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1.
Medicine (Baltimore) ; 99(28): e20930, 2020 Jul 10.
Artigo em Inglês | MEDLINE | ID: mdl-32664089

RESUMO

Surgical lung biopsy is regarded as the golden standard for the diagnosis of idiopathic interstitial pneumonias (IIPs). Here, we attempted to show the diagnostic accuracy of multidisciplinary classifications based on transbronchial pathology including transbronchial lung cryobiopsy (TBLC) , bronchoalveolar lavage fluid (BALF) and endobronchial ultrasound-guided transbronchial needle aspiration biopsy (EBUS-TBNA).Patients with suspected interstitial lung diseases admitted from June 1, 2016 to December 31, 2018 were involved. Patients with known causes of interstitial lung diseases and typical idiopathic pulmonary fibrosis diagnosed through clinical, radiological information were excluded. Patients with atypical idiopathic pulmonary fibrosis and possible IIPs accepted transbronchial pathological evaluation. Initial multidisciplinary diagnosis (MDD) classifications were made depending on clinical, radiological and transbronchial pathological information by a multidisciplinary team (MDT). The final MDD classifications were confirmed by subsequent therapeutic effects. All patients were followed up for at least 6 months.A total of 70 patients were finally involved. The samples of lung parenchyma extracted through TBLC were enough for confirmation of pathological diagnoses in 68.6% (48/70) cases. Samples of 6 cases were extracted by EBUS-TBNA. Bacteriological diagnoses were positive in 1 case by BALF. Pathological diagnoses of 77.1% (54/70) cases were achieved through TBLC, EBUS-TBNA and BALF. During the follow up study, the pulmonary lesions of 60% patients were improved, 11.43% were relapsed when glucocorticoid was reduced to small dose or withdrawal, 14.29% were leveled off and 8.57% were progressed. The diagnoses of 4 patients with progressed clinical feature were revised. As a result, 94.3% initial MDD classifications based on transbronchial pathology were consistent with the final MDD, and the difference of diagnostic yield wasn't significant between initial and final MDD (Z = -1.414, P = .157).Classifications of IIPs based on transbronchial pathology were useful and quite agreed with final MDD.


Assuntos
Broncoscopia/métodos , Pneumonias Intersticiais Idiopáticas/classificação , Pneumonias Intersticiais Idiopáticas/patologia , Biópsia Guiada por Imagem/métodos , Idoso , Biópsia/tendências , Líquido da Lavagem Broncoalveolar/microbiologia , Broncoscopia/efeitos adversos , Broncoscopia/tendências , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico/métodos , Feminino , Seguimentos , Glucocorticoides/uso terapêutico , Hospitalização , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/tratamento farmacológico , Fibrose Pulmonar Idiopática/patologia , Comunicação Interdisciplinar , Pulmão/diagnóstico por imagem , Pulmão/patologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Recidiva , Tomografia Computadorizada por Raios X/métodos
2.
Medicine (Baltimore) ; 98(48): e18131, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31770246

RESUMO

Anaplastic lymphoma kinase (ALK) inhibitor-related pneumonitis (ALK-IIP) is relatively rare but sometimes fatal, so the timely diagnosis of ALK-IIP is important for enabling prompt management. However, the detailed radiologic characteristics and clinical course of ALK-IIP are still unclear. This study was performed to investigate the clinical and radiologic characteristics and risk factors of ALK-IIP in patients with non-small cell lung cancer (NSCLC).A total of 250 NSCLC patients who had been treated with ALK inhibitors were retrospectively enrolled. Chest computed tomography (CT) was classified into 4 CT patterns using the 2013 guideline for idiopathic interstitial pneumonia: cryptogenic organizing pneumonia (COP), hypersensitivity pneumonitis (HP), acute interstitial pneumonia (AIP), and nonspecific interstitial pneumonia. Clinical characteristics including toxicity grading and treatment course were analyzed in regarding to CT patterns. Clinical characteristics were compared between patients with ALK-IIP and without ALK-IIP.ALK-IIP was identified in 11 patients (4.4%). The most common CT pattern was the COP pattern (n = 7, 63.6%) and followed by HP and AIP patterns (both, n = 2, 18.2%). ALK-IIP showed pneumonitis toxicity grade ranged from 1 to 4, and AIP pattern had the highest toxicity grade, followed by HP and COP patterns (median grade: 3.5, 2.5, 1). All of the patients with the COP pattern were successfully treated, while half of patients with the AIP pattern died during treatment. The smoking history and extrathoracic metastasis were more frequent in patients with ALK-IIP (P < .005). The smoking history was associated with a higher incidence of ALK-IIP (odds ratio: 3.586, 95% confidence interval: 1.058-13.432, P = .049).ALK-IIP showed a spectrum of chest CT patterns, which reflected the toxicity grades. The COP pattern was the most common CT pattern of ALK-IIP, and patients with ALK-IIP of the COP pattern were successfully treated. ALK inhibitors should be used with caution in NSCLC patients with smoking history.


