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1.
J Digit Imaging ; 33(3): 797-813, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32253657

RESUMO

Radiology teaching file repositories contain a large amount of information about patient health and radiologist interpretation of medical findings. Although valuable for radiology education, the use of teaching file repositories has been hindered by the ability to perform advanced searches on these repositories given the unstructured format of the data and the sparseness of the different repositories. Our term coverage analysis of two major medical ontologies, Radiology Lexicon (RadLex) and Unified Medical Language System (UMLS) Systematized Nomenclature of Medicine Clinical Terms (SNOMED CT), and two teaching file repositories, Medical Imaging Resource Community (MIRC) and MyPacs, showed that both ontologies combined cover 56.3% of terms in the MIRC and only 17.9% of terms in MyPacs. Furthermore, the overlap between the two ontologies (i.e., terms included by both the RadLex and UMLS SNOMED CT) was a mere 5.6% for the MIRC and 2% for the RadLex. Clustering the content of the teaching file repositories showed that they focus on different diagnostic areas within radiology. The MIRC teaching file covers mostly pediatric cases; a few cases are female patients with heart-, chest-, and bone-related diseases. The MyPacs contains a range of different diseases with no focus on a particular disease category, gender, or age group. MyPacs also provides a wide variety of cases related to the neck, face, heart, chest, and breast. These findings provide valuable insights on what new cases should be added or how existent cases may be integrated to provide more comprehensive data repositories. Similarly, the low-term coverage by the ontologies shows the need to expand ontologies with new terminology such as new terms learned from these teaching file repositories and validated by experts. While our methodology to organize and index data using clustering approaches and medical ontologies is applied to teaching file repositories, it can be applied to any other medical clinical data.

2.
Respir Med ; 146: 23-27, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30665514

RESUMO

INTRODUCTION: Idiopathic interstitial pneumonias (IIP) are diffuse lung diseases whose cause is unknown and often present with features of autoimmunity despite not meeting criteria for a connective tissue disease (CTD). Recent studies suggest that anti-RNA binding protein (anti-RBP) antibodies, which include anti-SSA, anti-SSB, anti-Sm, and anti-RNP, play a role in the loss of immune tolerance and severity of pulmonary hypertension (PH) in CTDs. We hypothesized that anti-RBP positive (RBP+) subjects would have worse measures of lung function, radiographic findings, PH, and survival than anti-RBP negative (RBP-) subjects. METHODS: Subjects with both IIP and serologies for review were identified retrospectively and stratified based on anti-RBP antibody seropositivity. Baseline cohort characteristics, pulmonary function tests (PFT), ambulatory oxygen requirement, radiographic characteristics, markers of PH, and transplant-free survival were compared between anti-RBP positive and negative groups. RESULTS: Five hundred twenty patients with IIP were identified, of which ten percent (n = 53) were anti-RBP positive. RBP+ as compared to RBP- subjects had significantly worse PFTs as indicated by FEV1 (59.6 vs. 64.9, p = 0.046) and FVC (71.6 vs. 78.8, p = 0.018). There was a higher prevalence of radiographic honeycombing (49.1% vs. 38.3%, p = 0.006) and emphysema (22.6% vs. 5.1%, p < 0.001) in the RBP+ group despite no difference in smoking history. The Pulmonary Artery-Aorta ratio was also larger in the RBP+ group (0.93 vs. 0.88, p = 0.040). There was no difference in transplant-free survival between groups (log rank = 0.912). CONCLUSION: Anti-RBP+ IIP patients may have worse lung function, increased chest radiographic abnormalities, and PH compared with those without these antibodies.

