Long-term evaluation of pulmonary function and survival of patients with interstitial pneumonia with autoimmune features.

OBJECTIVES: Interstitial pneumonia with autoimmune features (IPAF) includes patients with interstitial lung disease with autoimmune features who do not meet criteria for a connective tissue disease (CTD). Previous studies showed a wide variation in the radiologic pattern, pulmonary function and prognosis but there is still limited data on longitudinal outcomes. We aim to describe the long-term pulmonary function, radiological patterns, and survival of IPAF patients and explore a classification based on CTD-like subgroups by using clinical/serologic data. METHODS: Retrospective analysis of IPAF patients who were sub-classified into six CTD-(like) subgroups: systemic lupus erythematosus-like, rheumatoid arthritis-like, Sjögren's syndrome-like, scleroderma, myositis-like, and unclassifiable. Linear mixed-effect models were used to compare the change in percent-predicted forced vital capacity (FVC%), percent-predicted diffusion capacity (DLCO%), and six-minute walk distance (SMWD) over time; and survival in the entire cohort and according to CTD-like subgroups and radiological patterns. RESULTS: Fifty-nine patients fulfilled IPAF criteria. FVC%, DLCO%, and SMWD remained stable over time. There was no difference between usual interstitial pneumonia (UIP) versus non-UIP radiologic patterns. Thirty-five patients were sub-classified into CTD-like subgroups. Survival decreased from 79% at 60 months to 53% at 120 months in the entire cohort but was similar among CTD-like subgroups and radiological patterns. CONCLUSIONS: Long-term pulmonary function and six-minute walk test remained stable over 36 months in our IPAF cohort. Prognosis and pulmonary function in UIP had similar outcomes compared to non-UIP. Although 40% of IPAF patients could not be sub-classified, our exploratory subclassification stratified 60% of patients into a CTD-like subgroup.

Rapidly progressive metastatic cholangiocarcinoma in a postpartum patient with cystic fibrosis: a case report.

BACKGROUND: Cholangiocarcinoma is a rare gastrointestinal malignancy that arises within the intrahepatic, perihilar, and/or extrahepatic bile ducts. Individuals with cystic fibrosis are at increased risk for gastrointestinal malignancies. The most common gastrointestinal malignancy in cystic fibrosis is colon cancer, but other gastrointestinal malignancies also occur at greater rates than the general population. CASE PRESENTATION: We present a case of a rapidly progressive metastatic intrahepatic cholangiocarcinoma in an individual with cystic fibrosis who was 5 months postpartum, incidentally found while undergoing a lung transplantation evaluation. CONCLUSION: A heightened clinical awareness of gastrointestinal malignancies, beyond colon cancer, in individuals with cystic fibrosis is warranted. It remains unclear if pregnancy is an additional risk factor for gastrointestinal malignancies in cystic fibrosis.

Indoor air pollution exposure is associated with greater morbidity in cystic fibrosis.

BACKGROUND: Exposure to higher levels of outdoor air pollution is associated with increased morbidity in individuals with cystic fibrosis. Limited information exist regarding the potential adverse effects of indoor air pollution on those with cystic fibrosis. METHODS: Individuals with cystic fibrosis who were enrolled in the Twin and Sibling Study from 2000-2013, self-reported exposure to four known sources of indoor air pollution (secondhand smoke, forced hot air, wood stove and fireplace). Change in lung function, rates of hospitalizations and pulmonary exacerbations were followed over 4 years to compare outcomes in those who were exposed to those who were not exposed. RESULTS: Of 1432 participants with data on secondhand smoke exposure, 362 (25.3%) were exposed. Of 765 individuals with data on forced hot air exposure, 491 (64.2%) were exposed. Of 1247 participants with data on wood stove exposure and 830 with data on fireplace exposure, 182 (14.6%) and 373 (44.9%) were exposed, respectively. In longitudinal analysis, pediatric individuals either exposed to secondhand smoke or to forced hot air had a 0.60% predicted/year decrease in FEV1% predicted (P=0.002) or a 0.46% predicted/year decrease in FEV1% predicted (P=0.048), respectively compared to individuals who were not exposed. Adults exposed to secondhand smoke had a 42% increased yearly risk of hospitalization compared to those who were not exposed (P=0.045). CONCLUSIONS: Our questionnaire-based data suggest that exposure to sources of indoor air pollution increase morbidity in both the pediatric and adult cystic fibrosis populations. Future studies with quantitative indoor air quality assessments are needed.