Assuntos
Antineoplásicos/efeitos adversos , Carcinoma Pulmonar de Células não Pequenas/tratamento farmacológico , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Neoplasias Pulmonares/tratamento farmacológico , Pneumonia/diagnóstico por imagem , Inibidores de Proteínas Quinases/efeitos adversos , Tomografia Computadorizada por Raios X , Adulto , Idoso , Quinase do Linfoma Anaplásico/antagonistas & inibidores , Carcinoma Pulmonar de Células não Pequenas/diagnóstico por imagem , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/induzido quimicamente , Neoplasias Pulmonares/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Pneumonia/induzido quimicamente , Estudos Retrospectivos , Fatores de Risco
3.
Respir Med ; 152: 44-50, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31128609

RESUMO

BACKGROUND AND PURPOSE: Patients with anti-aminoacyl-tRNA synthetase (ARS) antibodies frequently experience complications of interstitial pneumonia (ARS-IP), and the computed tomography (CT) of ARS-IP frequently shows nonspecific interstitial pneumonia (NSIP) pattern. The CT pattern of ARS-IP might be different from that of idiopathic IP. However, the clinical differences in patients with ARS-IP and idiopathic IP showing the similar CT patterns have not yet been well studied. The objective of this study was to evaluate the clinical differences between patients with ARS-NSIP and idiopathic NSIP (I-NSIP). METHODS: Two groups of 34 patients each, with ARS-NSIP and I-NSIP, who visited Hiroshima University Hospital between January 2005 and December 2017, were enrolled. Clinical features and outcomes were retrospectively compared between the two groups. RESULTS: The ARS-NSIP group included more female patients and significantly younger patients than the I-NSIP group. The percentage of lymphocytes in bronchoalveolar lavage fluid (BALF) was significantly higher, and the CD4/CD8 ratio in BALF was significantly lower in the ARS-NSIP group compared with the I-NSIP group. The proportion of patients with traction bronchiectasis detected by CT was significantly higher in I-NSIP compared with ARS-NSIP. The number of patients who received corticosteroid and/or immunosuppressant therapy was significantly larger in the ARS-NSIP group than in the I-NSIP group. In addition, the patients in the I-NSIP group who underwent the immunosuppressive therapy demonstrated shorter survival than those who underwent no treatment; this tendency was not observed in the ARS-NSIP group. The 10-year survival rate of patients in the ARS-NSIP group was significantly higher than that of patients in the I-NSIP group (91.8% vs. 43.0%; log-rank, p = 0.012). The multivariate survival analysis revealed that positive anti-ARS antibody was an independent favorable prognostic factor in the patients with NSIP (OR, [95% CI]:0.12 [0.02-0.55], p = 0.013). CONCLUSIONS: Patients with ARS-NSIP had a significantly better prognosis than those with I-NSIP; this may be associated with the sensitivity to immunosuppressive therapies, and the different findings of BALF and HRCT between the two groups.


Assuntos
Aminoacil-tRNA Sintetases/antagonistas & inibidores , Líquido da Lavagem Broncoalveolar/imunologia , Pneumonias Intersticiais Idiopáticas/imunologia , Doenças Pulmonares Intersticiais/imunologia , Corticosteroides/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Aminoacil-tRNA Sintetases/imunologia , Autoanticorpos/imunologia , Bronquiectasia/diagnóstico por imagem , Bronquiectasia/epidemiologia , Relação CD4-CD8/métodos , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/tratamento farmacológico , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Imunossupressores/uso terapêutico , Japão/epidemiologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/fisiopatologia , Linfócitos/citologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Análise de Sobrevida , Taxa de Sobrevida , Tomografia Computadorizada por Raios X/métodos
4.
Kaohsiung J Med Sci ; 35(6): 365-372, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30913371