3.
Ann Am Thorac Soc ; 16(5): 580-588, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30653927

RESUMO

Rationale: Honeycombing on chest computed tomography (CT) has been described in diverse forms of interstitial lung disease (ILD); however, its prevalence and association with mortality across the spectrum of ILD remains unclear. Objective: To determine the prevalence and prognostic value of CT honeycombing and characterize associated mortality patterns across diverse ILD subtypes in a multicenter cohort. Methods: This was an observational cohort study of adult participants with multidisciplinary or adjudicated ILD diagnosis and documentation of chest CT imaging at index diagnosis across five U.S. hospitals (one tertiary and four nontertiary medical centers). Participants were stratified based on presence or absence of CT honeycombing. Vital status was determined from review of medical records and social security death index. Transplant-free survival was analyzed using univariate and multivariable Cox regression. Results: The sample comprised 1,330 participants (mean age, 66.8 yr; 50% men) with 4,831 person-years of follow-up. The prevalences of CT honeycombing were 42.0%, 41.9%, 37.6%, and 28.6% in chronic hypersensitivity pneumonitis, connective tissue disease-related ILD (CTD-ILD), idiopathic pulmonary fibrosis (IPF), and unclassifiable/other ILDs, respectively. Among those with CT honeycombing, cumulative mortality hazards were similar across ILD subtypes, except for CTD-ILD, which had a lower mortality hazard. Overall, the mean survival time was shorter among those with CT honeycombing (107 mo; 95% confidence interval [CI], 92-122 mo) than those without CT honeycombing (161 mo; 95% CI, 147-174 mo). CT honeycombing was associated with an increased mortality rate (hazard ratio, 1.72; 95% CI, 1.38-2.14) even after adjustment for center, sex, age, forced vital capacity, diffusing capacity, ILD subtype, and use of immunosuppressive therapy (hazard ratio, 1.62; 95% CI, 1.29-2.02). CT honeycombing was associated with an increased mortality rate within non-IPF ILD subgroups (chronic hypersensitivity pneumonitis, CTD-ILD, and unclassifiable/other ILD). In IPF, however, mortality rates were similar between those with and without CT honeycombing. Conclusions: CT honeycombing is prevalent in diverse forms of ILD and uniquely identifies a progressive fibrotic ILD phenotype with a high mortality rate similar to IPF. CT honeycombing did not confer additional risk in IPF, which is already known to be a progressive fibrotic ILD phenotype regardless of the presence of CT honeycombing.

4.
Am J Respir Crit Care Med ; 199(6): 747-759, 2019 03 15.
Artigo em Inglês | MEDLINE | ID: mdl-30216085

RESUMO

RATIONALE: Mediastinal lymph node (MLN) enlargement on chest computed tomography (CT) is prevalent in patients with interstitial lung disease (ILD) and may reflect immunologic activation and subsequent cytokine-mediated immune cell trafficking. OBJECTIVES: We aimed to determine whether MLN enlargement on chest CT predicts clinical outcomes and circulating cytokine levels in ILD. METHODS: MLN measurements were obtained from chest CT scans of patients with ILD at baseline evaluation over a 10-year period. Patients with sarcoidosis and drug toxicity-related ILD were excluded. MLN diameter and location were assessed. Plasma cytokine levels were analyzed in a subset of patients. The primary outcome was transplant-free survival (TFS). Secondary outcomes included all-cause and respiratory hospitalizations, lung function, and plasma cytokine concentrations. Cox regression was used to assess mortality risk. Outcomes were assessed in three independent ILD cohorts. MEASUREMENTS AND MAIN RESULTS: Chest CT scans were assessed in 1,094 patients (mean age, 64 yr; 52% male). MLN enlargement (≥10 mm) was present in 66% (n = 726) and strongly predicted TFS (hazard ratio [HR], 1.53; 95% confidence interval [CI], 1.12-2.10; P = 0.008) and risk of all-cause and respiratory hospitalizations (internal rate of return [IRR], 1.52; 95% CI, 1.17-1.98; P = 0.002; and IRR, 1.71; 95% CI, 1.15-2.53; P = 0.008, respectively) when compared with subjects with MLN <10 mm. Patients with MLN enlargement had lower lung function and decreased plasma concentrations of soluble CD40L (376 pg/ml vs. 505 pg/ml, P = 0.001) compared with those without MLN enlargement. Plasma IL-10 concentration >45 pg/ml predicted mortality (HR, 4.21; 95% CI, 1.21-14.68; P = 0.024). Independent analysis of external datasets confirmed these findings. CONCLUSIONS: MLN enlargement predicts TFS and hospitalization risk in ILD and is associated with decreased levels of a key circulating cytokine, soluble CD40L. Incorporating MLN and cytokine findings into current prediction models might improve ILD prognostication.


