Current Respiratory Medicine Reviews - Volume 9, Issue 2, 2013

The development of a solitary chest wall mass due to underlying malignant lymphoma is an uncommon clinical presentation. Diffuse large B-cell lymphoma, a type of non-Hodgkin’s lymphoma, can affect multiple organs including the lungs and pleural space. In some instances, there may be preexisting inflammation such as a chronic pyothorax before lymphoma arises in these locations. Diffuse large B-cell lymphoma may frequently disseminate to extranodal sites, but involvement of the chest wall has been reported in nearly 100 cases. This report details an unusual presentation of diffuse large B-cell lymphoma from an enlarging chest wall mass and an underlying lymphomatous pleural effusion.

Pulmonary hypertension refers to pathological elevation of the pulmonary arterial pressure and can be seen in a variety of clinical conditions. It is progressive and ultimately fatal if untreated. Timely recognition and accurate diagnosis are therefore paramount in providing effective management to patients so as to prevent, reduce or stabilise irreversible damage to the pulmonary vasculature, which ultimately causes right heart failure. PH is often not considered or misdiagnosed and a median time of 14 months from the onset of symptoms to diagnosis has been reported. As well as an awareness of the condition it is imperative that physicians know how to correctly interpret a range of investigations that are necessary in reaching the diagnosis.

Purpose of Review: This review focuses on the pathophysiology of proximal airway infection in the ventilated patient and ways to prevent or treat it. Ventilator-associated tracheobronchitis (VAT) is increasingly recognized as an important entity. While there is some controversy whether VAT is always an essential step in the pathway from oral colonization to deep lung infection, all agree that it is an infection associated with its own morbidity and that it acts as a reservoir of highly resistant organisms. Recent Findings: Recent clinical trials have focused on new prophylactic and treatment protocols for ventilator-associated infection. There are multiple steps in the pathway from pathogenic oropharyngeal colonization to ventilator-associated tracheobronchitis, and/or to deep lung infection, ventilator-associated pneumonia (VAP) where intervention is possible. Oral care protocols, innovative types of endotracheal tubes and cuffs, and targeted therapy for VAT in recent investigations, have shown promise in improving clinical outcomes in the critically ill patients. However, even with diligent attention to all the modifiable risk factors for respiratory infection, complete elimination of VAT and VAP remains unlikely. As long as a patient requires an endotracheal tube which disturbs airway integrity, host defenses will be impaired, and resistant virulent organisms which result from our liberal use of systemic antibiotics will continue to challenge critical care specialists. Summary: This review will focus on: 1) the current understanding of the pathogenesis of VAT, 2) modifiable risk factors, and 3) new approaches to treatment in the ICU which may decrease systemic antibiotic use.

Idiopathic Pulmonary Fibrosis (IPF) is a chronic and progressive lung disease associated with a poor prognosis. A diagnosis of IPF dramatically impacts the life of patients. We conducted a qualitative survey to evaluate patients’ insight and emotions during and after diagnosis. Patients with a physician-confirmed IPF diagnosis participated in an indepth qualitative interview. Common projective techniques were used to reveal underlying feelings and attitudes, with the main objective to generate insights into patients’ emotions and journey, including symptoms, referral patterns, diagnosis process, and follow-up. Additionally, some patients participated in an online Insight Room where they could discuss the disease and its impact on quality of life. Word use related to living with IPF was analyzed. Forty-five patients from five European countries were interviewed. Based on groupings into coping approach and age, four segments of emotions were identified: “combative”, “dejected”, “serene”, and “stoic”. Patients in each of the segments approached the disease in different ways. At the time of diagnosis, patients experienced a range of emotions: devastation, confusion, trust, and apprehension. After diagnosis, three successive emotional phases were identified: coming to terms (diagnosis), reactive coping (acceptance), and proactive coping (ownership of condition). The conversational co-word Insight Room analysis identified four areas of patient concern: the physical problem, family support, the medical world and hope for research. Supporting the emotional status of patients should become a more integral part of the management of IPF at diagnosis as is the standard for other terminal conditions, such as cancer.