RESUMO

Interstitial lung disease (ILD) is a common pulmonary manifestation of connective tissue diseases (CTD). Prognostic effect of radiological usual interstitial pneumonia (UIP) pattern in CTD-associated interstitial lung disease (CTD-ILD) is unknown. This study aimed to investigate the disease progression and mortality of patients with CTD-ILD and idiopathic interstitial pneumonias (IIP) including idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia and the prognostic impact of the radiological UIP pattern on both disease groups. The medical records of 91 patients (55 with CTD-ILD and 36 with IIP) diagnosed with ILD at pulmonary medicine department, Faculty of Medicine, Gazi University from 2004 to 2014 were retrospectively reviewed. Patients included whose baseline high-resolution computed tomography (HRCT) scans showed either a UIP or non-UIP pattern. While 67.3% (n = 37) of CTD-ILD patients possessed UIP pattern, 38.9% (n = 14) of IIP patients had UIP pattern in HRCT. Respiratory functions including the forced expiratory volume in the first second (FEV1 ), functional vital capacity (FVC), and transfer coefficient for carbon monoxide (diffusing capacity of the lung for carbon monoxide [DLCO]) of IIP group at the time of diagnosis were significantly lower than CTD-ILD group (P = .007, P = .002, and P = .019, respectively). There was no significant survival difference between CTD-ILD and IIP by using the log-rank test (P = .76). Multivariate analysis revealed that UIP pattern in HRCT (Hazard ratio: 1.85; 95% Confidence interval = 1.14-3; P = .013), annual FVC (Hazard ratio: 0.521; 95% Confidence interval = 0.32-0.84; P = .007), and annual DLCO declines (Hazard ratio: 0.943; 95% Confidence interval = 0.897-0.991; P = .02) were independent risk factors for mortality in both CTD-ILD and IIP groups. We found that UIP pattern in HRCT and annual losses in respiratory functions were the main determinants of prognosis of ILDs either idiopathic or CTD-associated.


Assuntos
Pneumonias Intersticiais Idiopáticas/fisiopatologia , Fibrose Pulmonar Idiopática/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Pulmão/fisiopatologia , Idoso , Monóxido de Carbono/metabolismo , Progressão da Doença , Feminino , Volume Expiratório Forçado , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/mortalidade , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/mortalidade , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/mortalidade , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Prognóstico , Estudos Retrospectivos , Análise de Sobrevida , Tomografia Computadorizada por Raios X , Capacidade Vital
6.
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.


Assuntos
Proteínas de Transporte/metabolismo , Pneumonias Intersticiais Idiopáticas/sangue , Pneumonias Intersticiais Idiopáticas/epidemiologia , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Idoso , Anticorpos Antinucleares/imunologia , Autoimunidade/imunologia , Proteínas de Transporte/sangue , Doenças do Tecido Conjuntivo/epidemiologia , Feminino , Humanos , Hipertensão Pulmonar/fisiopatologia , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pulmão/irrigação sanguínea , Pulmão/diagnóstico por imagem , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prevalência , Radiografia Torácica/métodos , Testes de Função Respiratória/métodos , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
7.
Surg Today ; 49(6): 467-473, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30612207

RESUMO

PURPOSE: Several vascular measurements in computed tomography (CT) were reported to be indicators of pulmonary hypertension in chronic obstructive pulmonary disease (COPD) patients. We evaluated the usefulness of these parameters as predictors of postoperative mortality in lung cancer patients with IIP. METHODS: This retrospective study was performed on 1888 patients. The following CT findings were evaluated: diameter of the main pulmonary artery, ascending aorta, and the short axis of the inferior vena cava (IVC). Univariate and multivariate analyses were conducted to determine predictors of surgical mortality. RESULTS: In the IIP patients, the 90-day mortality was 0.8%, and the 2-year mortality was 5.8%. Regarding the 90-day mortality in patients with IIP, a multivariate analysis revealed a short axis of IVC > 21 mm [odds ratio (OR) 6.4, p < 0.01) and the risk score reported by Japanese Association for Chest Surgery (JACS) (OR 1.4, p = 0.01) as independent predictors. Regarding the 2-year mortality in patients with IIP, a multivariate analysis revealed IVC > 21 mm (OR 2.3, p < 0.04), %VC < 80% (OR 2.4, p = 0.02), and pathological cancer stages II and III vs. I (OR 7.2, p < 0.001) as independent predictors. CONCLUSIONS: Enlargement of the IVC as measured by CT was a significant predictor of mortality after surgery for lung cancer with IIP patients.