Assuntos
Doenças Pulmonares Intersticiais/mortalidade , Linfonodos/diagnóstico por imagem , Mediastino/diagnóstico por imagem , Valor Preditivo dos Testes , Tomografia Computadorizada por Raios X/métodos , Idoso , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
5.
Eur Respir J ; 51(6)2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29724923

RESUMO

We studied whether African-American race is associated with younger age and decreased survival time at diagnosis of interstitial lung disease (ILD).We performed a multicentre, propensity score-matched analysis of patients with an ILD diagnosis followed at five US hospitals between 2006 and 2016. African-Americans were matched with patients of other races based on a time-dependent propensity score calculated from multiple patient, physiological, diagnostic and hospital characteristics. Multivariable logistic regression models were used. All-cause mortality and hospitalisations were compared between race-stratified patient cohorts with ILD, and sensitivity analyses were performed.The study included 1640 patients with ILD, 13% of whom were African-American, followed over 5041 person-years. When compared with patients of other races, African-Americans with ILD were younger at diagnosis (56 years versus 67 years), but in the propensity-matched analyses had greater survival (hazard ratio 0.46, 95% CI 0.28-0.77; p=0.003) despite similar risk of respiratory hospitalisations (relative risk 1.04, 95% CI 0.83-1.31; p=0.709), and similar GAP-ILD (gender-age-physiology-ILD) scores at study entry. Sensitivity analyses in a separate cohort of 9503 patients with code-based ILD diagnosis demonstrated a similar association of baseline demographic characteristics with all-cause mortality.We conclude that African-Americans demonstrate a unique phenotype associated with younger age at ILD diagnosis and perhaps longer survival time.


Assuntos
Afro-Americanos , Hospitalização/estatística & dados numéricos , Doenças Pulmonares Intersticiais/mortalidade , Adulto , Idoso , Causas de Morte , Feminino , Humanos , Modelos Logísticos , Doenças Pulmonares Intersticiais/etnologia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Pontuação de Propensão , Estudos Retrospectivos , Estados Unidos
6.
J Thorac Imaging ; 33(4): 266-270, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29683868

RESUMO

PURPOSE: The purpose of this article was to determine whether a novel finding on coronal computed tomography (CT) can help differentiate usual interstitial pneumonia (UIP) from nonspecific interstitial pneumonia (NSIP) in order to obviate lung biopsy. MATERIALS AND METHODS: Two chest radiologists, blinded to clinical data, reviewed 3 preselected coronal images from CT scans, performed within 1 year of surgical lung biopsy (SLB), from 51 patients with biopsy-proven UIP and 15 with biopsy-proven NSIP. The 198 (66×3) images were anonymized and randomized. The radiologists assessed each coronal image for the presence or absence of the straight-edge sign (SES) on both the right and left sides, anecdotally thought to be more common in NSIP than in UIP. The SES was defined as reticulation isolated to the lung bases with sharp demarcation in the craniocaudal plane and without substantial extension along the lateral margins of the lungs. A validation cohort from a second medical center was also evaluated to reassess our findings. RESULTS: The absence of a bilateral SES yielded a sensitivity, specificity, positive predictive value (PPV), and negative predictive value of 56.9%, 93.3%, 96.7%, and 38.9%, respectively, for UIP on SLB. The unilateral or bilateral absence of the SES yielded a sensitivity, specificity, PPV, and negative predictive value of 76.5%, 66.7%, 88.6%, and 45.5%, respectively, for UIP on SLB. For the 11 subjects with an overall CT pattern consistent with NSIP but a pathologic diagnosis of UIP, the SES was absent in 6 (54.5%) subjects. In the validation cohort, the SES was much more common in NSIP than in UIP (46.6% compared with 3.3%, respectively; P<0.001). CONCLUSION: The absence of the SES has a high PPV for biopsy-proven UIP. Bilateral absence of the SES has high specificity (93.3%) for biopsy-proven UIP. The SES may be useful for differentiating UIP from NSIP.


Assuntos
Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Diagnóstico Diferencial , Humanos , Pulmão/diagnóstico por imagem , Reprodutibilidade dos Testes , Sensibilidade e Especificidade
7.
AJR Am J Roentgenol ; 210(5): 1034-1041, 2018 May.
Artigo em Inglês | MEDLINE | ID: mdl-29547052

