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1.
Medicine (Baltimore) ; 99(4): e18589, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31977850

RESUMO

To date, there is no clear agreement regarding which is the best method to detect a connective tissue disease (CTD) during the initial diagnosis of interstitial lung diseases (ILD). The aim of our study was to explore the impact of a systematic diagnostic strategy to detect CTD-associated ILD (CTD-ILD) in clinical practice, and to clarify the significance of interstitial pneumonia with autoimmune features (IPAF) diagnosis in ILD patients.Consecutive patients evaluated in an ILD Diagnostic Program were divided in 3 groups: IPAF, CTD-ILD, and other ILD forms. Clinical characteristics, exhaustive serologic testing, high resolution computed tomography (HRCT) images, lung biopsy specimens, and follow-up were prospectively collected and analyzed.Among 139 patients with ILD, CTD was present in 21 (15.1%), 24 (17.3%) fulfilled IPAF criteria, and 94 (67.6%) were classified as other ILD forms. Specific systemic autoimmune symptoms such as Raynaud phenomenon (19%), inflammatory arthropathy (66.7%), and skin manifestations (38.1%) were more frequent in CTD-ILD patients than in the other groups (all P < .001). Among autoantibodies, antinuclear antibody was the most frequently found in IPAF (42%), and CTD-ILD (40%) (P = .04). Nonspecific interstitial pneumonia, detected by HRCT scan, was the most frequently seen pattern in patients with IPAF (63.5%), or CTD-ILD (57.1%) (P < .001). In multivariate analysis, a suggestive radiological pattern by HRCT scan (odds ratio [OR] 15.1, 95% confidence interval [CI] 4.7-48.3, P < .001) was the strongest independent predictor of CTD-ILD or IPAF, followed by the presence of clinical features (OR 14.6, 95% CI 4.3-49.5, P < .001), and serological features (OR 12.4, 95% CI 3.5-44.0, P < .001).This systematic diagnostic strategy was useful in discriminating an underlying CTD in patients with ILD. The defined criteria for IPAF are fulfilled by a considerable proportion of patients referred for ILD.


Assuntos
Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/diagnóstico , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Autoanticorpos/imunologia , Biópsia , Doenças do Tecido Conjuntivo/patologia , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X
2.
Cancer Immunol Immunother ; 69(1): 15-22, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31745589

RESUMO

The safety of anti-programmed cell death 1 (PD-1) antibody for patients with preexisting interstitial lung disease (ILD) remains unknown. The aim of this study was to evaluate the dependence of preexisting ILD on anti-PD-1 antibody-induced pneumonitis in non-small cell lung cancer (NSCLC) patients. We retrospectively reviewed the association of preexisting ILD with the incidence, radiographic pattern, and outcome of pneumonitis in NSCLC patients receiving anti-PD-1 antibody. A total of 331 patients were included in this study. Of these patients, 17 had preexisting ILD. The incidence of pneumonitis was higher among the patients with preexisting ILD than among those without preexisting ILD (29% vs. 10%, P = 0.027). The distributions of the CT appearances at the onset of anti-PD-1 antibody-induced pneumonitis were as follows: for the patients with preexisting ILD, two patients (40%) had diffuse alveolar damage (DAD), one patient each with organizing pneumonia-like (OP), hypersensitivity pneumonitis (HP), and other patterns (20% each); for the patients without preexisting ILD, 19 patients (61%) had OP, 8 (26%) had HP, 3 (10%) had DAD, and 1 (3.2%) had other patterns. The median onset time from the initiation of anti-PD-1 antibody treatment until the development of pneumonitis was 1.3 months (range 0.3-2.1 months) for the patients with preexisting ILD and 2.3 months (range 0.2-14.6 months) for the patients without preexisting ILD. Careful attention to the development of pneumonitis is needed, especially within the first 3 months after the start of anti-PD-1 antibody treatment, when using anti-PD-1 antibody to treat patients with preexisting ILD.