Assuntos
Pneumonias Intersticiais Idiopáticas/complicações , Neoplasias Pulmonares/mortalidade , Neoplasias Pulmonares/cirurgia , Procedimentos Cirúrgicos Torácicos/mortalidade , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/patologia , Idoso , Análise de Variância , Feminino , Previsões , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Período Pós-Operatório , Estudos Retrospectivos , Fatores de Tempo , Tomografia Computadorizada por Raios X
8.
Vet J ; 243: 48-54, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30606439

RESUMO

Interstitial lung diseases (ILDs), also called diffuse parenchymal lung diseases, are a large heterogenous group of non-infectious, non-neoplastic disorders characterized by varied patterns of inflammation and fibrosis (Travis et al., 2002). In humans, accurate classification of interstitial lung diseases (ILDs) requires multidisciplinary collaboration between clinicians, radiologists and pathologists. The same is likely to be true for canine and feline ILDs; however, this collaborative approach is rarely taken, leading to a paucity of knowledge of ILDs in small animal species. A proposed classification scheme of canine and feline ILDs, modified from a human classification scheme, consists of three major groups: idiopathic interstitial pneumonias (IIPs), ILDs secondary to known causes, and miscellaneous ILDs (Travis et al., 2002). The focus of this review is on the IIPs in dogs and cats. A framework of what is known about the major IIPs in humans will be used to draw parallels when relevant to the canine and feline species. Differences will also be highlighted. When available from the veterinary literature, clinical presentation, diagnostic results, treatment and/or prognosis will be reported. The review underscores that to advance in our knowledge of veterinary IIPs and other ILDs, clinicopathologic features, advanced imaging and histopathology must be carefully integrated and larger groups of animals studied.


Assuntos
Doenças do Gato , Doenças do Cão , Pneumonias Intersticiais Idiopáticas/veterinária , Animais , Doenças do Gato/diagnóstico , Doenças do Gato/diagnóstico por imagem , Doenças do Gato/terapia , Gatos , Doenças do Cão/diagnóstico , Doenças do Cão/diagnóstico por imagem , Doenças do Cão/terapia , Cães , Pneumonias Intersticiais Idiopáticas/diagnóstico , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/terapia , Prognóstico
9.
Respir Res ; 19(1): 153, 2018 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-30111332

RESUMO

BACKGROUND: The clinical-radiographic distinction between idiopathic pulmonary fibrosis (IPF) and non-specific interstitial pneumonia (NSIP) is challenging. We sought to investigate the gene expression profiles of IPF and NSIP vs. normal controls. METHODS: Gene expression from explanted lungs of patients with IPF (n = 22), NSIP (n = 10) and from normal controls (n = 11) was assessed. Microarray analysis included Significance Analysis of Microarray (SAM), Ingenuity Pathway, Gene-Set Enrichment and unsupervised hierarchical clustering analyses. Immunohistochemistry and serology of proteins of interest were conducted. RESULTS: NSIP cases were significantly enriched for genes related to mechanisms of immune reaction, such as T-cell response and recruitment of leukocytes into the lung compartment. In IPF, in contrast, these involved senescence, epithelial-to-mesenchymal transition, myofibroblast differentiation and collagen deposition. Unlike the IPF group, NSIP cases exhibited a strikingly homogenous gene signature. Clustering analysis identified a subgroup of IPF patients with intermediate and ambiguous expression of SAM-selected genes, with the interesting upregulation of both NSIP-specific and senescence-related genes. Immunohistochemistry for p16, a senescence marker, on fibroblasts differentiated most IPF cases from NSIP. Serial serum levels of periostin, a senescence effector, predicted clinical progression in a cohort of patients with IPF. CONCLUSIONS: Comprehensive gene expression profiling in explanted lungs identifies distinct transcriptional profiles and differentially expressed genes in IPF and NSIP, supporting the notion of NSIP as a standalone condition. Potential gene and protein markers to discriminate IPF from NSIP were identified, with a prominent role of senescence in IPF. The finding of a subgroup of IPF patients with transcriptional features of both NSIP and senescence raises the hypothesis that "senescent" NSIP may represent a risk factor to develop superimposed IPF.


