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1.
Eur Respir J ; 2020 Jul 16.
Artigo em Inglês | MEDLINE | ID: mdl-32675203

RESUMO

Interstitial pneumonia with autoimmune features (IPAF) characterises individuals with interstitial lung disease (ILD) and features of connective tissue disease (CTD) who fail to satisfy CTD criteria. Inclusion of myositis-specific antibodies (MSAs) in the IPAF criteria has generated controversy, as these patients also meet proposed criteria for an anti-synthetase syndrome. Whether MSAs and myositis associated antibodies (MAA) identify phenotypically distinct IPAF subgroups remains unclear.A multi-center, retrospective investigation was conducted to assess clinical features and outcomes in patients meeting IPAF criteria stratified by the presence of MSAs and MAAs. IPAF subgroups were compared to cohorts of patients with idiopathic inflammatory myopathy-ILD (IIM-ILD), idiopathic pulmonary fibrosis (IPF) and non-IIM CTD-ILDs. The primary endpoint assessed was three-year transplant-free survival. Two hundred sixty-nine patients met IPAF criteria, including 35 (13%) with MSAs and 65 (24.2%) with MAAs. Survival was highest among patients with IPAF-MSA and closely approximated those with IIM-ILD. Survival did not differ between IPAF-MAA and IPAF without MSA/MAA cohorts. Usual interstitial pneumonia (UIP) morphology was associated with differential outcome risk, with IPAF patients with non-UIP morphology approximating survival observed in non-IIM CTD-ILDs. MSAs, but not MAAs identified a unique IPAF phenotype characterised by clinical features and outcomes similar to IIM-ILD. UIP morphology was a strong predictor of outcome in others meeting IPAF criteria. Because IPAF is a research classification without clear treatment approach, these findings suggest MSAs should be removed from the IPAF criteria and such patients should be managed as an IIM-ILD.

3.
J Sleep Res ; : e12894, 2019 Jul 28.
Artigo em Inglês | MEDLINE | ID: mdl-31352685

RESUMO

Atypical EEG patterns not consistent with standard sleep staging criteria have been observed in medical intensive care unit (ICU) patients. Our aim was to examine the relationship between sleep architecture and sedation in critically ill mechanically ventilated patients pre- and post-extubation. We performed a prospective observational repeated measures study where 50 mechanically ventilated patients with 31 paired analyses were examined at an academic medical centre. The sleep efficiency was 58.3 ± 25.4% for intubated patients and 45.6 ± 25.4% for extubated patients (p = .02). Intubated patients spent 76.33 ± 3.34% of time in non-rapid eye movement (NREM) sleep compared to 64.66 ± 4.06% of time for extubated patients (p = .02). REM sleep constituted 1.36 ± 0.67% of total sleep time in intubated patients and 2.06 ± 1.09% in extubated patients (p = .58). Relative sleep atypia was higher in intubated patients compared to extubated patients (3.38 ± 0.87 versus 2.79 ± 0.42; p < .001). Eleven patients were sedated with propofol only, 18 patients with fentanyl only, 11 patients with fentanyl and propofol, and 10 patients had no sedation. The mean sleep times on "propofol", "fentanyl", "propofol and fentanyl," and "no sedation" were 6.54 ± 0.64, 4.88 ± 0.75, 6.20 ± 0.75 and 4.02 ± 0.62 hr, respectively. The sigma/alpha values for patients on "propofol", "fentanyl", "propofol and fentanyl" and "no sedation" were 0.69 ± 0.04, 0.54 ± 0.01, 0.62 ± 0.02 and 0.57 ± 0.02, respectively. Sedated patients on mechanical ventilation had higher sleep efficiency and more atypia compared to the same patients following extubation. Propofol was associated with higher sleep duration and less disrupted sleep architecture compared to fentanyl, propofol and fentanyl, or no sedation.

4.
Chest ; 156(4): 715-723, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31181198

RESUMO

BACKGROUND: Antineutrophil cytoplasmic antibodies (ANCAs) have been reported to occur in 7% to 10% of patients with idiopathic pulmonary fibrosis (IPF), but their clinical relevance remains unclear. The aim of this study was to estimate the prevalence of ANCAs in a North American population with IPF and evaluate their clinical significance. METHODS: This was a retrospective study of two independent cohorts of patients diagnosed with IPF at the University of California San Francisco (discovery cohort) and the University of Chicago (replication cohort). Myeloperoxidase (MPO) and proteinase 3 (PR3) ANCAs were measured in all patients. Prevalence and associations of ANCAs with clinical characteristics and transplant-free survival were evaluated. RESULTS: A total of 14 of 353 (4.0%; 95% CI, 2.2-6.5) and 20 of 392 (5.1%; 95% CI, 3.1-7.8) patients with IPF were positive for ANCAs at the time of diagnosis in the discovery and replication cohorts, respectively. Among those positive for MPO antibodies, two of six (33%) in the discovery cohort and three of 12 (25%) in the replication cohort developed vasculitis. None of the patients who were PR3-positive developed vasculitis. Patients who were ANCA-positive were more likely to be women than patients who were ANCA-negative, and were more likely to have some ground-glass opacities on CT scan. In the combined cohort of 745 patients, median transplant-free survival was not significantly different in patients who were ANCA-positive vs ANCA-negative (P = .57). CONCLUSIONS: ANCA positivity is uncommon in North American patients with IPF and not associated with baseline disease severity or transplant-free survival; however, a significant proportion of patients who are MPO-positive with IPF develop clinical vasculitis.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/sangue , Fibrose Pulmonar Idiopática/sangue , Mieloblastina/sangue , Peroxidase/sangue , Idoso , Feminino , Humanos , Fibrose Pulmonar Idiopática/complicações , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Estados Unidos , Vasculite/sangue , Vasculite/etiologia
5.
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
6.
J Rheumatol ; 45(9): 1220-1228, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29764963

RESUMO

OBJECTIVE: To assess a multimodal intervention for reducing missed opportunities for outpatient influenza vaccination in individuals with rheumatoid arthritis (RA). METHODS: Patients with RA were enrolled from a single center and each rheumatology outpatient visit was tracked for missed opportunities for influenza vaccination, defined as a visit in which an unvaccinated patient without contraindications remained unvaccinated or lacked documentation of vaccine recommendation in the electronic medical record (EMR). Providers then received a multimodal intervention consisting of an education session, EMR alerts, and weekly provider-specific e-mail reminders. Missed opportunities before and after the intervention were compared, and the determinants of missed opportunities were analyzed. RESULTS: A total of 228 patients with RA were enrolled (904 preintervention visits) and 197 returned for at least 1 postintervention visit (721 postintervention visits). The preintervention frequency of any missed opportunities for influenza vaccination was 47%. This was reduced to 23% postintervention (p < 0.001). Among those vaccinated, the relative hazard for influenza vaccination post- versus preintervention period was 1.24 (p = 0.038). Younger age, less frequent office visits, higher erythrocyte sedimentation rate, and negative attitudes about vaccines were each independently associated with missed opportunities preintervention. Postintervention, these factors were no longer associated with missed opportunities; however, the intervention was not as effective in non-Hispanic black patients, non-English speakers, those residing outside of the New York City metropolitan area, and those reporting prior adverse reactions to vaccines. CONCLUSION: Improved uptake of influenza vaccination in patients with RA is possible using a multimodal approach. Certain subgroups may need a more potent intervention for equivalent efficacy.


Assuntos
Artrite Reumatoide/imunologia , Vacinas contra Influenza , Influenza Humana/prevenção & controle , Visita a Consultório Médico , Vacinação , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
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