Interstitial Lung Disease Mortality Disparities Along the United States - Mexico Border, 1999-2020.

BACKGROUND: Optimal diagnosis and management of interstitial lung diseases (ILD) needs access to specialized centers, frequent monitoring, and complex therapeutic options. In underprivileged areas, these necessities can often lead to barriers in delivering care. RESEARCH QUESTION: What are the ILD mortality disparities in the regions along the United States-Mexico (US-MX) border? STUDY DESIGN AND METHODS: We obtained ILD mortality information through death certificate queries from the Centers for Disease Control and Prevention repository. Death data were adjusted for age and stratified by US-MX border regions and non-border regions in the US. Log-linear regression models were utilized to analyze mortality trends in the period from 1999 to 2020 followed by calculation of annual percentage changes (APC). Age-adjusted mortality rates (AAMR) were compared across cumulative and sub-demographic populations. RESULTS: ILD-related mortality among border regions (AAMR 5.31) was higher compared to non-border regions (AAMR 4.86). Mortality within border regions remained unchanged from 1999 to 2020 (APC +0.3, p=0.269). Non-border regions experienced a significant rise in mortality rates (APC +2.6, p=0.017) from 1999 to 2005 and remained unchanged from 2005 to 2020. Mortality was higher within both men (AAMR 6.57) and women (AAMR 4.36) populations among border regions compared to their non-border counterparts (AAMR 6.27 and 3.87, respectively). Hispanic populations among the border regions experienced higher mortality rates (AAMR 6.15) compared to Hispanic populations within non-border regions (AAMR 5.44). Non-Hispanic populations encountered similar mortality rates between the two regions. Mortality rates among Hispanic (APC +0.0, p=0.938) and non-Hispanic (APC +0.2, p=0.531) populations in the border regions remained unchanged from 1999 to 2020. INTERPRETATION: These results revealed ILD-related mortality disparities among the US-MX border regions, emphasizing the importance of public health measures to increase access to equitable medical care and implement targeted interventions among these vulnerable populations.

Consensus Guidelines for Evaluation and Management of Pulmonary Disease in Sjögren's.

BACKGROUND: Pulmonary disease is a potentially serious yet underdiagnosed complication of Sjögren's syndrome, the second most common autoimmune rheumatic disease. Approximately 16% of patients with Sjögren's demonstrate pulmonary involvement with higher mortality and lower quality of life. RESEARCH QUESTION: Clinical practice guidelines for pulmonary manifestations of Sjögren's were developed by the Sjögren's Foundation after identifying a critical need for early diagnosis and improved quality and consistency of care. STUDY DESIGN AND METHODS: A rigorous and transparent methodology was followed according to American College of Rheumatology guidelines. The Pulmonary Topic Review Group (TRG) developed clinical questions in the PICO (Patient, Intervention, Comparison, Outcome) format and selected literature search parameters. Each article was reviewed by a minimum of two TRG members for eligibility and assessment of quality of evidence and strength of recommendation. Guidelines were then drafted based on available evidence, expert opinion, and clinical importance. Draft recommendations with a clinical rationale and data extraction tables were submitted to a Consensus Expert Panel for consideration and approval, with at least 75% agreement required for individual recommendations to be included in the final version. RESULTS: The literature search revealed 1,192 articles, of which 150 qualified for consideration in guideline development. Of the original 85 PICO questions posed by the TRG, 52 recommendations were generated. These were then reviewed by the Consensus Expert Panel and 52 recommendations were finalized, with a mean agreement of 97.71% (range, 79%-100%). The recommendations span topics of evaluating Sjögren's patients for pulmonary manifestations and assessing, managing, and treating upper and lower airway disease, interstitial lung disease, and lymphoproliferative disease. INTERPRETATION: Clinical practice guidelines for pulmonary manifestations in Sjögren's will improve early identification, evaluation, and uniformity of care by primary care physicians, rheumatologists, and pulmonologists. Additionally, opportunities for future research are identified.

Approach to tapering antisarcoidosis therapy.

PURPOSE OF REVIEW: Sarcoidosis is a multisystemic granulomatous disease, which commonly affects the lung. The natural course of the disease and prognosis are variable from asymptomatic, spontaneous remission to progressive disease, which requires treatment. Once treatment is initiated, tapering therapy can be problematic. RECENT FINDINGS: Corticosteroids are recommended as first-line therapy, but optimal regimen and duration of treatment is not well established. Treatment may differ based on severity of disease, extrapulmonary involvement, physician and patient preferences. We reviewed currently recommended regimens, particularly, in pulmonary sarcoidosis and the use of alternative treatments as corticosteroid-sparing agents. SUMMARY: Corticosteroid use is quite effective as initial therapy but is associated with significant side effects. An approach to tapering sarcoidosis therapy is not standardized, given the lack of evidence-based data. This review provides guidance based on the current literature.