RESUMO

OBJECTIVE: The purpose of this study was to assess the diagnostic significance of CT patterns that cannot be classified according to current idiopathic pulmonary fibrosis (IPF) guidelines and of specific findings of the inconsistent with usual interstitial pneumonitis (UIP) pattern. MATERIALS AND METHODS: Subjects with a multidisciplinary diagnosis of interstitial lung disease who had undergone surgical lung biopsy and chest CT within 1 year of each other were included in the study. The predominant distribution and pattern of disease were scored. Cases were classified as UIP, possible UIP, or inconsistent with UIP at chest CT according to 2011 IPF guidelines. Cases that could not be confidently categorized with current guidelines were annotated as indeterminate. RESULTS: UIP, possible UIP, and inconsistent with UIP CT patterns were associated with pathologic UIP in 89.6%, 81.6%, and 60.0% of subjects. An indeterminate CT pattern (7.7% [20/259]) was associated with a UIP pathologic diagnosis in 55.0% of cases. This finding was not statistically different from the findings in the group with the inconsistent with UIP CT pattern (p = 0.677) but was different from the findings in the UIP (p < 0.001) and possible UIP (p = 0.031) groups. In regard to specific findings of the inconsistent with UIP CT category, ground-glass opacity, air-trapping, consolidation, and axial distribution were associated with a non-UIP pathologic diagnosis; however, there was no significant association with zonal distribution. CONCLUSION: A substantial minority of cases cannot be confidently categorized according to current guidelines for IPF and differ diagnostically from the possible UIP and UIP CT categories. The term "inconsistent with UIP" is misleading and should be renamed.


Assuntos
Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/patologia , Guias de Prática Clínica como Assunto , Tomografia Computadorizada por Raios X/métodos , Idoso , Biópsia , Feminino , Humanos , Fibrose Pulmonar Idiopática/cirurgia , Masculino , Pessoa de Meia-Idade
8.
BMC Pulm Med ; 18(1): 30, 2018 Feb 08.
Artigo em Inglês | MEDLINE | ID: mdl-29422061

RESUMO

BACKGROUND: Mortality is similarly high among individuals with usual interstitial pneumonia (UIP) due to idiopathic pulmonary fibrosis (IPF) and interstitial pneumonia with autoimmune features (IPAF). Circulating anti-nuclear antibodies (ANA) are commonly found in this patient population, suggesting possible aberrant immune activation. Because an environment of oxidative stress can result from immunologic activation, we hypothesized that ANA positive patients with UIP would have improved outcome when exposed to the antioxidant N-acetylcysteine (NAC) compared to ANA negative patients. METHODS: A single center, retrospective cohort analysis was performed. Patients with UIP due to IPF and IPAF were stratified according to ANA status to and NAC exposure. Transplant-free survival (TFS) was assessed using the Kaplan-Meier estimator and multivariable Cox regression adjusted for diagnosis, gender/age/physiology score, immunosuppressant exposure and anti-fibrotic exposure. RESULTS: Of 293 individuals with UIP due to IPF (74%) or IPAF (26%), NAC exposure was documented in 58 (19.8%). Among NAC exposed individuals, 33 (56.9%) were ANA seropositive and 25 (43.1%) were seronegative. NAC exposure was associated with improved TFS survival among ANA seropositive individuals in unadjusted analysis (plogrank = 0.02) and after multi-variable adjustment (HR 0.51, 95% CI 0.30-0.87; p = 0.01). There was no association between NAC exposure and TFS in ANA seronegative individuals (HR 1.26, 95% CI 0.69-2.32; p = 0.45). Formal interaction testing confirmed NAC*ANA interaction (p = 0.04) and sensitivity analysis demonstrated an increasing effect size associated with NAC therapy as ANA titer increased. Among patients with available genetic data, a marginally higher proportion of ANA positive patients (p = 0.08) carried the rs3750920 (TOLLIP) genotype previously shown to predict favorable outcome in NAC exposed patients. CONCLUSION: NAC exposure is associated with improved transplant-free survival ANA positive patients with UIP. These findings support the prospective collection of ANA data in in future NAC clinical trials performed in patients with UIP.