Patterns and Predictors of Air Cleaner Adherence Among Adults with COPD.

Rational: Poor indoor air quality has been associated with worse chronic obstructive pulmonary disease (COPD) morbidity. In-home portable air cleaners reduce indoor pollutants and could improve respiratory health. Factors associated with air cleaner adherence among adults with COPD remains unknown. Methods: In a 6-month trial of former smokers with COPD, participants (n=116) received active or sham portable air cleaners. Air cleaner adherence was measured by electronic monitors. Potential baseline predictors of adherence included individual factors (demographics, socioeconomic status, smoking history, psychological well-being), COPD disease severity, and housing characteristics. Time and season were also considered. Stepwise logistic regression and longitudinal fixed effect analysis were performed to assess independent predictors of adherence. Results: A total of 109 participants had an objective measure of adherence, and 76.1% used at least 1 air cleaner 80% of the time (defined a priori as adherent). Higher annual household income ≥$35,000 (odds ratio [OR]=4.4, 95% confidence interval [CI], 1.1-18.0) and use of heat pump/electricity (versus gas) for heating (OR=6.1, 95%CI, 1.7-22.4) were associated with higher odds of adherence. Further, poor quality of life (St George's Respiratory Questionnaire, per 10-point increase) and prior year exacerbations were associated with lower odds of adherence (OR=0.65, 95%CI, 0.4-1.0) and (OR=0.26, 95%CI, 0.1-0.9), respectively. Adherence was highest during the first month and lower during winter compared to other seasons. Conclusion: These findings suggest that cold weather season, use of gas for home heating, and lower annual income negatively impact adherence. Poor quality of life and worse disease control may also decrease adherence. Addressing factors associated with air cleaner adherence should be considered when designing future environmental studies.

Sex differences in treatment patterns in cystic fibrosis pulmonary exacerbations.

BACKGROUND: Females with cystic fibrosis (CF) have been shown to have worse pulmonary exacerbation (PEx) related outcomes compared to males. However, it is unknown if sex differences in treatment patterns are contributing to these outcomes. Thus, we sought to explore sex differences in treatment patterns in the Standardized Treatment of Pulmonary Exacerbations (STOP) cohort. METHODS: Data for 220 participants from the STOP cohort were analyzed. Multivariable regression models were used to assess if female sex was associated with duration of treatment with IV antibiotics and inpatient length of stay. Secondary outcomes included antibiotic selection, adjunctive therapies, mean FEV1pp and CFRSD-CRISS respiratory symptom scores at the four study assessments. RESULTS: In our adjusted model, the average number of IV antibiotic treatment days was 13% higher in females compared to males (IRR 1.13, 95% CI=1.02,1.25; p=0.02). We found no sex differences in inpatient length of stay, number of IV antibiotics, antibiotic selection or initiation of adjunctive therapies. Overall, females had higher CFRSD-CRISS scores at the end of IV therapy indicating worse symptom severity (23.6 for females vs. 18.5 for males, p=0.03). CONCLUSIONS: Despite females having a longer treatment duration, our findings demonstrate that males and females are receiving similar treatments which suggest that the outcome disparities in females with CF may not be due to failure to provide the same level of care. Further research dedicated to sex differences in CF is necessary to understand why clinical outcomes differ between males and females.

A contemporary experience of open aortic reconstruction in patients with chronic atherosclerotic occlusion of the abdominal aorta.