Low-dose sirolimus in retroperitoneal lymphangioleiomyomas.

Lymphangioleiomyomatosis (LAM) is a rare disease associated with cystic lung destruction and abdominal tumors, including lymphangioleiomyomas, which frequently occur in the retroperitoneal region. Sirolimus therapy is currently recommended for LAM patients with abnormal or declining lung function with an adjusted dose to maintain a serum trough level of 5-15 ng/mL. We describe a significant reduction of retroperitoneal lymphangioleiomyomas after treatment with low-dose sirolimus therapy (serum trough level <5 ng/mL) in a patient with sporadic LAM.

Prevalence of metabolic syndrome in patients with pulmonary hypertension.

BACKGROUND AND AIMS: To determine the prevalence of metabolic syndrome (MetS) - a potential phenotypical risk for pulmonary hypertension (PH) - in patients with PH and by PH diagnostic group including group 1 pulmonary arterial hypertension (PAH); group 1.1 idiopathic PAH, groups 1.2 heritable PAH, 1.3 drug and toxin induced, 1.4 associated PAH, group 2 PH with left heart disease, group 3 PH associated with lung disease and/or hypoxemia, group 4 PH owing to chronic thrombotic and/or embolic disease, and group 5 PH from multifactorial mechanisms. METHODS: We conducted a retrospective chart review of patients referred to Mayo Clinic in Florida from January 1990 to October 2013 (N = 1287). Only patients whom PH was confirmed by right heart catheterization were included (N = 640). MetS was defined with at least three of the following: systemic hypertension, diabetes mellitus, hyperlipidemia and body mass index > 30. RESULTS: MetS was prevalent in 247 of 640 (39%) with PH: 13% in group 1.1 PAH, 12% in groups 1.2-1.4 PAH, 54% in group 2, 55% in group 3, 23% in group 4 and 24% in group 5. After adjusting for age, sex and race, diagnostic subgroup analysis revealed a higher prevalence of MetS in WHO Group 2 PH patients [adjusted odd ratios (OR): 2.58, P < 0.0001] and WHO group 3 PH (adjusted OR: 2.77, P < 0.0001) when compared to group 1 PH. CONCLUSION: The prevalence of MetS was observed in 39% of patients with PH; however, among PH patients, the prevalence was higher in group 2 and 3 PH.

Transbronchial Cryobiopsy in Diffuse Parenchymal Lung Disease: Retrospective Analysis of 74 Cases.

BACKGROUND: Diagnostic evaluation of patients with diffuse parenchymal lung disease (DPLD) is best achieved by a multidisciplinary team correlating clinical, radiological, and pathologic features. Surgical lung biopsy remains the gold standard for histopathologic diagnosis of idiopathic interstitial pneumonias. Emerging data suggest an increasing role for transbronchial cryobiopsy (TBC) in DPLD evaluation. We describe our experience with TBC in patients with DPLD. METHODS: We retrospectively reviewed medical records of patients with radiographic features of DPLD who underwent TBC at Mayo Clinic in Rochester, Minnesota from June 2013 to September 2015. RESULTS: Seventy-four patients (33 women [45%]) with a mean age of 63 years (SD, 13.8) were included. The mean maximal diameter of the samples was 9.2 mm (range, 2-20 mm [SD, 3.9]). The median number of samples per procedure was three (range, one to seven). Diagnostic yield was 51% (38 of 74 specimens). The most frequent histopathologic patterns were granulomatous inflammation (12 patients) and organizing pneumonia (OP) (11 patients), resulting in the final diagnoses of hypersensitivity pneumonitis (six patients), cryptogenic OP (six patients), connective tissue disease-associated OP (three patients), drug toxicity (three patients), infection-related OP (two patients), sarcoidosis (two patients), and aspiration (one patient). Other histopathologic patterns included respiratory bronchiolitis (three patients), acute fibrinous and organizing pneumonia (two patients), desquamative interstitial pneumonia (1 patient), diffuse alveolar damage (one patient), pulmonary alveolar proteinosis (one patient), amyloidosis (one patient), eosinophilic pneumonia (one patient), necrotizing vasculitis (one patient), bronchiolitis with food particles (one patient), and malignancy (three patients). Pneumothorax developed in one patient (1.4%), and bleeding occurred in 16 patients (22%). CONCLUSIONS: Our single-center cohort demonstrated a 51% diagnostic yield from TBC; the rates of pneumothorax and bleeding were 1.4% and 22%, respectively. The optimal use of TBC needs to be determined.