Cheyne-Stokes breathing/central sleep apnea (CSB/CSA) commonly occurs in patients with congestive heart failure. Since our previous review, there has been further evidence supporting the clinical significance of this primarily nocturnal breathing disorder in terms of the associated physiologic and biochemical changes that may lead to further deterioration of cardiac function, as well as increased mortality. Suppression of CSB/CSA can be difficult to achieve and a consistently effective therapeutic strategy has not been identified. Although optimizing medical management of the underlying heart failure would be a logical initial intervention, lower prevalence of CSB/CSA has not been observed in groups of patients receiving modern medical therapy. Recently published guidelines have supported nocturnal oxygen, CPAP and adaptive servo-ventilation as standard recommendations for treatment. Further evidence since the last review has backed pacemaker resynchronization as an effective means of suppressing CSB/CSA. Early studies on the feasibility of phrenic nerve stimulation as a means for interrupting CSB/CSA period breathing have shown promise. Other methods including positional therapy, carbon dioxide and acetazolamide have continued to show effect in attenuating CSB/CSA in some patients. More recently, combination therapies, such as nocturnal oxygen and CPAP have been suggested as alternative treatment approaches for CSB/CSA, but have not yet been validated by published evidence. The interaction between HF and SDB are complex, and call for close collaboration between cardiologists and pulmonary specialists for optimal care of patients with these disorders.

Polymyositis and dermatomyositis (PM-DM) are forms of idiopathic inflammatory myositis. Interstitial lung disease (ILD) in PM-DM is recognized as a serious complication and a major cause of death in this disease. In particular, patients with clinically amyopathic dermatomyositis (ADM) sometimes develop rapidly progressive ILD that remains unresponsive to intensive immunosuppresive therapy. A novel autoantibody associated with PM-DM was identified and termed anti-CADM-140/MDA5 antibody. Anti-CADM-140/MDA5 antibody titer correlates with disease activity and predicts the course of ILD associated with ADM. Glucocorticoids are considered the first-line drug treatment for PM-DM patients with ILD, however they are often not sufficient to obtain improvement of ILD as a single agent. Furthermore, the addition of immunosuppressive drugs becomes necessary as steroid sparing agents to avoid the severe side-effects often seen with high-dose steroid treatment. Cyclophosphamide, cyclosporin, and tacrolimus were reported to be effective in treatment of refractory ILD in PM-DM. Although other immunosuppressive agents; mycophenolate mofetil, intravenous immunoglobulin, and anti-TNF agents have appeared as promising agents for refractory PM-DM, the efficacy on ILD in PM-DM is still unknown. Even if treatment is initiated early in the course of the disease, some patients still develop irreversible fatal lung fibrosis under aggressive immunosuppressive therapy. Recently, cases with rapidly progressive ILD associated with clinically ADM were successfully treated with direct hemoperfusion with polymyxin B-immobilized fiber column.

Chronic obstructive pulmonary disease (COPD) affects over 10% of the adult population worldwide and is currently projected to become the third leading cause of death by year 2030 [1]. We believe, COPD should be viewed as a multisystem disorder with clearly identified extra-pulmonary manifestations including weight loss, myopathy, cardiovascular diseases, diabetes, malignancies, bone mineral disease and psychological disorders. Most of these comorbidities are also important not only to understand the burden of this progressive disease, but also to devise effective therapeutic strategies [2]. This article reviews the current understanding of the systemic manifestations of COPD, which includes everything except airflow obstruction, emphysema and chronic bronchitis. For colleagues interested in reviewing the main component of COPD therapy, we urge to review an excellent article published in American Journal of Respiratory Critical Care Medicine volume 187, page 337- 365, Feb 15, 2013.