Inflammatory signature in lung tissues in patients with combined pulmonary fibrosis and emphysema.

Background: The aetiology and inflammatory profile of combined pulmonary fibrosis and emphysema (CPFE) remain uncertain currently. Objective: We aimed to examine the levels of inflammatory proteins in lung tissue in a cohort of patients with emphysema, interstitial pulmonary fibrosis (IPF), and CPFE. Materials and methods: Explanted lungs were obtained from subjects with emphysema, IPF, CPFE, (or normal subjects), and tissue extracts were prepared. Thirty-four inflammatory proteins were measured in each tissue section. Results: The levels of all 34 proteins were virtually indistinguishable in IPF compared with CPFE tissues, and collectively, the inflammatory profile in the emphysematous tissues were distinct from IPF and CPFE. Moreover, inflammatory protein levels were independent of the severity of the level of diseased tissue. Conclusions: We find that emphysematous lung tissues have a distinct inflammatory profile compared with either IPF or CPFE. However, the inflammatory profile in CPFE lungs is essentially identical to lungs from patients with IPF. These data suggest that distinct inflammatory processes collectively contribute to the disease processes in patients with emphysema, when compared to IPF and CPFE.

Moving toward standardization: physician reporting of sleep studies.

Detailed primary data collected from sleep studies should lead to specific and clear reports with evidence-based clinical recommendations that, when introduced by sleep medicine specialists, create a window of opportunity to support our non-sleep medicine referring teams and to engage patients and caregivers in their care as recipients of the reports. This is how sleep study reporting differs from other test reports; currently, there is wide variation in how the data collected are presented and summarized. The goal of this document is to offer recommendations for structured reporting of sleep studies. We offer a practical, complete, and relevant document and a structure that can be implemented across sleep centers nationwide and does not burden the interpreter. We anticipate some readers will opine that some of the content is beyond the scope of what the interpreter physician needs to include, while others will propose missing data that they feel should have been included. We feel that the flexibility of the proposal accommodates for this and allows for a "first step" toward standardization of physician reporting of sleep studies. High-quality structured reporting of sleep studies is becoming ever more important for patient care, benefiting patients, caregivers, clinicians, durable medical equipment companies, and payers. CITATION: Lastra AC, Ingram D, Park J, et al. Moving toward standardization: physician reporting of sleep studies. J Clin Sleep Med. 2023;19(3):595-603.

Obstructive and central sleep apnoea in a patient with medically intractable epilepsy.

A woman in her 30s with medically intractable epilepsy and Lennox-Gastaut Syndrome on multiple antiseizure medications and with a deep brain stimulator presented to the epilepsy monitoring unit with increased seizure frequency. She was noted to have periods of apparent apnoea time linked to bursts of epileptiform activity on continuous video EEG monitoring. Once the clinical seizures were controlled, she was discharged to the sleep laboratory. She was noted to have obstructive and central sleep apnoea, which improved with the use of positive airway pressure. Central sleep apnoeas were time linked to electrographic seizures. Ictal central apnoea can easily be overlooked and is likely more common than currently recognised in patients with epilepsy. Ictal central apnoea may be a biomarker for sudden unexpected death in epilepsy.

Kleine-Levin syndrome related to pregnancy: a case report.

Kleine-Levin syndrome (KLS) is a rare disorder of recurrent hypersomnolence. The pathophysiology continues to be poorly understood. Autoimmunity, genetic polymorphisms, dysfunction of the hypothalamic axis, and abnormalities in functional imaging have been proposed. Several triggers have been described, including infection, toxins, head trauma, sleep deprivation, lactation, and menses. We present the first case report in the medical literature of KLS triggered by pregnancy and the first case of KLS from Armenia. Our patient has a pattern of mostly pregnancy-related episodes of several day sleepiness occurring monthly. This case adds to the published literature as we present a new association and explore the pathophysiology of KLS. CITATION: Khachatryan SG, Lastra AC, Vardanyan LV, Khachatryan LG, Attarian HP. Kleine-Levin syndrome related to pregnancy: a case report. J Clin Sleep Med. 2021;17(11):2325-2327.

CPAP titration failure is not equivalent to long-term CPAP treatment failure in patients with obesity hypoventilation syndrome: a case series.