Can a Six-Minute Walk Distance Predict Right Ventricular Dysfunction in Patients with Diffuse Parenchymal Lung Disease and Pulmonary Hypertension?

OBJECTIVES: Pulmonary hypertension (PH) is commonly observed in patients with diffuse parenchymal lung disease (DPLD). The purpose of this study was to explore the influence of the 6-minute walk test (6MWT) as a simple, non-invasive tool to assess right ventricular (RV) function in patients with DPLD and to identify the need for an echocardiogram (ECHO) to screen for PH. METHODS: We retrospectively reviewed 48 patients with PH secondary to DPLD, who were evaluated in the PH clinic at the Mayo Clinic in Jacksonville, Florida, from January 1999 to December 2014. RESULTS: Fifty-two percent of patients had RV dysfunction. They had a significantly greater right heart pressure by ECHO and mean pulmonary arterial pressure (MPAP) from right heart catheterization (RHC) than those with normal RV function. A reduced 6-minute walk distance (6MWD) did not predict RV dysfunction (OR 0.995; 95% CI 0.980-1.001, p = 0.138). In addition, worsening restrictive physiology, heart rate at one-minute recovery and desaturation were not different between patients with and without RV dysfunction. However, there were inverse correlations between 6MWD and MPAP from RHC (r = -0.41, p = 0.010), 6MWD and RV systolic pressure (r = -0.51, p < 0.001), and 6MWD and MPAP measured by ECHO (r = -0.46, p =0.013). We also found no significant correlation between 6MWD and pulmonary function test parameters. CONCLUSIONS: Our single-center cohort of patients with PH secondary to DPLD, PH was found to have an impact on 6MWD. In contrast to our expectations, 6MWD was not useful to predict RV dysfunction. Interestingly, a severe reduction in the 6MWD was related to PH and not to pulmonary function; therefore, it may be used to justify an ECHO to identify patients with a worse prognosis.

Mayo clinic experience of lung transplantation in pulmonary lymphangioleiomyomatosis.

OBJECTIVES: Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease that generally results in progressive decline in lung function. Despite advancement of pharmacological therapy for LAM, lung transplantation remains an important option for women with end-stage LAM. METHODS: Patients with LAM undergoing lung transplantation at the Mayo Clinic campuses in Rochester, Minnesota and Jacksonville, Florida since 1995 were retrospectively reviewed. RESULTS: Overall, 12 women underwent lung transplantation. Nine of 12 (75%) underwent double lung transplant. The mean age was 42 ± 8 years at the time of transplant. One patient (8%) had a chylothorax and 7 (58%) had recurrent pneumothoraces, 4 (33%) of which required pleurodesis. All had diffuse, cystic lung disease on chest CT consistent with LAM which was confirmed in the explant of all patients. The average length of ICU and hospital stays were 5 ± 4 and 19 ± 19 days, respectively. Mild to moderate anastomotic ischemia was evident in all patients but resolved with time. No patient was treated with sirolimus pre-transplant. Seven patients received sirolimus post-transplant; however, clinical benefit was documented in only 2 patients, 1 of which was treated for large retroperitoneal cysts with ureteral obstruction and another with persistent chylothorax and retroperitoneal lymphangioleimyomas. Five patients are deceased. The median survival by Kaplan-Meier analysis was 119 months with a median follow-up of 68 months (range 2-225 months). CONCLUSIONS: Lung transplant remains a viable treatment for patients with end-stage LAM. The role of sirolimus peri-transplantation remains ill-defined.

Mosaic Pattern of Lung Attenuation on Chest CT in Patients with Pulmonary Hypertension.

A mosaic pattern of lung attenuation on chest computed tomography (CT) may be due to various etiologies. There is limited published data on CT results when used to evaluate pulmonary hypertension (PH). We retrospectively studied the frequency of mosaic pattern in patients with PH and the cause of the PH by diagnostic group, as well as the correlation between the mosaic pattern and the following: demographics, severity of the PH, main pulmonary artery (PA) size, PA/aorta (PA/Ao) ratio, pulmonary function tests (PFT), and ventilation perfusion scan results. Overall, 18% of the cohort had CT mosaic pattern (34/189). Mosaic pattern was present in 17/113 (15%) in Group 1 pulmonary arterial hypertension, 5/13 (28%) in Group 2 pulmonary venous hypertension and 8/50 (16%) in Group 3 PH. Conversely, Group 4 chronic thromboembolic PH was more prevalent in 4/8 (50%). Main PA size, PA/Ao ratio, and segmental perfusion defect were positively associated with mosaic pattern. In contrast, factors such as age, gender, body mass index, functional class, hemodynamic data, and PFT values were not associated with mosaic pattern. Mosaic pattern is not specific as an isolated finding for distinguishing the subtype of PH.