Assuntos
Acetilcisteína/uso terapêutico , Depuradores de Radicais Livres/uso terapêutico , Fibrose Pulmonar Idiopática/tratamento farmacológico , Idoso , Anticorpos Antinucleares/imunologia , Estudos de Coortes , Feminino , Humanos , Fibrose Pulmonar Idiopática/imunologia , Fibrose Pulmonar Idiopática/mortalidade , Estimativa de Kaplan-Meier , Transplante de Pulmão/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Taxa de Sobrevida
9.
AJR Am J Roentgenol ; 210(2): 307-313, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29140119

RESUMO

OBJECTIVE: A substantial proportion of cases of usual interstitial pneumonia (UIP) are due to connective tissue disease (CTD)-associated interstitial lung disease (ILD). The purpose of this study was to determine whether specific CT findings can help differentiate a UIP pattern of CTD-ILD from a UIP pattern of idiopathic pulmonary fibrosis (IPF) and whether these signs are associated with survival. MATERIALS AND METHODS: Adults visiting an ILD clinic from 2006 to 2015 enrolled in a research registry with a multidisciplinary diagnosis of CTD-ILD or IPF and a UIP pattern at high-resolution CT were included in the study. In these subjects with CT findings of UIP due to either IPF or CTD-ILD, three CT findings anecdotally associated with CTD-ILD were assessed for diagnostic accuracy: the "straight-edge" sign, the "exuberant honeycombing" sign, and the "anterior upper lobe" sign. Survival assessments were performed with univariate and multivariable techniques. RESULTS: The subjects included 63 patients who had CTD-ILD and 133 patients who had IPF with a UIP pattern at CT. All three CT signs were significantly more common in subjects with CTD-ILD than those with IPF (prevalence, 22.2-25.4% for CTD-ILD, 6.0-12.8% for IPF; p = 0.028 to < 0.001). The highest specificity (94.0%) and sensitivity (25.4%) were seen for the straight-edge sign. No CT sign was associated with survival in multivariable analysis. CONCLUSION: Although UIP is usually associated with IPF, the index of suspicion for CTD-ILD should be raised in the care of patients with any of the three CT signs. A thorough workup for CTD-ILD should be pursued, including referral to the rheumatology department.


Assuntos
Doenças do Tecido Conjuntivo/diagnóstico por imagem , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Sistema de Registros , Sensibilidade e Especificidade
10.
Chest ; 153(2): 349-360, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-28964798

RESUMO

BACKGROUND: The current interstitial lung disease (ILD) classification has overlapping clinical presentations and outcomes. Cluster analysis modeling is a valuable tool in identifying distinct clinical phenotypes in heterogeneous diseases. However, this approach has yet to be implemented in ILD. METHODS: Using cluster analysis, novel ILD phenotypes were identified among subjects from a longitudinal ILD cohort, and outcomes were stratified according to phenotypic clusters compared with subgroups according to current American Thoracic Society/European Respiratory Society ILD classification criteria. RESULTS: Among subjects with complete data for baseline variables (N = 770), four clusters were identified. Cluster 1 (ie, younger white obese female subjects) had the highest baseline FVC and diffusion capacity of the lung for carbon monoxide (Dlco). Cluster 2 (ie, younger African-American female subjects with elevated antinuclear antibody titers) had the lowest baseline FVC. Cluster 3 (ie, elderly white male smokers with coexistent emphysema) had intermediate FVC and Dlco. Cluster 4 (ie, elderly white male smokers with severe honeycombing) had the lowest baseline Dlco. Compared with classification according to ILD subgroup, stratification according to phenotypic clusters was associated with significant differences in monthly FVC decline (Cluster 4, -0.30% vs Cluster 2, 0.01%; P < .0001). Stratification by using clusters also independently predicted progression-free survival (P < .001) and transplant-free survival (P < .001). CONCLUSIONS: Among adults with diverse chronic ILDs, cluster analysis using baseline characteristics identified four distinct clinical phenotypes that might better predict meaningful clinical outcomes than current ILD diagnostic criteria.


Assuntos
Doenças Pulmonares Intersticiais/classificação , Idoso , Doença Crônica , Análise por Conglomerados , Feminino , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Fenótipo , Fibrose Pulmonar/patologia
11.
Front Med (Lausanne) ; 4: 170, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-29085824

