Granulomatosis with polyangiitis: The trigger cannot be long hidden.

Granulomatosis with polyangiitis, or GPA, is a form of vasculitis that affects multiple organs especially the respiratory tract and the kidneys. The diagnosis is suspected with the clinical presentation and elevated serum titer of antineutrophil cytoplasmic antibodies and confirmed with the biopsy of the affected organ. Viral infection has been described as one of the triggers of the immune system in developing GPA. In this report, we describe a rare case of GPA that developed following cytomegalovirus infection in a patient with unknown immunocompromised medical condition.

Secondary pulmonary alveolar proteinosis in hematologic malignancies.

Pulmonary alveolar proteinosis (PAP), characterized by deposition of intra-alveolar PAS positive protein and lipid rich material, is a rare cause of progressive respiratory failure first described by Rosen et al. in 1958. The intra-alveolar lipoproteinaceous material was subsequently proven to have been derived from pulmonary surfactant in 1980 by Singh et al. Levinson et al. also reported in 1958 the case of 19-year-old female with panmyelosis afflicted with a diffuse pulmonary disease characterized by filling of the alveoli with amorphous material described as "intra-alveolar coagulum". This is probably the first reported case of PAP in relation to hematologic malignancy. Much progress has been made on PAP first described by Rosen which is currently classified as idiopathic or primary or autoimmune PAP. Idiopathic PAP occurs as a result of auto-antibodies directed against granulocyte-macrophage colony stimulating factor (GM-CSF) impeding the surfactant clearing function of alveolar macrophages leading to progressive respiratory failure. Whole lung lavage and GM-CSF therapy has improved outcomes in patients with idiopathic PAP. Despite major advancement in the management of hematologic malignancy and its complications, little is known about the type of PAP first described by Levinson and now known as secondary PAP; a term also used when PAP occurs due to other causes such as occupational dusts. In this article we review and analyze the limited literature available in secondary PAP due to hematologic malignancies and present a case of PAP associated with chronic lymphocytic leukemia successfully treated with bendamustine and rituximab.

Hemoptysis: a serendipitous discovery.

The etiology of hemoptysis can sometimes be difficult to identify and requires a complete and sequential approach. It is also important to initially maintain a broad differential diagnosis. We present a patient who had an extremely rare adenoid cystic carcinoma of the nasopharynx as the cause of hemoptysis. This also highlights the importance of having a uniformly systematic approach to bronchoscopic inspection, with careful attention to areas beyond the actual tracheobronchial tree. Often, the nasopharyngeal and oropharyngeal regions are not as meticulously inspected during bronchoscopy. The fact that careful attention to both nares by bronchoscopic inspection aided the final diagnosis suggests that a thorough examination to these often overlooked areas should be routine.

Post tracheostomy and post intubation tracheal stenosis: report of 31 cases and review of the literature.

BACKGROUND: Severe post tracheostomy (PT) and post intubation (PI) tracheal stenosis is an uncommon clinical entity that often requires interventional bronchoscopy before surgery is considered. We present our experience with severe PI and PT stenosis in regards to patient characteristics, possible risk factors, and therapy. METHODS: We conducted a retrospective chart review of 31 patients with PI and PT stenosis treated at Lahey Clinic over the past 8 years. Demographic characteristics, body mass index, co-morbidities, stenosis type and site, procedures performed and local treatments applied were recorded. RESULTS: The most common profile of a patient with tracheal stenosis in our series was a female (75%), obese (66%) patient with a history of diabetes mellitus (35.4%), hypertension (51.6%), and cardiovascular disease (45.1%), who was a current smoker (38.7%). Eleven patients (PI group) had only oro-tracheal intubation (5.2 days of intubation) and developed web-like stenosis at the cuff site. Twenty patients (PT group) had undergone tracheostomy (54.5 days of intubation) and in 17 (85%) of them the stenosis appeared around the tracheal stoma. There was an average of 2.4 procedures performed per patient. Rigid bronchoscopy with Nd:YAG laser and dilatation (mechanical or balloon) were the preferred methods used. Only 1(3.2%) patient was sent to surgery for re-stenosis after multiple interventional bronchoscopy treatments. CONCLUSION: We have identified putative risk factors for the development of PI and PT stenosis. Differences in lesions characteristics and stenosis site were noted in our two patient groups. All patients underwent interventional bronchoscopy procedures as the first-line, and frequently the only treatment approach.

Obstructive sleep apnea: A practical review.

Obstructive Sleep Apnea (OSA) is a condition of repetitive stoppage of breathing during sleep. OSA has assumed major clinical importance as it is linked with the pathophysiology of many serious medical conditions such as congestive heart failure and stroke. We shall review the history, epidemiology, clinical presentation, evaluation, and management of sleep apnea.

Relationship between the cardiometabolic syndrome and obstructive sleep apnea.

Obstructive sleep apnea (OSA), characterized by cessation of air flow for a minimum of 10 seconds despite continuous respiratory effort, is a prevalent condition in our society. Recent studies demonstrate that OSA is an independent risk factor for insulin resistance and the cardiometabolic syndrome. Hypoxemia and sleep fragmentation from OSA appear to produce autonomic activation, alterations in neuroendocrine function, and increased amounts of inflammatory cytokines (interleukin 6 and tumor necrosis factor alpha). These variables have important roles in the pathogenesis of insulin resistance and the cardiometabolic syndrome. It can be concluded that insulin sensitivity, a key contributor to the pathogenesis of the cardiometabolic syndrome, is mainly determined by the extent of obesity and, to a lesser extent, by OSA. The authors review OSA and summarize recent discoveries and proposed mechanisms of the causal relationship that OSA has with insulin resistance and the cardiometabolic syndrome independent of many confounding comorbidities.