Survey of restless legs syndrome in a pulmonary hypertension population.

INTRODUCTION: The prevalence of restless legs syndrome (RLS) varies from 5% to 24% in the general population and is associated with a variety of medical disorders. However, the association between RLS and pulmonary hypertension (PH) is unknown. OBJECTIVES: To determine the prevalence of RLS in PH patients. METHODS: A cross-sectional questionnaire was given to patients with PH who attended the Pulmonary Hypertension Association conference. We used the RLS Diagnostic Index questionnaire. Demographic data included age, gender, height, weight, body mass index (BMI), medication list, PH World Health Organization (WHO) diagnosis group and current WHO functional class. RESULTS: The study included 128 PH patients. Most were females (86.8%) with a mean age of 49.68 years [standard deviation (SD) 14.24]. The mean BMI was 31.3 (SD 20.46). One hundred and twenty-one patients (93.75%) were classified as WHO group 1 (pulmonary arterial hypertension). Three patients were identified in WHO group 3 (hypoxemic states), four patients in group 4 (chronic thromboemboli) and one patient in group 5 (2.3%, 3.1% and 0.8%, respectively). Definite RLS was found in 16 of 128 patients with PH resulting in a prevalence of 12.5%, possible RLS in 39 of 128 patients (30.46%) and no RLS in 73 (57.03%) patients. CONCLUSION: The prevalence of RLS is not increased in PH. There is a high prevalence of possible RLS in our study. The overall prevalence of combined definite and possible RLS is significant. Future research is needed to assess more patients with PH and the association or correlation with RLS.

A 40-year-old woman with multiple pulmonary nodules. Pulmonary hyalinizing granuloma.

A 40-year-old woman (a nonsmoker) with history of idiopathic thrombocytopenic purpura and a platelet count > 90,000 cells/µL without specific medication was referred to pulmonary clinic for evaluation of multiple pulmonary nodules. The patient presented to an outside hospital with fatigue, lack of energy, and dyspnea on exertion for 2 years. She denied fever, cough, chest pain, or weight loss. An initial chest radiograph showed bilateral multiple pulmonary nodules. A chest CT scan revealed multiple nodular lesions, varying in size, in all lobes of both lungs. There was no mediastinal lymphadenopathy or pleural effusion. There was no significant hypermetabolic activity on a subsequent fluorodeoxyglucose PET scan/CT scan, and there had been no significant change. She underwent CT scan-guided percutaneous transthoracic biopsy and bronchoscopy with transbronchial biopsies, all of which were inconclusive. An open lung biopsy was considered.

Accelerometer-based devices can be used to monitor sedation/agitation in the intensive care unit.

PURPOSE: Monitoring sedation/agitation levels in patients in the intensive care unit (ICU) are important to direct treatment and to improve outcomes. This study was designed to determine the potential use of accelerometer-based sensors/devices to objectively measure sedation/agitation in patients admitted to the ICU. MATERIALS AND METHODS: Accelerometer-based devices (actigraphs) were placed on nondominant wrists of 86 patients in the ICU after informed consent. The sedation/agitation levels were classified as deep sedation, light sedation, alert and calm, mild agitation and severe agitation, and measured at regular intervals. The sedation/agitation levels were correlated with the accelerometer data (downloaded raw actigraphy data). RESULTS: The sedation/agitation levels correlated strongly with the accelerometer readings represented by mean actigraphy counts (r = 0.968; P = .007) and the proportion of time spent moving as determined by actigraphy (r = 0.979; P = .004). CONCLUSIONS: Accelerometer data correlate strongly with the sedation/agitation levels of patients in the ICUs, and appropriately designed accelerometer-based sensors/devices have the potential to be used for automating objective and continuous monitoring of sedation/agitation levels in patients in the ICU.

Cardiac tamponade due to low-volume effusive constrictive pericarditis in a patient with uncontrolled type II autoimmune polyglandular syndrome.