RESUMO

BACKGROUND: Chronic hypersensitivity pneumonitis (CHP) is a fibrotic parenchymal lung disease that occurs when inhalation of environmental antigens leads to immune dysregulation. Autoimmune features have recently been identified as potentially important among patients with CHP. However, the relationship between hypothyroidism (HT) and CHP is unknown. In this study, we investigate the prevalence and impact of HT among patients with CHP. METHODS: We conducted a retrospective, case-control analysis. We identified 121 patients at the University of Chicago Interstitial Lung Disease Center with a multidisciplinary diagnosis of CHP. These patients were matched 3:1 according to age, sex, and race to 363 control subjects with asthma from 2006 to 2015. We analyzed demographics, clinical characteristics, and survival between both groups and assessed the relationship of HT with CHP. Survival analysis was performed using Cox proportional hazards modeling. RESULTS: Patients with CHP had higher prevalence of HT (25.6%, n = 31) compared to controls (10.7%, n = 39; OR, 2.86; 95% CI, 1.62-4.99; P < 0.0001). Compared to CHP alone, patients with CHP/HT were more likely to be female (80.6 vs 51.1%, P = 0.004), have increased incidence of autoimmune disease (19.4 vs 3.3%, P = 0.009), antinuclear antibody seropositivity (80.6 vs 57.0%, P = 0.019), and higher TSH levels (4.0 vs 1.9 mIU/L, P < 0.0001). HT was a significant independent predictor of mortality among CHP patients with seropositive ANA (HR, 3.39; 95% CI, 1.31-8.80; P = 0.012). CONCLUSION: HT is common in patients with CHP and may carry prognostic significance in patients with features of autoimmunity. Further research exploring common pathogenic pathways between autoimmune HT and CHP may illuminate the association of HT with survival.

13.
ERJ Open Res ; 3(3)2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28845429

RESUMO

In chronic hypersensitivity pneumonitis (CHP), lack of improvement or declining lung function may prompt use of immunosuppressive therapy. We hypothesised that use of azathioprine or mycophenolate mofetil with prednisone reduces adverse events and lung function decline, and improves transplant-free survival. Patients with CHP were identified. Demographic features, pulmonary function tests, incidence of treatment-emergent adverse events (TEAEs) and transplant-free survival were characterised, compared and analysed between patients stratified by immunosuppressive therapy. A multicentre comparison was performed across four independent tertiary medical centres. Among 131 CHP patients at the University of Chicago medical centre (Chicago, IL, USA), 93 (71%) received immunosuppressive therapy, and had worse baseline forced vital capacity (FVC) and diffusing capacity, and increased mortality compared with those who did not. Compared to patients treated with prednisone alone, TEAEs were 54% less frequent with azathioprine therapy (p=0.04) and 66% less frequent with mycophenolate mofetil (p=0.002). FVC decline and survival were similar between treatment groups. Analyses of datasets from four external tertiary medical centres confirmed these findings. CHP patients who did not receive immunosuppressive therapy had better survival than those who did. Use of mycophenolate mofetil or azathioprine was associated with a decreased incidence of TEAEs, and no difference in lung function decline or survival when compared with prednisone alone. Early transition to mycophenolate mofetil or azathioprine may be an appropriate therapeutic approach in CHP, but more studies are needed.

14.
Eur Radiol ; 27(12): 5127-5135, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28687910

RESUMO

OBJECTIVES: To identify CT findings in chronic hypersensitivity pneumonitis (cHP) associated with survival. MATERIAL AND METHODS: Two thoracic radiologists assessed CT scans for specific imaging findings and patterns in 132 subjects with cHP. Survival analyses were performed. RESULTS: The majority of subjects had an inconsistent with usual interstitial pneumonitis pattern on CT (55.3%,73/132). Hypersensitivity pneumonitis (HP) diagnosis on CT was less common in those with fibrosis (66.1%, 74/112) than those without fibrosis (85%,17/20). Smoking was associated with a lower prevalence of HP on CT (p=0.04). CT features of pulmonary fibrosis, especially traction bronchiectasis (HR 8.34, 95% CI 1.98-35.21) and increased pulmonary artery (PA)/aorta ratio (HR 2.49, 95% CI 1.27-4.89) were associated with worse survival, while ground-glass opacity (HR 0.31, 95% CI 0.12-0.79) was associated with improved survival. Survival association with imaging was less pronounced after adjustment for gender, age and physiology score. CONCLUSIONS: A substantial proportion of cHP cases have a non-HP-like appearance. Ground-glass opacity, pulmonary fibrosis features and elevated PA/aorta ratio on CT likely reflect varying degrees of disease severity in cHP and may inform future clinical prediction models. KEY POINTS: • A substantial proportion of subjects with chronic HP have a UIP-like pattern. • A UIP pattern in HP may be potentiated by smoking. • A diagnosis of HP should not be excluded based solely on CT appearance. • CT fibrosis and increased PA/aorta ratio signal worse survival.