Assuntos
Antineoplásicos Imunológicos/efeitos adversos , Carcinoma Pulmonar de Células não Pequenas/tratamento farmacológico , Doenças Pulmonares Intersticiais/complicações , Neoplasias Pulmonares/tratamento farmacológico , Pneumonia/induzido quimicamente , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma Pulmonar de Células não Pequenas/imunologia , Feminino , Humanos , Incidência , Pulmão/diagnóstico por imagem , Pulmão/imunologia , Doenças Pulmonares Intersticiais/imunologia , Neoplasias Pulmonares/imunologia , Masculino , Pessoa de Meia-Idade , Pneumonia/epidemiologia , Pneumonia/imunologia , Receptor de Morte Celular Programada 1/antagonistas & inibidores , Receptor de Morte Celular Programada 1/imunologia , Estudos Retrospectivos , Fatores de Tempo
3.
Autoimmun Rev ; 19(2): 102451, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31838159

RESUMO

BACKGROUND AND OBJECTIVE: Recently the term "interstitial pneumonia with autoimmune features" (IPAF) has been proposed to identify patients with interstitial lung disease and autoimmune characteristics, not fulfilling the criteria for specific connective tissue diseases (CTD). Only few data are available about the clinical and serological features of IPAF patients, their survival and the possible evolution in a CTD. The aims of the study were to investigate the demographic and clinico-serologic features of patients with IPAF, their relationship to survival, and the possible evolution in a definite CTD. PATIENTS AND METHODS: Fifty-two patients were consecutively enrolled and prospectively followed for 45 ± 31.6 months. Data about disease onset, serological, clinical and therapeutic features, pulmonary function tests and high-resolution computed tomography were periodically repeated. The survival of patients with IPAF was compared with that of 104 patients with idiopathic pulmonary fibrosis (IPF). RESULTS: The clinical domain for IPAF was satisfied in 44 patients, serological domain in 49 and the morphological domain in 29 patients. During the follow-up, a definite CTD was diagnosed in 7 patients, in particular Sjogren's syndrome in 4 patients, rheumatoid arthritis in 2, and polymyositis in the last. The estimated 5-year survival of IPAF patients 69.5 ± 7.8%, significantly higher than survival observed in IPF patients, and the baseline value of FVC and DLCO were the only factors associated to death. CONCLUSIONS: IPAF seems to a distinct entity, with a low tendency to evolve in a definite CTD. Nevertheless, further studies are needed to better define the clinical evolution and the outcome of IPAF.


Assuntos
Doenças Autoimunes/complicações , Fibrose Pulmonar Idiopática/complicações , Doenças Pulmonares Intersticiais/complicações , Idoso , Doenças Autoimunes/patologia , Feminino , Seguimentos , Humanos , Fibrose Pulmonar Idiopática/patologia , Doenças Pulmonares Intersticiais/patologia , Masculino , Estudos Prospectivos
4.
Autoimmun Rev ; 19(2): 102453, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31838163

RESUMO

INTRODUCTION: The effectiveness of CD20 targeting in connective tissue diseases (CTD) with lung involvement is controversial. This paper aims to review the current evidence about rituximab (RTX) use in CTD-related interstitial lung disease (ILD). METHODS: We performed a systematic review of papers published between January 2009 and May 2019. We included clinical trials, case/control studies and cohort studies. We excluded letters, case reports, case series, reviews, and full articles when not in English. The selected studies listed as primary or secondary outcome a variation in pulmonary function tests or in the scores used to radiologically stage lung involvement, in CTD-related ILD patients after RTX. RESULTS: Out of 1206 potentially eligible articles, 24 papers were selected: 3 retrospectively described cohorts of patients with different CTD, 14 dealt with systemic sclerosis (SSc)-related ILD, 5 with idiopathic inflammatory myopathies (IIMs)-related ILD, and 2 with Sjögren's Syndrome-related ILD. A direct comparison of the selected studies was hampered by their heterogeneity for outcomes, follow-up duration, the severity of lung involvement, and clinical features of study populations. However, an overall agreement existed concerning the effectiveness of RTX in the stabilization of lung disease, with some studies reporting an improvement of functional parameters from baseline. IIM-related ILD appeared more responsive than other CTD-related ILD to CD20 targeting. CONCLUSION: RTX is a promising therapeutic tool in CTD-related ILD. This systematic review remarks the unmet need of multicenter prospective studies aiming to evaluate the effectiveness of RTX with adequate sample size and study design.