Assuntos
Perfilação da Expressão Gênica/métodos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/genética , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/genética , Transcrição Genética/genética , Adulto , Idoso , Feminino , Humanos , Pulmão/patologia , Masculino , Pessoa de Meia-Idade
10.
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
11.
Respir Investig ; 56(1): 40-47, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29325680

RESUMO

BACKGROUND: The purpose of this study was to clarify the clinico-radio-pathological characteristics and prognostic factors of unclassifiable-idiopathic interstitial pneumonias (U-IIPs) diagnosed by surgical lung biopsy. METHODS: Among 86 patients with interstitial pneumonia who underwent surgical lung biopsy from January 2005 to September 2013, 33 (38.4%; 16 male patients; mean age, 64.4 ± 8.8 years) were diagnosed with U-IIPs. They were subsequently categorized into rapidly progressive (n = 7), slowly progressive (n = 7), and stable (n = 19) groups based on the decrease of the percent predicted forced vital capacity or percent predicted diffusing capacity of the lung carbon monoxide and the occurrence of acute exacerbation. The clinico-radio-pathological features and survival rates of the patients who were followed up for at least 3 years were examined. These cases were reevaluated retrospectively by multidisciplinary discussion. RESULTS: The rapidly progressive group had a significantly poorer prognosis than that of the other groups (p < 0.0001). Although there were no significant pattern differences on the chest high-resolution computed tomography, the fibrosis scores were significantly higher in the rapidly progressive group (p = 0.002). Furthermore, the percentage of fibroblastic foci assessed by the pathological analysis was also significantly higher in the rapidly progressive group (p = 0.006). Nine (27.3%) patients developed connective tissue diseases during follow-up. CONCLUSIONS: The radiologic patterns were not significantly different among the three clinical U-IIPs subgroups. Nevertheless, our findings suggested that the fibrosis scores and the percentage of fibroblastic foci could provide a prognostic assessment in U-IIPs.


Assuntos
Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/patologia , Pulmão/patologia , Idoso , Progressão da Doença , Feminino , Fibrose , Seguimentos , Humanos , Pneumonias Intersticiais Idiopáticas/classificação , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Tempo , Tomografia Computadorizada por Raios X
12.
Intern Med ; 57(7): 1039-1043, 2018 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-29269659

RESUMO

We describe a case of a woman who presented with a persistent cough, general fatigue, and a fever. Interstitial lung disease was rapidly progressive and resistant to high-dose steroid therapy. She tested positive for the presence of anti-melanoma differentiation-associated gene 5 (MDA-5) antibody, although she had no skin manifestations of dermatomyositis. She was eventually diagnosed with unclassifiable idiopathic interstitial pneumonia and was successfully treated with intensive immunosuppressive therapy including intravenous cyclophosphamide. To our knowledge, this is the first report of anti-MDA-5 antibody in a patient with idiopathic interstitial pneumonia.


Assuntos
Anticorpos/genética , Ciclofosfamida/uso terapêutico , Pneumonias Intersticiais Idiopáticas/tratamento farmacológico , Fibrose Pulmonar Idiopática/tratamento farmacológico , Imunossupressores/uso terapêutico , Melanoma/genética , Melanoma/imunologia , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/complicações , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Pessoa de Meia-Idade , Resultado do Tratamento
13.
Respirology ; 23(5): 507-511, 2018 May.
Artigo em Inglês | MEDLINE | ID: mdl-29178216

RESUMO

BACKGROUND AND OBJECTIVE: Idiopathic pulmonary fibrosis (IPF) is increasingly diagnosed by clinical and computed tomography (CT) criteria; however, surgical lung biopsy (SLB) may still be required in patients who lack definite CT features of usual interstitial pneumonia (UIP). We reviewed a cohort of elderly patients who underwent SLB, to evaluate the benefit of SLB in diagnosing idiopathic interstitial pneumonia (IIP). METHODS: We searched the pathology records of Mayo Clinic for ambulatory patients at least 75 years old, who underwent SLB between 2000 and 2012 for indeterminate IIP. Histologic slides were reviewed and clinical data were extracted from the record. RESULTS: A total of 55 patients (35 male) were enrolled. Median (interquartile range) age was 77 (76-80) years. Forced vital capacity was 70 (61-76)% and diffusing capacity of the lungs for carbon monoxide was 48 (42-54)% of predicted. In total, 37 (67%) patients had IPF, including 61% of those with HRCT findings inconsistent with UIP. Thirty-day mortality was 10% and 90-day mortality was 15%. CONCLUSION: The high mortality rate of SLB complicates the risk-benefit analysis in elderly patients with IIP. The expected value of the SLB is probably highest when the HRCT features are inconsistent with UIP, due to the frequent (39%) retrieval of patterns other than UIP.