OBJECTIVE: To examine and report surgical results from a contemporary experience of open abdominal aortic reconstruction in patients with chronic atherosclerotic abdominal aortic occlusion (CAAAO). METHODS: Between January 1999 through May 2010, 54 patients with CAAAO were identified and retrospectively reviewed. CAAAOs were categorized into infrarenal aortic occlusions (IRAOs) and juxtarenal aortic occlusions (JRAOs) based on superior extension of thrombus and requirement for supra-renal aortic clamping to repair. Morbidity, mortality, hospital stay, and operative variables were assessed. The χ2 or Fisher test and the Wilcoxon rank sum test were used to compare demographic and operative variables between two aortic occlusion groups (IRAO and JRAO). Univariate and multivariate analyses were performed to assess factors associated with surgical outcomes and hospital stay. The Kaplan-Meier method was used to calculate survival and patency rates. RESULTS: Fifty patients underwent aortic reconstructions with aorto-bifemoral or iliac bypass, and three underwent a remote axillo-femoral bypass procedure. There were 35 (64.8%) males, and 19 (35.2%) females. Median age was 51.9 years (range, 32-72 years). Of the two CAAAO groups, there were 20 IRAOs and 33 JRAOs. Aorto-renal thromboendartectomy was performed in 26 (49.1%) patients; 26 (75.8%) among JRAOs versus 1 (5%) of IRAOs (P<.01). Proximal aortic clamps were required in 28 (85%) of JRAOs and 3 (15%) of IRAOs (P<.01). Thirty-day and in-hospital mortality was zero. Median length of hospital stay was 7 days (range, 4 to 66 days), and median intensive care unit length of stay was 3 days (range, 1-22 days). Complications included cardiopulmonary dysfunction in four (8%), postoperative renal insufficiency in 10 (18.9%), and other postoperative complications in 15 (28.3%). All 10 with renal insufficiency recovered renal function to baseline creatinine or a creatinine value<1.1 mg/dL. Mean increases in right and left ankle-brachial indicess were 0.54±0.25 and 0.59±0.22, respectively. On univariate analysis, coronary artery disease and African American race were predictors of postoperative complications (P=.048). Age was significantly associated with total complications. Patients with postoperative complications and/or renal insufficiency were older than those without such complications (P=.02) Independent predictors of prolonged hospital stay were intraoperative blood replacement (P=.003), postoperative complications (P<.01), and postoperative renal insufficiency (P<.01). Prolonged intensive care unit stay was predicted by JRAO (P=.04), postoperative complications (P=.02), and postoperative renal insufficiency (P=.013). Survival at 3, 5, and 7 years were 86.6%, 76.5% and 50.9%, respectively. The reduced survival rates were predicted by previous myocardial infarction and existing coronary artery disease (P<.01). CONCLUSION: Abdominal aortic reconstruction is a safe method for treating CAAAO with low associated morbidity and mortality. Aorto-renal thromboendartectomy with supra-renal aortic clamping and aortic replacement remains an effective treatment for those with significant pararenal aortic disease, and can be performed without significant renal impairment.

Unmasking catamenial hemoptysis in the era of CFTR modulator therapy.

BACKGROUND: Thoracic endometriosis syndrome (TES) is a rare condition that occurs in women when endometriosis implants into the thoracic cavity. Catamenial hemoptysis, the occurrence of hemoptysis with menstruation, is a recognized clinical manifestation of TES commonly treated with hormonal therapy. CASE SUMMARY: We present the first documented case describing the recrudescence of catamenial hemoptysis in the setting of Lumacaftor/Ivacaftor administration in a 25-year-old woman with cystic fibrosis (CF). DISCUSSION: We review the literature on TES, pharmacologic management, and reported cystic fibrosis transmembrane conductance regulator (CFTR) modulator drug interactions. We propose that our patient's recrudescence of catamenial hemoptysis was secondary to a drug-drug interaction between Lumacaftor/Ivacaftor and oral contraceptive therapy. CONCLUSION: Our case suggests that women with CF who have catamenial hemoptysis and a genetic mutation approved for Tezacaftor/Ivacaftor or Elexacaftor/Tezacaftor/Ivacaftor can be managed effectively with either CFTR modulator and hormonal contraceptive therapy.