosis (IPF) in Spain. We aimed to characterize the demographic and clinical profile of IPF patients included in the IPF National Registry of the Spanish Respiratory Society (SEPAR). METHODS: This is a prospective, observational, multicentre and nationwide study that involved 608 IPF patients included in the SEPAR IPF Registry up to June 27th, 2017, and who received any treatment for their disease. IPF patients were predominantly males, ex-smokers, and aged in their 70s, similar to other registries. RESULTS: Upon inclusion, mean ± SD predicted forced vital capacity was 77.6% ± 19.4, diffusing capacity for carbon monoxide was 48.5% ± 17.7, and the 6-min walk distance was 423.5 m ± 110.4. The diagnosis was mainly established on results from the high-resolution computed tomography in the proper clinical context (55.0% of patients), while 21.2% of patients required invasive procedures (surgical lung biopsy) for definitive diagnosis. Anti-fibrotic treatment was prescribed in 69.4% of cases, 51.5% pirfenidone and 17.9% nintedanib, overall with a good safety profile. CONCLUSIONS: The SEPAR IPF Registry should help to further characterize current characteristics and future trends of IPF patients in Spain and compare/pool them with other registries and cohorts.


Assuntos
Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/epidemiologia , Sistema de Registros , Sociedades Médicas , Idoso , Idoso de 80 Anos ou mais , Anti-Inflamatórios não Esteroides/uso terapêutico , Feminino , Humanos , Fibrose Pulmonar Idiopática/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Piridonas/uso terapêutico , Estudos Retrospectivos , Espanha/epidemiologia
18.
Vet Radiol Ultrasound ; 60(5): 525-532, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31172636

RESUMO

Canine idiopathic pulmonary fibrosis is a chronic, progressive interstitial lung disease particularly prevalent in West Highland White Terriers. In the present prospective pilot study, we evaluated the feasibility of modified VetMousetrap™ device in high resolution CT to detect idiopathic pulmonary fibrosis in West Highland White Terriers. Twelve awake West Highland White Terriers with canine idiopathic pulmonary fibrosis and 24 clinically healthy West Highland White Terriers were scanned using a helical dual slice scanner utilizing VetMousetrap™ device without or with minimal chemical restraint with butorphanol. Three evaluators blindly assessed the images for image quality and the presence of canine idiopathic pulmonary fibrosis related imaging findings such as ground glass opacity and reticular opacities. Additionally, the attenuation of the lung was quantified with ImageJ software using histogram analysis of density over the lung fields. Computed tomography was successfully completed and motion artifact ranked in statistical analysis barely noticeable to mild in all dogs. The agreement between imaging findings and clinical status was very good with overall κ value 0.91 and percentage of agreement of 94%. There was also very good intraobserver (κrange = 0.79-0.91) and interobserver agreement (κ = 0.94). Moderate to severe ground glass opacity was present in all affected dogs. In the ImageJ analysis, a significant difference in lung attenuation between the study groups was observed. We conclude that modified VetMousetrap™ device is applicable in diagnosing canine idiopathic pulmonary fibrosis in awake West Highland White Terriers avoiding anesthetic risk in these often severely hypoxic patients.


Assuntos
Doenças do Cão/diagnóstico , Fibrose Pulmonar Idiopática/veterinária , Tomografia Computadorizada por Raios X/veterinária , Animais , Doenças do Cão/diagnóstico por imagem , Cães , Feminino , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Masculino , Projetos Piloto , Estudos Prospectivos , Tomografia Computadorizada por Raios X/métodos , Vigília
19.
Med Sci Monit ; 25: 4193-4201, 2019 Jun 05.
Artigo em Inglês | MEDLINE | ID: mdl-31166938