Assuntos
Antígenos CD20/imunologia , Doenças do Tecido Conjuntivo/complicações , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/tratamento farmacológico , Rituximab/imunologia , Rituximab/uso terapêutico , Humanos , Estudos Retrospectivos
5.
Autoimmun Rev ; 19(2): 102447, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31843713

RESUMO

OBJECTIVE: To evaluate the prevalence, clinical presentation, serological and morphological features of, and therapeutic options for Interstitial Lung Disease (ILD) in primary Sjögren's Syndrome (pSS). METHODS: Pubmed was searched between February 1996 and December 2018 using a combination of MESH terms related to pSS and ILD. Selected works were subjected to blind evaluation by two authors and a senior author in case of disagreement. The work followed PRISMA guidelines and was registered on PROSPERO (CRD42018118669). RESULTS: About 20% of pSS patients have ILD, with a 5-y survival of 84% and a need for supplemental oxygen in the 11-33% range. A significant proportion of ILD patients are seronegative without sicca syndrome. ILD seems to be associated with higher levels of Lactic Dehydrogenases and positivity for Anti-Ro52k. The prevalent pattern in High Resolution Computed Tomography is Nonspecific Interstitial Pneumonia (NSIP), but all other patterns can be present. No difference in mortality was found between patients with NSIP and Usual Interstitial Pneumonia patterns. Amyloidosis and primary lung lymphoma can be observed in about 10% of pSS patients. CONCLUSION: The recognition of pSS underlying an ILD can be challenging in seronegative patients with no or mild sicca symptoms. A complete diagnostic assessment, including minor salivary glands and, in some cases, lung biopsy, should be performed on all patients at risk. A better recognition of the clinical or serological markers of ILD progression in these patients is warranted to drive the physicians to an early diagnosis and an effective treatment.


Assuntos
Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/patologia , Síndrome de Sjogren/complicações , Humanos , Itália/epidemiologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Prognóstico , Reumatologia , Sociedades Médicas
7.
Cancer Sci ; 110(12): 3738-3745, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31608537

RESUMO

The prognosis of non-small-cell lung cancer (NSCLC) patients with interstitial lung disease (ILD) is poor, and 5%-20% of those receiving chemotherapy experience ILD exacerbation. To evaluate the safety and efficacy of nab-paclitaxel plus carboplatin for NSCLC patients with ILD, we undertook a multicenter phase II study. Chemotherapy-naïve patients with advanced NSCLC and mild or moderate ILD received nab-paclitaxel (100 mg/m2 , days 1, 8, and 15) plus carboplatin (area under the curve = 6, day 1) every 3 weeks for 4 cycles (maximum, 6 cycles). Interstitial lung diseases were diagnosed based on criteria for fibrosing interstitial pneumonia. The primary endpoint was the prevalence of exacerbation-free ILD 28 days after completion of protocol treatment. Secondary endpoints were response rate, progression-free survival, overall survival, prevalence of exacerbation-free ILD, and toxicity. Ninety-four patients were enrolled, and 92 patients received any protocol treatment. Median age was 70 years, and 58% had nonsquamous histology. In the primary analysis, the prevalence of exacerbation-free ILD 28 days after protocol treatment was 95.7% (88/92; 90% confidence interval, 90.3-98.5), which met the primary endpoint. Response rate was 51% (95% confidence interval, 40%-62%). At the time of data cut-off, median progression-free survival was 6.2 months, and median overall survival was 15.4 months. The most common grade 3/4 adverse events were neutropenia (75%), leukopenia (53%), anemia (48%), and thrombocytopenia (20%). Two treatment-related deaths (1 each of pulmonary infection and ILD exacerbation) were observed. This study showed that a combination of nab-paclitaxel with carboplatin was tolerable in NSCLC patients with mild or moderate ILD in terms of safety. This study is registered at the University Hospital Medical Information Network (UMIN) Clinical Trial Registry (UMIN 000012989).