Assuntos
Alveolite Alérgica Extrínseca/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Alveolite Alérgica Extrínseca/patologia , Doença Crônica , Feminino , Fibrose/patologia , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/patologia , Fibrose Pulmonar Idiopática/patologia , Masculino , Pessoa de Meia-Idade , Prevalência , Artéria Pulmonar/diagnóstico por imagem , Análise de Sobrevida
15.
Arch Pathol Lab Med ; 141(7): 960-969, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28467213

RESUMO

CONTEXT: - Patients with idiopathic interstitial pneumonia may display evidence of autoimmunity without meeting criteria for a defined connective tissue disease. A recent European Respiratory Society/American Thoracic Society statement proposed research criteria for interstitial pneumonia with autoimmune features (IPAF), which includes findings from the clinical, serologic, and morphologic domains. OBJECTIVES: - To investigate the importance of histopathologic criteria within the morphologic domain and to report our methodology for identifying these features. DESIGN: - Patients with idiopathic interstitial pneumonia at the University of Chicago who underwent surgical lung biopsy or lung transplantation were assessed for IPAF histopathologic features, using the initial pathology interpretation in the electronic records. A focused rereview of available slides by a pulmonary pathologist was then performed for patients who failed to meet IPAF criteria on initial pathology assessment. RESULTS: - Of 422 patients with idiopathic interstitial pneumonia, 176 (41.7%) underwent surgical lung biopsy or lung transplant. Forty-six of those 176 patients (26.1%) met IPAF criteria by initial pathology interpretation and a positive clinical or serologic feature. Of the remaining 130 patients, 73 (56.2%) met either the clinical or serologic domains without meeting the morphologic domain, whereas 36 (27.7%) had slides available for pathology rereview. This rereview demonstrated nonspecific interstitial pneumonia in 8 of 36 patients (22.2%) and lymphoplasmacytic infiltrates in 6 of 36 patients (16.7%), resulting in an additional 7 of 36 patients (19.4%) with idiopathic interstitial pneumonia that met the IPAF criteria. In IPAF, pulmonary vasculopathy was the most prevalent finding (45 of 84; 53.6%) and predicted increased mortality (hazard ratio, 2.5; P = .04). CONCLUSIONS: - Using a methodological approach to identifying IPAF pathology, we demonstrate a significant increase in the number of patients meeting IPAF criteria because of focused pathologic review and highlight the prognostic value of the IPAF pathologic findings.


Assuntos
Autoimunidade , Pneumonias Intersticiais Idiopáticas/diagnóstico , Patologia Clínica/métodos , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
16.
AJR Am J Roentgenol ; 208(6): 1229-1236, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28350485

RESUMO

OBJECTIVE: The objective of this study is to determine the CT findings and patterns of interstitial pneumonia with autoimmune features (IPAF) and to assess whether imaging can predict survival for patients with IPAF. MATERIALS AND METHODS: The study included 136 subjects who met the criteria for IPAF and had diagnostic-quality chest CT scans obtained from 2006 to 2015; a total of 74 of these subjects had pathologic samples available for review within 1 year of chest CT examination. CT findings and the presence of an usual interstitial pneumonitis (UIP) pattern of disease were assessed, as was the UIP pattern noted on pathologic analysis. Analysis of chest CT findings associated with survival was performed using standard univariate and multivariate Cox proportional hazards methods as well as the unadjusted log-rank test. Survival data were visually presented using the Kaplan-Meier survival curve estimator. RESULTS: Most subjects with IPAF (57.4%; 78/136) had a high-confidence diagnosis of a UIP pattern on CT. Substantially fewer subjects (28.7%; 39/136) had a pattern that was inconsistent with UIP noted on CT. The presence of a UIP pattern on CT was associated with smoking (p < 0.01), male sex (p < 0.01), and older age (p < 0.001). Approximately one-fourth of the subjects had a nonspecific interstitial pneumonitis pattern on CT. Of interest, nearly one-tenth of the subjects had a CT pattern that was most consistent with hypersensitivity pneumonitis rather than the customary CT patterns ascribed to lung disease resulting from connective tissue disease. Most subjects with a possible UIP pattern on CT (83.3%) had UIP diagnosed on the basis of pathologic findings. Focused multivariate analysis showed that honeycombing on CT (hazard ratio, 2.17; 95% CI, 1.05-4.47) and pulmonary artery enlargement on CT (hazard ratio, 2.08; 95% CI, 1.02-4.20) were independent predictors of survival. CONCLUSION: IPAF most often presents with a UIP pattern on CT and is associated with worse survival when concomitant honeycombing or pulmonary artery enlargement is present.