Assuntos
Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/patologia , Pulmão/patologia , Idoso , Idoso de 80 Anos ou mais , Biópsia/mortalidade , Monóxido de Carbono , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/patologia , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Fibrose Pulmonar Idiopática/fisiopatologia , Masculino , Capacidade de Difusão Pulmonar , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Capacidade Vital
14.
J Int Med Res ; 46(1): 448-456, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28758849

RESUMO

Objective Unclassifiable interstitial lung disease (ILD) is a common problem in clinical practice. These patients pose a distinct challenge with regard to appropriate evaluation and management. We investigated the clinical features and prognosis of unclassifiable ILD and compared its clinical profile with that of idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia (NSIP). Methods Patients with IPF (n = 40), NSIP (n = 14), and unclassifiable ILD (n = 27) were selected from an ongoing database. Baseline clinical features, pulmonary function, and the extent of fibrosis on high-resolution computed tomography (HRCT) were evaluated. Mortality was estimated based on the ILD-Gender, Age, Physiology (ILD-GAP) index and composite physiologic index (CPI). Results IPF was associated with the worst survival (hazard ratio [HR] = 4.361 compared with NSIP), followed by unclassifiable cases (HR = 1.251 compared with NSIP). Increasing mortality was significantly impacted by age (HR = 1.04 per 1-year increase), lower carbon monoxide diffusing capacity of the lung (HR = 0.97), HRCT interstitial score (HR = 1.119 per 1-point increase), ILD-GAP score (HR = 1.570 per 1-point increase), and CPI (HR = 1.039 per 1-point increase). Conclusions Patients with unclassifiable ILD had an intermediate prognosis between that of IPF and NSIP. Patients at high risk of mortality can be identified using baseline clinical, physiological, and radiological features.


Assuntos
Pneumonias Intersticiais Idiopáticas/fisiopatologia , Fibrose Pulmonar Idiopática/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/mortalidade , Pneumonias Intersticiais Idiopáticas/patologia , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/patologia , Pulmão/patologia , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Testes de Função Respiratória , Estudos Retrospectivos , Índice de Gravidade de Doença , Análise de Sobrevida , Tomografia Computadorizada por Raios X
15.
Sci Rep ; 7(1): 16912, 2017 12 05.
Artigo em Inglês | MEDLINE | ID: mdl-29208971

RESUMO

Nonspecific interstitial pneumonia (NSIP) is characterised by interstitial infiltration of lymphocytes and varying amounts of interstitial fibrosis. B cells have been suggested to contribute to the pathogenesis of NSIP. However, the relationship between B-lymphocyte and the clinical outcomes of NSIP was unclear. In this study, 50 patients with histopathologically confirmed NSIP from Peking Union Medical College Hospital between April 2003 to December 2012 were retrospectively analyzed. Using immunohistochemical analyses, CD20+ B cells were counted in the lymphoid follicles, perivascular, interstitial, and peribronchiolar regions of lung tissure. The CD20+ lymphocytes were mainly present in the lymphoid follicles. The number of follicular CD20+ lymphocytes was higher in the fibrosing than cellular NSIP pattern [255.08 (132.92-449.71) vs. 121.33 (63.54-282.88)/0.1 mm2, p = 0.017]. After 1 year of therapy, the follicular CD20+ lymphocytes were significantly higher in patients whose forced vital capacity (FVC) worsened as compared to those who improved (p = 0.014). Additionally, follicular CD20+ lymphocytes were negatively correlated with the post-treatment percentage change in FVC (rho = -0.397, p = 0.004). However, follicular CD20+ lymphocytes were not correlated with survival. These results suggested that pulmonary follicular CD20+ lymphocytes were correlated with the fibrosing pattern of NSIP and predicted less clinical improvement after treatment.


Assuntos
Antígenos CD20/metabolismo , Linfócitos B/patologia , Pneumonias Intersticiais Idiopáticas/patologia , Adulto , Idoso , Linfócitos B/metabolismo , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/mortalidade , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Receptores de Lipopolissacarídeos/metabolismo , Pulmão/patologia , Tecido Linfoide/patologia , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X , Capacidade Vital
16.
Respir Med ; 132: 189-194, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-29229096