Assuntos
Carcinoma Pulmonar de Células não Pequenas/tratamento farmacológico , Doenças Pulmonares Intersticiais/complicações , Neoplasias Pulmonares/tratamento farmacológico , Idoso , Idoso de 80 Anos ou mais , Albuminas/administração & dosagem , Albuminas/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Carboplatina/administração & dosagem , Carboplatina/efeitos adversos , Carcinoma Pulmonar de Células não Pequenas/mortalidade , Feminino , Humanos , Doenças Pulmonares Intersticiais/epidemiologia , Neoplasias Pulmonares/mortalidade , Masculino , Pessoa de Meia-Idade , Paclitaxel/administração & dosagem , Paclitaxel/efeitos adversos , Estudos Prospectivos
8.
Med Sci Monit ; 25: 7763-7769, 2019 Oct 16.
Artigo em Inglês | MEDLINE | ID: mdl-31618191

RESUMO

BACKGROUND Pulmonary hypertension is a common complication of interstitial lung disease. This study was conducted to retrospectively analyze the incidence of pulmonary hypertension among interstitial lung disease patients and the correlation between systolic pulmonary artery pressure (PASP) and pulmonary functions. We also intended to investigate whether antinuclear antibody (ANA) could be an effective indicator of pulmonary hypertension. MATERIAL AND METHODS There were 182 patients diagnosed with interstitial lung disease through high-resolution computed tomography (HRCT). Pulmonary hypertension was defined as an increase of mean pulmonary arterial pressure (PAPm) ≥25 mmHg (~PASP ≥40 mmHg) at rest. Severe pulmonary hypertension was defined as PAPm ≥35 mmHg. There were 104 cases including in this study. There were 67 cases from the ANA positive (ANA+) group and 37 cases from the ANA negative (ANA-) group. All study patients had pulmonary function tests, which included the measurements of maximal voluntary ventilation (MVV), residual volume (RV), total lung capacity (TLC), forced expiratory volume (FVC), vital capacity (VC), and diffusing capacity of the lungs for carbon monoxide (DLCO). RESULTS The pulmonary hypertension incidence in the study cohort was 25%, and the severe pulmonary hypertension incidence was 6.48%. The incidence of pulmonary hypertension in ANA+ cases was 22.22%. The incidence of pulmonary hypertension in the ANA- cases was 32.14%. The lung function test results showed moderate relationships between DLCO, FVC%, VC%, and PASP; no relationship between MVV, FEV1/FVC%, RV/TLC, and PASP; minimum relationship between FVC%, VC%, and PASP in the ANA+ group; and moderate relationship between FVC%, VC%, and PASP in the ANA- group. CONCLUSIONS Pulmonary hypertension occurred in 25% of the 182 interstitial lung disease patients and was negatively associated with deteriorated lung functions (specifically VC%, FVC%, and DLCO parameters). ANA level was not associated with the prognosis of pulmonary hypertension of patients with interstitial lung disease, and it did not significantly affect the correlation between PASP and pulmonary functions. Thus, ANA level did not seem to be a necessary indicator of pulmonary hypertension, and a more effective treatment method for pulmonary hypertension of patients with interstitial lung disease is urgently needed.