STUDY OBJECTIVES: Medium and long-term trials comparing continuous positive airway pressure (CPAP) with noninvasive ventilation in patients with obesity hypoventilation syndrome have shown no differences in outcomes. However, it remains unclear whether CPAP therapy should be prescribed if significant hypoxemia persists during CPAP titration, despite optimization of upper airway obstructive events or if maximum CPAP pressure is reached. We aimed to examine the effects of 6 weeks of home CPAP therapy on gas exchange in patients with obesity hypoventilation syndrome who failed CPAP titration due to persistent hypoxemia. METHODS: This case series is a substudy of a randomized-controlled trial evaluating efficacy of 3 different PAP modalities in obesity hypoventilation syndrome. Patients randomized to CPAP who failed titration and were prescribed CPAP are included. CPAP failure was defined as spending more than 20% of total sleep time with oxygen saturation below 90% despite adequate resolution of apneas and hypopneas. Follow-up data included in-laboratory polysomnogram on prescribed CPAP after 6 weeks of home CPAP therapy. RESULTS: Three of seven participants (43%) randomized to CPAP failed CPAP titration. All were morbidly obese, had severe OSA (apnea-hypopnea index > 90 events/h) and severe sleep hypoxemia (percentage of total sleep time with oxygen saturation < 90% [T90] = 60-89%). Hypoxemia (T90: 43-67%, T80: 0-31%, and T70: 0-11%) and hypercapnia (transcutaneous pressure of CO2 levels > 50 mm Hg) persisted during CPAP titration polysomnogram. The final polysomnogram after 6 weeks of adherent home CPAP therapy showed effective control of obstructive sleep apnea. Hypoventilation and hypoxemia severity decreased significantly in all 3 participants. CONCLUSIONS: Our data suggest that CPAP titration failure does not equal CPAP treatment failure. CLINICAL TRIAL REGISTRATION: Registry: ClinicalTrials.gov; Name: AVAPS-AE Efficacy Study; URL: https://clinicaltrials.gov/ct2/show/NCT01368614; Identifier: NCT01368614.

Ineffective CPAP Treatment After Effective CPAP Titration.

CASE PRESENTATION: A 67-year-old woman was evaluated for snoring, frequent awakenings, excessive sleepiness, nocturia, headaches, witnessed apneas, and choking and gasping from sleep. Medical history included OSA, hypertension, type 2 diabetes, depression in remission, and mild intermittent asthma. Epworth sleepiness scale score was 22 (abnormal is ≥10, maximum score is 24; increasing scores represent increasing sleepiness). She had been prescribed CPAP therapy. She reported initial nasal mask discomfort (ResMed AirFit N20 nasal mask), which improved with change to an oronasal mask. Patient used nightly, with acceptable tolerance. Sleep onset and wake times remained consistent, with an average total sleep time of 7 hours. She denied alcohol intake, sedative medication use, or changes in weight.

Daily Peak Expiratory Flow Rate and Disease Instability in Chronic Obstructive Pulmonary Disease.

Rationale: Chronic obstructive pulmonary disease, (COPD) is a major cause of morbidity and mortality in the United States. Peak expiratory flow rate (PEFR) monitoring could provide a daily objective measurement of lung function in COPD patients at home. We hypothesized that individuals with greater variability in daily PEFR would signal an unstable patient population with worse outcomes. Methods: This was a retrospective analysis of prospectively collected data using an electronic diary to record daily PEFR and symptoms in severe and very severe COPD patients. Rates of PEFR change were used to characterize patients into stable and unstable groups determined by the distribution of slopes. Exacerbation-free days, time to first hospitalization, hospitalization rate, length of hospitalization, and all-cause mortality were assessed. Results: A total of 104 severe and very severe COPD patients met entry criteria, and were observed for 37,702 patient-days. There were no significant differences in baseline symptoms, demographics, forced expiratory volume in 1 second (FEV1) or comorbidities between stable versus unstable groups. The unstable group had 34.7 less exacerbation-free days and significantly shorter 6 minute walk distances (6MWD) (227.1 versus 270.7 meters, p=0.031), shorter time to first hospitalization (163 versus 286 days, p=0.017), more frequent hospitalizations (2.6 versus 1.7 per year, p=0.032) and higher all-cause mortality (10.8 versus 5.1%, p= 0.04). Conclusion: Patients with severe to very severe COPD with greater changes in PEFR have shorter 6MWD, reduced time to first hospitalization, more frequent hospitalizations, and higher all-cause mortality despite similar demographic, spirometric and comorbid parameters at baseline. Daily peak flow monitoring can be a useful tool in identifying COPD patients predisposed to worse outcomes.