Type II autoimmune polyglandular syndrome (APS), a relatively common endocrine disorder, includes primary adrenal insufficiency coupled with type 1 diabetes mellitus and/or autoimmune primary hypothyroidism. Autoimmune serositis, an associated disease, may present as symptomatic pericardial effusion. We present a case of a 54-year old male with APS who developed pericarditis leading to cardiac tamponade with a subacute loculated effusion. After urgent pericardiocentesis intrapericardial pressure dropped to 0, while central venous pressures remain elevated, consistent with acute effusive constrictive pericarditis. Contrast computerized tomography confirmed increased pericardial contrast enhancement. The patient recovered after prolonged inotropic support and glucocorticoid administration. He re-accumulated the effusion 16 days later, requiring repeat pericardiocentesis. Effusive-constrictive pericarditis, an uncommon pericardial syndrome, is characterized by simultaneous pericardial inflammation and tamponade. Prior cases of APS associated with cardiac tamponade despite low volumes of effusion have been reported, albeit without good demonstration of hemodynamic findings. We report a case of APS with recurrent pericardial effusion due to pericarditis and marked hypotension with comprehensive clinical and hemodynamic assessment. These patients may require aggressive support with pericardiocentesis, inotropes, and hormone replacement therapy. They should be followed closely for recurrent tamponade.

Pulmonary arterial hypertension in a patient with ß-thalassemia intermedia and reversal with infusion epoprostenol then transition to oral calcium channel blocker therapy: review of literature.

Pulmonary arterial hypertension (PAH) is a potentially life-threatening complication of thalassemia. A sexagenarian with ß-thalassemia intermedia presented with new-onset dyspnea and syncope. Right heart catheterization confirmed severe PAH. Her functional class IV symptoms and severely elevated mean pulmonary artery pressure prompted the initiation of continuous epoprostenol therapy. Clinical follow-up documented significant improvement in functional class, 6-minute walk distance, and right ventricular size and function as well as pulmonary arterial pressure on echocardiogram. At the patient's request, epoprostenol was down-titrated and eventually discontinued. The patient was then safely transitioned to nifedipine therapy after verification of vasoresponsiveness.

Is the sinonasal questionnaire a useful screening instrument for chronic sinonasal diseases in pulmonary clinics?

Dixon and coworkers (Dixon AE, Sugar EA, Zinreich SJ, et al. Criteria to screen for chronic sinonasal disease. Chest 2009; 136: 1324-1332.) recently developed a five-item questionnaire for diagnosing chronic sinonasal disease in patients with asthma. Our study was designed to determine how frequently patients attending pulmonary clinics have chronic sinonasal disease and the relationship between questionnaire results and a history of sinus disease and current treatment for sinus disease. The participants were patients in pulmonary clinics at Texas Tech University Health Science Center, who answered the sinonasal questionnaire (SNQ). Additional information included a history of sinus disease or sinus/nasal surgery, smoking status, and current relevant medications. The participants included 28 men and 51 women. Patient ages ranged from 38 to 94 years (mean 62.2 years). Of all the patients, 28 patients (35.4%) had prior sinus disease, 10 (12.7%) had history of sinus or nasal surgery, 24 (30.4%) had history of asthma, and 10 (12.7%) were current smokers. Of them, 14 patients (17.7%) used nasal steroids, 12 (15.2%) used antihistamines, four (5.1%) used decongestants, nine (11.4%) used leukotriene inhibitors, 13 (16.5%) used oral corticosteroids, and two (2.5%) used combinations. The SNQ identified 39 patients (49.4%) as having chronic sinonasal diseases. Therefore, 14% of the participants had sinonasal symptoms but no prior diagnosis of sinus disease. Patients with prior sinus disease and nasal steroid use were more likely to have positive questionnaire result (p<0.001 and p<0.032, respectively). The SNQ can identify patients with chronic sinonasal disease in pulmonary clinics. However, the significance of sinonasal disease and its treatment in these patients needs more study.

Sirolimus induced granulomatous interstitial pneumonitis.

OBJECTIVES: Report a case of sirolimus induced granulomatous pneumonitis. BACKGROUND: Sirolimus is used in clinical transplantation as an immunosuppressive agent. Pulmonary toxicity does occur, but only a few cases of sirolimus associated granulomatous interstitial pneumonitis have been reported. METHODS: Case report and literature review. RESULTS: This 53-year-old woman with ESRD from polycystic kidney disease status post deceased donor kidney transplantation presented with fever, progressive dyspnea, and hypoxia for two weeks. She had been switched to sirolimus two months before admission. A CT scan of the chest revealed bilateral ill-defined patchy ground glass opacities. Extensive investigations were negative for infection. Video-assisted thoracoscopic biopsy showed granulomatous interstitial pneumonitis. Her symptoms and infiltrates resolved after sirolimus discontinuation and corticosteroid treatment. CONCLUSIONS: Drugs induced pneumonitis should always be considered in transplant patients after infectious or other etiologies have been excluded. Sirolimus can cause granulomatous infiltrates in the lung possibly secondary to T-cell mediated hypersensitivity.