Assuntos
Hipertensão Pulmonar/epidemiologia , Doenças Pulmonares Intersticiais/epidemiologia , Idoso , Anticorpos Antinucleares/análise , Biomarcadores/sangue , Pressão Sanguínea/fisiologia , China/epidemiologia , Estudos de Coortes , Feminino , Volume Expiratório Forçado , Humanos , Hipertensão Pulmonar/sangue , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Doenças Pulmonares Intersticiais/sangue , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Testes de Função Respiratória/métodos , Estudos Retrospectivos , Capacidade Vital
10.
Skinmed ; 17(3): 211-214, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31496481

RESUMO

A 68-year-old Indian man presented with a pruritic eruption on his neck, back, elbows, knees, and the dorsum of his hands. He was initially treated for possible Lyme's disease by his primary care physician, but without improvement. Then he developed daily chills and fevers up to 101 °F, as well as shortness of breath. A chest radiograph showed patchy airspace opacities suggestive of atypical pneumonia, and the patient was treated with levofloxacin and prednisone. Although prednisone diminished the eruption, the patient continued to experience fever, malaise, and generalized weakness, at which point he was hospitalized. Blood cultures and an antinuclear antibodies (ANA) were negative and extensive lab workup was only notable for an elevated erythrocyte sedimentation rate (ESR) (63 mm/hr, Reference Range 0-22), mild transaminitis (AST 77 U/L, Reference Range 10-40), hyponatremia (131 mEq/L, Reference Range 135-145) and elevated ferritin (440, Reference Range 20-500). The patient was discharged on 20 mg of prednisone, with referral to rheumatology and dermatology for possible autoimmune diseases.


Assuntos
Autoanticorpos/sangue , Dermatomiosite/complicações , Dermatomiosite/imunologia , Helicase IFIH1 Induzida por Interferon/imunologia , Doenças Pulmonares Intersticiais/complicações , Idoso , Biópsia , Dermatomiosite/patologia , Humanos , Masculino , Pele/patologia
11.
Med Sci Monit ; 25: 6474-6481, 2019 Aug 29.
Artigo em Inglês | MEDLINE | ID: mdl-31462627

RESUMO

BACKGROUND The objective of this study was to assess the diagnostic value of platelet/lymphocyte ratio (PLR), lymphocyte/monocyte ratio (LMR), and neutrophil/lymphocyte ratio (NLR) as biomarkers in patients with rheumatoid arthritis (RA) and rheumatoid arthritis-associated interstitial lung disease (RA-ILD). MATERIAL AND METHODS Demographic and laboratory data were acquired for 198 RA and 103 RA-ILD patients and 290 healthy controls. The subjects were categorized into female and male groups and further subcategorized based on age into <60 years and ≥60 years subgroups. One-way analysis of variance (ANOVA), receiver operating characteristics (ROC), Pearson analysis, multiple linear regression analysis, and logistic regression analysis were performed to analyze the association of PLR, NLR, and LMR with RA and RA-ILD. RESULTS Mean PLR and NLR were lowest in the control group, followed by the RA and RA-ILD groups (p<0.05). Mean LMR was lowest in the RA-ILD group, followed by the RA and control groups (p<0.05). The area under the ROC (AUROC) values of the PLR to distinguish between RA and controls, RA-ILD and controls, and RA-ILD and RA were 0.676, 0.776, and 0.650, respectively (p<0.001). Multiple linear regression analysis suggested a significantly positive association between the level of PLR and the level of DAS28 (p<0.001). The odds ratio of PLR was 1.101 for RA (p=0.023) and 1.217 for RA-ILD (p<0.001) when compared to the controls. CONCLUSIONS PLR may be applied as a new biomarker for predicting and diagnosing RA and RA-ILD and for distinguishing RA-ILD patients from RA patients and healthy subjects.


Assuntos
Artrite Reumatoide/patologia , Biomarcadores/sangue , Plaquetas/patologia , Doenças Pulmonares Intersticiais/patologia , Linfócitos/patologia , Monócitos/patologia , Neutrófilos/patologia , Artrite Reumatoide/sangue , Artrite Reumatoide/complicações , Feminino , Humanos , Modelos Logísticos , Doenças Pulmonares Intersticiais/sangue , Doenças Pulmonares Intersticiais/complicações , Masculino , Pessoa de Meia-Idade , Curva ROC
12.
Intern Med ; 58(21): 3083-3086, 2019 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-31292378

