Combined pulmonary fibrosis and emphysema (CPFE) has been increasingly recognized over the past 10–15 years as a clinical entity characterized by rather severe imaging and gas exchange abnormalities, but often only mild impairment in spirometric and lung volume indices. In this review, we explore the gas exchange and mechanical pathophysiologic abnormalities of pulmonary emphysema, pulmonary fibrosis, and combined emphysema and fibrosis with the goal of understanding how individual pathophysiologic observations in emphysema and fibrosis alone may impact clinical observations on pulmonary function testing (PFT) patterns in patients with CPFE. Lung elastance and lung compliance in patients with CPFE are likely intermediate between those of patients with emphysema and fibrosis alone, suggesting a counter-balancing effect of each individual process. The outcome of combined emphysema and fibrosis results in higher lung volumes overall on PFTs compared to patients with pulmonary fibrosis alone, and the forced expiratory volume in one second (FEV1)/forced vital capacity (FVC) ratio in CPFE patients is generally preserved despite the presence of emphysema on chest computed tomography (CT) imaging. Conversely, there appears to be an additive deleterious effect on gas exchange properties of the lungs, reflecting a loss of normally functioning alveolar capillary units and effective surface area available for gas exchange, and manifested by a uniformly observed severe reduction in the diffusing capacity for carbon monoxide (DLCO). Despite normal or only mildly impaired spirometric and lung volume indices, patients with CPFE are often severely functionally impaired with an overall rather poor prognosis. As chest CT imaging continues to be a frequent imaging modality in patients with cardiopulmonary disease, we expect that patients with a combination of pulmonary emphysema and pulmonary fibrosis will continue to be observed. Understanding the pathophysiology of this combined process and the abnormalities that manifest on PFT testing will likely be helpful to clinicians involved with the care of patients with CPFE.
Intravenous Ig (IVIg) is a pooled plasma product consisting primarily of monomeric IgG. For the past several decades, the use of IVIg has expanded to include the treatment of various autoimmune and inflammatory disorders, including Kawasaki's disease, antineutrophil cytoplasmic antibody-associated vasculitis, systemic lupus erythematosis, and the inflammatory myopathies. IVIg is thought to exert its immunomodulatory effects through a variety of mechanisms: neutralization of pathogenic autoantibodies; alteration of immune cell effector function; suppression of cytokine and chemokine activity; and interference with complement activation. Interstitial lung disease (ILD) is a frequent complication of autoimmune disorders and connective tissue diseases, and the presence of ILD is associated with significant morbidity and mortality. Although there are currently no large studies to support the use of IVIg in the treatment of ILD, it is being used off-label with increasing frequency for refractory cases that have failed to respond to standard immunosuppression. Although associated with less systemic toxicity and global immunosuppression than traditional agents, IVIg is much more costly. Therefore, although the routine use of IVIg to treat ILD is not currently recommended, future studies to determine its role in pulmonary disease are warranted.
BACKGROUND: Our Cooling to Help Injured Lungs (CHILL) trial of therapeutic hypothermia in ARDS includes neuromuscular blockade (NMB) as an inclusion criterion to avoid shivering. NMB has been used to facilitate mechanical ventilation in ARDS and was shown to reduce mortality in the ACURASYS trial. To assess the feasibility of a multi-center CHILL trial, we conducted a survey of academic intensivists about their NMB use in patients with ARDS. METHODS: We distributed via email a 16-question survey about NMB use in patients with ARDS including frequency, indications, and dosing strategy. RESULTS: 212 (24.3%) of 871 respondents completed the survey: 94.7% were board-certified in internal medicine, 88% in pulmonary and critical care; 90.3% practiced in academic medical centers, with 87% working in medical ICUs; 96.6% of respondents who treat ARDS use NMB, and 39.7% use NMB in > 50% of these patients. Of 4 listed indications for initiating NMB in ARDS, allowing adherence with lung-protective ventilator strategies and patient-ventilator synchrony were cited as the most important reasons, followed by the results of the ACURASYS trial and facilitating prone positioning. CONCLUSIONS: We conclude that NMB is frequently used by academic intensivists to facilitate mechanical ventilation in patients with moderate to severe ARDS.
Background: Patients with idiopathic Parkinson's disease (PD) or atypical Parkinsonism (AP) often present with orthostatic hypotension (OH) as a result of the dysautonomia associated with the disease or as a side effect of the dopaminergic medications used to treat it. Our recent study suggested that OH negatively impacts gross motor, balance, and cognitive functions in patients with PD. Objective: To determine if correcting the orthostatic hypotension (OH) of patients with PD or AP improves their gross motor, balance, and cognitive functions. Methods: Forty patients with PD or AP were assessed before and after correcting their OH using a staged approach with a goal of SBP >80 when standing and SBP <180 when laying. Step 1 of treatment included reducing antihypertensive medications, as possible, maintaining hydration with 1500cc/day, decreasing dietary salt, wearing high compression stockings, and keeping the head of bed elevated at 30 degrees when supine. If SBP <80 with standing after step 1, then treatment was started with fludrocortisone and/or midodrine. Results: Patients' OH was managed as part of a rehabilitation program. Tests such as the Motor and Cognitive Functional Independence Measures, Berg Balance Scale, Two Minute Walking test, and the Finger Tapping test showed significant improvements (p < 0.05) in their gross motor, walking, balance and cognitive function with our OH management plan. No significant differences between admission and discharge were found in the Timed Up and Go test. Conclusion: Our data suggest that monitoring and correcting the OH of patients with PD or AP improves their gross motor, balance, and cognitive function.
The use of intrapleural alteplase and dornase in pregnant patients remains an uncertain practice because bleeding complications in these cases could be devastating. We present a case in which we successfully used a modified protocol safely.
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