RESUMO

BACKGROUND The prognosis of idiopathic pulmonary fibrosis (IPF) is the worst among all interstitial lung diseases, and is related to the disease itself. Comorbidities or complications can worsen IPF. We assessed the effect of comorbidities on the survival of IPF patients. A retrospective review of patients with IPF was completed. MATERIAL AND METHODS Information on demographic features, clinical examination, and comorbidities at baseline were obtained. Then, median, 1-year, and 5-year survival was calculated. A total of 380 patients with IPF admitted to Beijing Chao-Yang Hospital from 1 April 2002 to 31 March 2015 were followed up until December 2016. RESULTS Of these 380 patients, 71.9% died during the study period. Median survival was 2.25 years and overall 5-year survival was 28.5%. Also, 86.3% of patients were males. A total of 248 cases underwent lung function tests, and 178 patients underwent bronchoalveolar lavage (BAL). Multivariate analyses showed that forced expiratory volume in 1 second/forced vital capacity (FVC), diffusing capacity of the lungs for carbon monoxide percent predicted, FVC% predicted, the number of macrophages, neutrophils, and lymphocytes in BAL fluid, pulmonary hypertension, hypoxemia, and hydropower disorder were independent prognostic indicators of IPF, GAP gender (G), age (A), and 2 pulmonary physiological parameters (P) model can help to predict prognosis of IPF. CONCLUSIONS Spirometry, GAP model, and BAL are helpful to forecast the prognosis of IPF. IPF patients also suffering from pulmonary arterial hypertension, hypoxemia, and hydropower disorder have a poor prognosis.


Assuntos
Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Lavagem Broncoalveolar , Líquido da Lavagem Broncoalveolar , China , Comorbidade , Feminino , Volume Expiratório Forçado , Humanos , Hipertensão Pulmonar/patologia , Fibrose Pulmonar Idiopática/diagnóstico , Pulmão/patologia , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Testes de Função Respiratória , Estudos Retrospectivos , Análise de Sobrevida , Volume de Ventilação Pulmonar , Capacidade Vital
20.
Respir Res ; 20(1): 105, 2019 May 30.
Artigo em Inglês | MEDLINE | ID: mdl-31142314

RESUMO

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a variable clinical course and high mortality. We used data from a large national US registry of patients with IPF to investigate relationships between patient characteristics, including markers of disease severity, and mortality. METHODS: The analysis cohort comprised patients enrolled in the IPF-PRO Registry from its inception on 5 June 2014 to 26 October 2017. The primary criterion for inclusion in this registry is that patients must be diagnosed or confirmed with IPF at the enrolling centre within 6 months. Associations between patient characteristics and markers of disease severity at enrolment and mortality outcomes were investigated using univariable, multivariable and adjustment models. RESULTS: Among 662 patients enrolled, 111 patients died or had a lung transplant over a follow-up period of 30 months. The probability of being free of both events at month 30 was 50.6% (95% CI: 40.0, 60.2). When patient characteristics and markers of disease severity were jointly examined in a multivariable analysis, oxygen use at rest (hazard ratio [HR] 2.44 [95% CI: 1.45, 4.10]), lower forced vital capacity (FVC) % predicted (HR 1.28 [95% CI: 1.10, 1.49] per 10% decrease) and diffusion capacity for carbon monoxide (DLco) % predicted (HR 1.25 [95% CI: 1.04, 1.51] per 10% decrease) were significantly associated with increased risk of death or lung transplant. The risk of death or lung transplant increased with increasing age in patients ≥62 years old (HR 1.18 [95% CI: 0.99, 1.40] per 5-year increase), and decreased with increasing age in patients <62 years old (HR 0.60 [95% CI: 0.39, 0.92] per 5-year increase). CONCLUSIONS: In an observational US registry of patients with IPF, oxygen use at rest, lower FVC % predicted, and lower DLco % predicted were associated with risk of death or lung transplant. An audio podcast of the lead author discussing these data can be downloaded from: http://www.usscicomms.com/respiratory/snyder/IPF-PROsurvival1/ . TRIAL REGISTRATION: ClinicalTrials.gov number: NCT01915511 .


Assuntos
Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/cirurgia , Transplante de Pulmão/mortalidade , Sistema de Registros , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Seguimentos , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Transplante de Pulmão/tendências , Masculino , Pessoa de Meia-Idade , Mortalidade/tendências , Valor Preditivo dos Testes
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