RESUMO

Objective Chest physicians often encounter patients with interstitial pneumonia with autoimmune features. However, there have so far been few reports of patients presenting with concurrent immune thrombocytopenia (ITP) and interstitial pneumonia. The prevalence of interstitial pneumonia in patients with ITP is less well known. Methods We surveyed patients diagnosed with ITP and interstitial pneumonia at the departments of Hematology and Respiratory Medicine to evaluate the association between these diseases. Results Among 73 patients with ITP, 7 patients (9.6%) presented with interstitial pneumonia, including 4 patients (2%) who developed ITP in the course of 204 patients with interstitial pneumonia. All 7 patients were men. Four patients were positive for some autoantibodies. Two patients had autoimmune diseases other than ITP. There were significant differences in age and gender between the ITP patients with and without interstitial pneumonia. Conclusion The present study suggests the possibility that the development of ITP, other autoimmune diseases, and interstitial pneumonia may be mutually associated. Advanced age and male sex in ITP may be significant predisposing factors for interstitial pneumonia. Clinicians should be aware of the potential for the coexistence of these diseases.


Assuntos
Autoanticorpos/sangue , Doenças Pulmonares Intersticiais/complicações , Púrpura Trombocitopênica Idiopática/complicações , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Doenças Autoimunes/complicações , Feminino , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Doenças Pulmonares Intersticiais/imunologia , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/diagnóstico por imagem , Púrpura Trombocitopênica Idiopática/imunologia , Fatores de Risco , Fatores Sexuais , Tomografia Computadorizada por Raios X
13.
Medicine (Baltimore) ; 98(26): e16086, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31261523

RESUMO

Pleuroparenchymal fibroelastosis (PPFE) is a rare new interstitial lung disease (ILD) characterized by the fibrotic thickening of the visceral pleura and subadjacent parenchymal areas of the upper lobes This study reveals that patients with ILD-SSc associated with chest HRCT evidence of PPFE require close and recurrent follow-up with periodic evaluation of lung function parameters, DLCO and chest HRCT. Rheumatologists should be aware of this new radiological finding which is accompanied by a negative prognosis, especially when associated with a progressive course. Patients with this radiological pattern need to be monitored with particular attention.


Assuntos
Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pleurais/complicações , Doenças Pleurais/diagnóstico por imagem , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico por imagem , Idoso , Feminino , Seguimentos , Humanos , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/terapia , Masculino , Doenças Pleurais/terapia , Prognóstico , Fibrose Pulmonar/complicações , Fibrose Pulmonar/diagnóstico por imagem , Fibrose Pulmonar/terapia , Estudos Retrospectivos , Reumatologistas , Escleroderma Sistêmico/terapia
14.
Intern Med ; 58(14): 2063-2066, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31308343

RESUMO

A 71-year-old woman with congenital rubella syndrome (CRS) presented with prolonged cough. No physical findings suggested the presence of any connective tissue diseases. Chest computed tomography showed ground-glass opacities and consolidations in the bilateral lower lobes. She had elevated serum Krebs von den Lungen-6, hypoxemia and positive serum anti-Jo-1 antibody. Bronchoalveolar lavage fluid revealed lymphocytosis with a decreased CD4/CD8 ratio. A transbronchial lung biopsy specimen revealed organizing pneumonia. Based on a diagnosis of interstitial pneumonia with autoimmune features (IPAF), systemic corticosteroids were administered, and a good outcome was obtained. A possible relationship between CRS and IPAF is herein discussed.