RESUMO

BACKGROUND: Anti-aminoacyl-tRNA synthetase (ARS) antibodies have been detected in patients with polymyositis/dermatomyositis (PM/DM) and are especially correlated with interstitial lung disease (ILD). The aim of this study was to clarify the clinical features of patients with anti-ARS antibody positive idiopathic interstitial pneumonias (IIPs). METHODS: Patients were classified into three groups: 1) IIP with anti-ARS antibodies (ARS(+)IIP), 2) IIP without anti-ARS antibodies (ARS(-)IIP), and 3) PM/DM-associated ILD with anti-ARS antibodies (ARS(+)PM/DM-ILD). Clinical characteristics were compared retrospectively between the ARS(+)IIP group and the ARS(-)IIP group or ARS(+)PM/DM-ILD group. RESULTS: Eighteen ARS(+)IIP, 284 ARS(-)IIP, and 20 ARS(+)PM/DM-ILD patients were enrolled. The ARS(+)IIP group was significantly older and the male sex was predominant, had a lower prevalence of signs of connective tissue disease, differences in HRCT findings and patterns, and higher KL-6 levels compared to the ARS(+)PM/DM-ILD group. The findings in the bronchoalveolar lavage fluid (BALF) showing lymphocytosis and a lower CD4/CD8 ratio were similar between the two groups. However, the ARS(+)IIP group had significantly lower percentage of sputum, higher prevalence of mechanic's hand, higher KL-6 levels, lower percentage of vital capacity in the pulmonary function test, and lower CD4/CD8 ratio in BALF, compared to the ARS(-)IIP group. CONCLUSIONS: The present study demonstrated that features of pulmonary involvement were similar to those in the ARS(+)PM/DM-ILD group; however, some differences including HRCT findings and higher KL-6 levels suggest that ARS(+)IIP has severe ILD compared with ARS(+)PM/DM-ILD. Further prospective studies with a larger number of patients will elucidate the exact role of anti-ARS antibodies in IIPs.


Assuntos
Aminoacil-tRNA Sintetases/imunologia , Autoanticorpos/imunologia , Pneumonias Intersticiais Idiopáticas/imunologia , Fatores Etários , Idoso , Líquido da Lavagem Broncoalveolar/citologia , Relação CD4-CD8 , Doenças do Tecido Conjuntivo/epidemiologia , Dermatomiosite/epidemiologia , Dermatomiosite/imunologia , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/epidemiologia , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Mucina-1 , Estudos Retrospectivos , Fatores Sexuais , Tomografia Computadorizada por Raios X , Capacidade Vital
17.
Medicine (Baltimore) ; 96(19): e6900, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28489801

RESUMO

RATIONAL: Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease that is characterized radiologically by apical pleural thickening and histologically by elastic fibrosis of the visceral pleura. Although PPFE cases have been reported occasionally since this disease was initially described, most such cases have involved secondary PPFE. Idiopathic PPFE (iPPFE) cases have been less thoroughly studied. There are no effective medications for patients with iPPFE. PATIENT CONCERNS: A 34-year-old man with no asbestos or cigarette exposure was admitted to our ward due to worsening cough and exertional dyspnea for 10 years. He had a "flattened thoracic cage" and bibasilar inspiratory crackles without finger clubbing. A series of chest computed tomography scans during the preceding 10 years revealed the presence of gradual, exaggerated, upper lung-predominant, diffuse pleural thickening and dense subpleural opacification with traction bronchiectasis. DIAGNOSIS: He was performed with video-assisted thoracic surgical (VATS) lung biopsy. The pulmonary histopathologic examination showed thickened visceral pleura and prominent subpleural fibroelastosis, confirming the diagnosis of iPPFE. INTERVENTION: After the failure of treatment with prednisone plus cyclophosphamide and sequential pirfenidone administration, he was arranged with bilateral lung transplantation two years later. OUTCOMES: The patient did not require supplemental oxygenation anymore after he recovered from lung transplantation. LESSONS: Bilateral lung transplantation might be tried for the end-stage iPPFE cases.


Assuntos
Pneumonias Intersticiais Idiopáticas/cirurgia , Transplante de Pulmão , Adulto , Diagnóstico Diferencial , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/patologia , Pulmão/diagnóstico por imagem , Pulmão/patologia , Pulmão/cirurgia , Masculino , Pleura/patologia
18.
Rom J Morphol Embryol ; 58(1): 261-265, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28523329

RESUMO

Cigarette smoking is the most frequently encountered risk factor for chronic obstructive pulmonary disease and lung cancer. The latest American Thoracic Society÷European Respiratory Society classification of idiopathic interstitial pneumonia includes two entities related to smoking habits: respiratory bronchiolitis-associated interstitial lung disease and desquamative interstitial pneumonia. The new approach to diagnosis is to combine pathological pattern with clinical and radiological data. Lung biopsy is no longer considered the "gold standard" for diagnosis, but as a part of the diagnosis, which shall be set only after the pulmonologist, radiologist and pathologist reviewed all clinical, imaging and pathological aspects. We report a case of a young male, who complained of respiratory symptoms, had normal volumes and flows on lung function tests, moderately reduced transfer factor for carbon monoxide and "ground glass" attenuation on high-resolution computed tomography. Because the patient had exposure to contact with parrots, hypersensitivity pneumonitis was considered, but the broncho-alveolar lavage was without lymphocytosis. Open lung biopsy confirmed the diagnosis of respiratory bronchiolitis-associated interstitial lung disease, a rarely described entity in the medical literature. The patient had a good clinical outcome after smoking cessation.