Assuntos
Doenças Autoimunes/diagnóstico por imagem , Doenças Autoimunes/mortalidade , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/mortalidade , Análise de Sobrevida , Tomografia Computadorizada por Raios X/estatística & dados numéricos , Doenças Autoimunes/patologia , Chicago/epidemiologia , Comorbidade , Feminino , Humanos , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Prevalência , Prognóstico , Reprodutibilidade dos Testes , Estudos Retrospectivos , Medição de Risco , Sensibilidade e Especificidade , Estatística como Assunto
18.
Respir Med ; 121: 117-122, 2016 12.
Artigo em Inglês | MEDLINE | ID: mdl-27888985

RESUMO

BACKGROUND: Azathioprine is a commonly prescribed therapy for connective tissue disease-associated interstitial lung disease (CTD-ILD). Combination therapy that included azathioprine was recently shown to increase the risk of death and hospitalization in patients with idiopathic pulmonary fibrosis. Whether azathioprine increases the risk of adverse outcomes in patients with fibrotic CTD-ILD, including those with CTD-associated usual interstitial pneumonia (UIP), remains unknown. METHODS: A retrospective cohort analysis was performed to determine the combined incidence rate of death, transplant and respiratory hospitalization associated with azathioprine exposure. A fibrotic CTD-ILD cohort treated with mycophenolate mofetil served as a comparator group. Incidence rates were compared with an incidence rate ratio (IRR) generated by negative binomial regression. Longitudinal pulmonary function response was then assessed using mixed effects linear regression models. RESULTS: Fifty-four patients were treated with azathioprine and forty-three with mycophenolate. Medication discontinuation due to non-respiratory side effects occurred in 27% and 5% of the azathioprine and mycophenolate cohorts, respectively. The combined incidence rate of adverse outcomes was 0.015 and 0.013 for azathioprine and mycophenolate, respectively (IRR 1.23; 95% CI 0.49-3.12; p = 0.66). Similar incidence rates were observed among those with CTD-UIP (IRR 0.83; 95% CI 0.21-3.31; p = 0.79). Both groups demonstrated pulmonary function stability over time, with the azathioprine group demonstrating a marginal improvement. CONCLUSIONS: A significant minority of patients could not tolerate azathioprine due to non-respiratory side effects. Of those who did tolerate azathioprine, a similar incidence of adverse outcomes was observed as those treated with mycophenolate. Both therapies were associated with stability in pulmonary function.


Assuntos
Azatioprina/uso terapêutico , Doenças do Tecido Conjuntivo/complicações , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Adulto , Idoso , Azatioprina/efeitos adversos , Doenças do Tecido Conjuntivo/fisiopatologia , Estudos Cross-Over , Feminino , Hospitalização/estatística & dados numéricos , Humanos , Imunossupressores/efeitos adversos , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Ácido Micofenólico/efeitos adversos , Ácido Micofenólico/uso terapêutico , Capacidade de Difusão Pulmonar/efeitos dos fármacos , Sistema de Registros , Estudos Retrospectivos , Capacidade Vital/efeitos dos fármacos
19.
Radiol Clin North Am ; 54(6): 1033-1046, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27719974

RESUMO

The management of hypersensitivity pneumonitis (HP) depends on early identification of the disease process, which is complicated by its nonspecific clinical presentation in addition to variable and diverse laboratory and radiologic findings. HP is the result of exposure and sensitization to myriad aerosolized antigens. HP develops in the minority of antigenic exposures, and conversely has been documented in patients with no identifiable exposure, complicating the diagnostic algorithm significantly. Prompt diagnosis and early intervention are critical in slowing the progression of irreversible parenchymal damage, and additionally in preserving the quality of life of affected patients.


Assuntos
Alveolite Alérgica Extrínseca/diagnóstico por imagem , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Alvéolos Pulmonares/diagnóstico por imagem , Radiografia Torácica/métodos , Tomografia Computadorizada por Raios X/métodos , Alveolite Alérgica Extrínseca/complicações , Diagnóstico Diferencial , Medicina Baseada em Evidências , Humanos , Fibrose Pulmonar Idiopática/etiologia , Intensificação de Imagem Radiográfica/métodos
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