Assuntos
Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Síndrome da Rubéola Congênita/complicações , Idoso , Anticorpos Antinucleares/imunologia , Líquido da Lavagem Broncoalveolar/imunologia , Tosse , Feminino , Humanos , Pulmão/patologia , Doenças Pulmonares Intersticiais/imunologia , Tomografia Computadorizada por Raios X
15.
Medicine (Baltimore) ; 98(29): e16419, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31335692

RESUMO

Squawks are lung adventitious sounds with a mix of both musical and nonmusical components heard during the inspiratory phase. Small series have described squawks in interstitial lung diseases. Hypersensitivity pneumonitis and other diseases involving small airways can result in squawks, but new interstitial lung diseases (ILDs) involving peripheral airways are being described. A retrospective analysis was performed on 1000 consecutive patients from a database of ILD of a tertiary referral center. Squawks were recorded in 49 cases (4.9%), hypersensitivity pneumonitis (23 cases), connective tissue disease (7), microaspiration (4), pleuroparenchymal fibroelastosis (4), fibrosing cryptogenic organizing pneumonia (, 3), familial ILD (2), sarcoidosis (2), idiopathic pulmonary fibrosis (IPF; 1), bronchiolitis (2), and nonspecific interstitial pneumonia (1). One patient had a final diagnosis of IPF. There was a significant association between mosaic pattern and squawks: 20 cases with squawks (40.8%) had mosaic pattern compared with 140 (14.7%) cases without squawks (x = 23.6, P < .001).Findings indicative of fibrosis were described on high-resolution chest tomography (HRCT) in 715 cases (71.5%). Squawks were more common in patients with findings indicative of fibrosis on HRCT: 45 of 715 (6.3%) compared with 4 of 285 (1.4%) of those without findings indicative of fibrosis (x = 10.46, P = .001).In conclusion, squawks are an uncommon finding on physical examination in patients with ILD, but when present suggest fibrosing ILD associated with bronchiolar involvement. However, squawks are rare in IPF.


Assuntos
Doenças Pulmonares Intersticiais , Fibrose Pulmonar , Sons Respiratórios , Auscultação/métodos , Bronquíolos/patologia , Bronquíolos/fisiopatologia , Estudos de Coortes , Feminino , Humanos , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Fibrose Pulmonar/diagnóstico por imagem , Fibrose Pulmonar/etiologia , Fibrose Pulmonar/fisiopatologia , Sons Respiratórios/diagnóstico , Sons Respiratórios/fisiopatologia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos
17.
Int J Med Sci ; 16(7): 967-980, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31341410

RESUMO

Interstitial lung diseases (ILDs) may be complicated by chronic respiratory failure (CRF), especially in the advanced stages. Aim of this narrative review is to evaluate the current evidence in management of CRF in ILDs. Many physiological mechanisms underlie CRF in ILDs, including lung restriction, ventilation/perfusion mismatch, impaired diffusion capacity and pulmonary vascular damage. Intermittent exertional hypoxemia is often the initial sign of CRF, evolving, as ILD progresses, into continuous hypoxemia. In the majority of the cases, the development of CRF is secondary to the worsening of the underlying disease; however, associated comorbidities may also play a role. When managing CRF in ILDs, the need for pulmonary rehabilitation, the referral to lung transplant centers and palliative care should be assessed and, if necessary, promptly offered. Long-term oxygen therapy is commonly prescribed in case of resting or exertional hypoxemia with the purpose to decrease dyspnea and improve exercise tolerance. High-Flow Nasal Cannula oxygen therapy may be used as an alternative to conventional oxygen therapy for ILD patients with severe hypoxemia requiring both high flows and high oxygen concentrations. Non-Invasive Ventilation may be used in the chronic setting for palliation of end-stage ILD patients, although the evidence to support this application is very limited.


Assuntos
Hipóxia/terapia , Doenças Pulmonares Intersticiais/complicações , Insuficiência Respiratória/terapia , Doença Crônica/terapia , Progressão da Doença , Humanos , Hipóxia/sangue , Hipóxia/etiologia , Pulmão , Doenças Pulmonares Intersticiais/sangue , Doenças Pulmonares Intersticiais/terapia , Transplante de Pulmão , Oxigênio/administração & dosagem , Oxigênio/sangue , Oxigenoterapia/métodos , Cuidados Paliativos/métodos , Insuficiência Respiratória/sangue , Insuficiência Respiratória/etiologia , Resultado do Tratamento
18.
Intern Med ; 58(18): 2605-2613, 2019 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-31178482