Assuntos
Bronquiolite/complicações , Pneumonias Intersticiais Idiopáticas/complicações , Adulto , Bronquiolite/diagnóstico por imagem , Bronquiolite/patologia , Diagnóstico Diferencial , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/patologia , Pulmão/diagnóstico por imagem , Pulmão/patologia , Macrófagos/patologia , Masculino , Fibrose Pulmonar/complicações , Fibrose Pulmonar/diagnóstico por imagem , Fibrose Pulmonar/patologia , Tomografia Computadorizada por Raios X
19.
J Med Invest ; 64(1.2): 110-116, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28373606

RESUMO

OBJECTIVE: Idiopathic interstitial pneumonias (IIPs) are a group of heterogeneous diffuse parenchymal lung disorders of unknown etiology. An acute exacerbation (AE) is an acute respiratory deterioration that occurs in IIPs. The prognosis of AE of IIPs (AE-IIPs) is extremely severe; however, no established therapies exist. We aimed to evaluate the efficacy of leukocytapheresis (LCAP) to treat patients with AE-IIPs. PATIENTS AND METHODS: Six chronic IIPs patients who developed AE were enrolled in this study. We performed LCAP on days 2, 3, 9 and 10 in all six patients. All patients were also treated with high-dose corticosteroids and a continuous administration of low-molecular-weight heparin. We observed 30-day survival after the diagnosis of AE to evaluate the efficacy of LCAP. We also assessed oxygenation, high-resolution computed tomography (HRCT) findings, and certain chemical mediators in the peripheral blood. RESULTS: Five of six patients survived more than 30 days. One patient died of progressive respiratory failure. Oxygenation and HRCT findings tended to improve in all survivors. The serum levels of lactate dehydrogenase, high mobility group box-1, and interleukin-18 were significantly decreased statistically post-LCAP. No severe adverse events occurred. CONCLUSION: We suggest that LCAP is a safe and effective therapy for treating patients with AE-IIPs. J. Med. Invest. 64: 110-116, February, 2017.


Assuntos
Pneumonias Intersticiais Idiopáticas/terapia , Leucaférese , Doença Aguda , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/sangue , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Fibrose Pulmonar Idiopática/terapia , Mediadores da Inflamação/sangue , Masculino , Projetos Piloto
20.
Adv Respir Med ; 85(1): 46-50, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28198994

RESUMO

Interstitial pneumonia with autoimmune features (IPAF) is a term to describe individuals with both interstitial lung disease (ILD) and combinations of other clinical, serologic, and/or pulmonary morphologic features, which presumably originate from an underlying systemic autoimmune condition, but do not meet current rheumatologic criteria for a defined connective tissue disease (CTD). Predominantly, interstitial pneumonia arises in the course of an established CTD, but it is not so rare for the ILD to be the first, and possibly the one and only manifestation of a latent CTD. Lymphocytic Interstitial Pneumonia (LIP) is an uncommon disease, characterized by infiltration of the interstitium and alveolar spaces of the lung by lymphocytes, plasma cells and other lymphoreticular elements. The cause of LIP is still unknown but it could be also a manifestation of CTD. Clinically, it is highly variable, from spontaneous resolution to progressive respiratory failure and death despite glucocorticoid treatment. Since there are no recent standards for the management of LIP, the disease is treated empirically. We report a case of a HIV-negative 54-year-old woman, who was suspected of LIP according to clinical features and radiological findings. Positive laboratory results were highly suggestive of underlying autoimmune process, but did not fulfil the criteria of any particular CTD. Because of severe general condition of the patient, immunosuppressive treatment was started immediately, without further invasive diagnostics including lung biopsy, which is required for a definitive diagnosis. We present two-year observation of the patient with all our doubts concerning clinical proceedings.


Assuntos
Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/complicações , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pneumonias Intersticiais Idiopáticas/tratamento farmacológico , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças do Tecido Conjuntivo/tratamento farmacológico , Feminino , Humanos , Imunossupressores/uso terapêutico , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/dietoterapia , Pessoa de Meia-Idade
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