RESUMO

Objective Rapidly progressive interstitial lung disease (RP-ILD) with anti-melanoma differentiation-associated protein 5 (MDA5) antibody potentially presents with a fatal clinical course and requires early intensive treatment. Recently, perilobular opacity was reported to pathologically correspond to the acute phase of diffuse alveolar damage in RP-ILD with anti-MDA5 antibody. We aimed to investigate whether or not perilobular opacity was a common radiological finding in RP-ILD patients with anti-MDA5 antibody. Methods We conducted a retrospective review of the medical records of eight consecutive patients with RP-ILD with anti-MDA5 antibody. The clinical features and radiological findings of follow-up computed tomography (CT) during the course of their disease were evaluated. Results Among eight RP-ILD patients with anti-MDA-5 antibody, six showed perilobular opacity in the lower lobes, and the remaining two had only consolidation on high-resolution CT. Of note, the perilobular opacity in all six patients thickened and progressed to consolidation with a loss of lung volume in a short period. Despite intensive treatment, 6 patients (75%) died within 100 days after the first visit. Notably, the two patients with consolidation presented with a very rapid clinical course and died in 13 days each. In the two survivors, the perilobular opacity and consolidation recovered with improvement in the loss of lung volume. Conclusion Rapidly progressive perilobular opacity that thickens and progresses to consolidation is characteristic of RP-ILD with anti-MDA5 antibody. Chest physicians should immediately check the status of anti-MDA-5 antibody in order to initiate early aggressive therapy in RP-ILD patients with rapidly progressive perilobular opacity.


Assuntos
Autoanticorpos/imunologia , Helicase IFIH1 Induzida por Interferon/imunologia , Doenças Pulmonares Intersticiais , Adulto , Feminino , Humanos , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Alvéolos Pulmonares/patologia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
19.
Intern Med ; 58(18): 2683-2687, 2019 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-31178485

RESUMO

Dyskeratosis congenita (DC) is occasionally complicated in patients with familial interstitial pneumonia (FIP). However, there have been no reports of FIP patients with DC that develop acute exacerbation (AE). We herein report a FIP patient with DC that showed AE of FIP after influenza virus B infection. Although DC is a rare disease in clinical practice, physicians should keep in mind that FIP combined with DC has the potential to cause AE.


Assuntos
Disceratose Congênita/complicações , Influenza Humana/complicações , Doenças Pulmonares Intersticiais/complicações , Adulto , Progressão da Doença , Evolução Fatal , Humanos , Vírus da Influenza B , Influenza Humana/virologia , Masculino
20.
Intern Med ; 58(18): 2679-2682, 2019 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-31178492

RESUMO

We herein report a 45-year-old woman with lung adenocarcinoma stage IV (cT4N3M1a). She was treated with pemetrexed (PEM) monotherapy following four cycles of first-line treatment with carboplatin, paclitaxel, and veliparib. After three cycles of PEM treatment, she presented with dyspnea, and chest computed tomography showed diffuse ground-glass attenuation (GGA), suggesting hypersensitivity pneumonia (HP). Bronchoalveolar lavage revealed a marked increase in lymphocytes (90.5%), and a transbronchial lung biopsy confirmed lymphocytic alveolitis with granuloma. Because her symptoms and diffuse GGA were spontaneously resolved with PEM discontinuation alone, PEM-induced interstitial lung disease was diagnosed. Chest physicians should be aware that PEM can induce HP-type interstitial lung disease.


Assuntos
Adenocarcinoma de Pulmão/tratamento farmacológico , Alveolite Alérgica Extrínseca/diagnóstico , Antineoplásicos/efeitos adversos , Doenças Pulmonares Intersticiais/induzido quimicamente , Neoplasias Pulmonares/tratamento farmacológico , Pemetrexede/efeitos adversos , Biópsia , Diagnóstico Diferencial , Dispneia/etiologia , Feminino , Humanos , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/patologia , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X
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