Dyspnea is a common, distressing symptom of cardiopulmonary and neuromuscular disease. In a consensus statement, the American Thoracic Society defined dyspnea as “a subjective experience of breathing discomfort that consists of qualitatively distinct sensations that vary in intensity.” Dyspnea is a nonspecific complaint and is one of the most frequent patient complaints. This review discusses the definition, epidemiology, etiology, pathophysiology, peripheral mechanisms, and evaluation of dyspnea. Figures depict cellular metabolism and exercise physiology, and an invasive cardiopulmonary exercise testing (iCPET) flow diagram. Tables list the common causes of dyspnea, invasive cardiopulmonary exercise testing (iCPET) diagnosis, and iCPET characteristics of pulmonary hypertension. This review contains 2 highly rendered figures, 3 tables, and 51 references.

Cholinergic drugs exert their functions by inhibiting acetylcholinesterase, the enzyme responsible for hydrolyzing acetylcholine and ending neuronal or neuromuscular neurotransmission. These compounds are used in clinical medicine to treat various disorders, as pesticides, and as weapons of mass destruction. This review describes the drugs that affect the cholinergic system and discusses stabilization, diagnosis and definitive therapy, principles and controversies of definitive care, and disposition and outcomes for these agents. Figures show acetylcholinesterase hydrolysis of acetylcholine, neurotransmission in the nervous system, the mechanism of inhibition of acetylcholinesterase by an organophosphorus (OP) compound, and the general chemical structure of thion OP and oxon OP agents and carbamates. Tables list OP pesticides associated with OP-induced delayed neuropathy, the effects of cholinesterase inhibition, the symptoms of cholinergic poisoning according to severity, and a treatment algorithm for acetylcholinesterase poisoning. This review contains 4 highly rendered figures, 4 tables, and 85 references.

IgG4-related disease (IgG4-RD) has been observed to affect almost every organ system, with consistent histopathologic findings across systems. IgG4-RD can mimic malignant, infectious, and inflammatory disorders; accordingly, consideration of the histopathologic features of tissue biopsies and rigorous clinicopathologic correlations are essential to avoid misdiagnosis. Since the early 2000s, IgG4-RD has increasingly been recognized as a cause of what was previously referred to as “idiopathic” retroperitoneal fibrosis (RPF), and this IgG4-related RPF is now considered to comprise an important subset of IgG4-RD. This review includes an overview of IgG4-RD and discusses the pathology, pathophysiology, and clinical manifestations of IgG4. IgG4-related RPF is also discussed in this review, with topics including IgG4-related RPF versus RPF of other causes, the differences between RPF and other subsets of IgG4-RD, and treatment of both IgG4-RD and IgG4-related RPF. Figures show the histopathology features of IgG4-RD, immunostaining of tissue for IgG4, IgG4-related RPF and chronic periaortitis, “Mikulicz disease”, IgG4-RD of the lung, IgG4-related renal disease, and type 1 (IgG4-related) pancreatitis. The table lists conditions known previously by other names that often fall within the spectrum of IgG4-RD. This review contains 7 highly rendered figures, 1 table, and 66 references.

A bleeding disorder may be suspected when a patient reports spontaneous or excessive bleeding or bruising, often secondary to trauma. Possible causes can vary between abnormal platelet number or function, abnormal vascular integrity, coagulation defects, fibrinolysis, or a combination thereof. This review addresses hemorrhagic disorders associated with quantitative or qualitative platelet abnormalities, such as thrombocytopenia, platelet function disorders, thrombocytosis and thrombocythemia, and vascular purpuras. Hemorrhagic dis­orders associated with abnormalities in coagulation (e.g., von Willebrand disease and hemophilia) are not covered. An algorithm shows evidence-based practice guidelines for the management of immune thrombocytopenic purpura. Tables list questions regarding bleeding and bruising to ask patients, clinical manifestations of hemorrhagic disorders, typical results of tests for hemostatic function in bleeding disorders, causes of thrombocytopenia, other forms of drug-induced thrombocytopenia, classification of platelet function disorders, and selected platelet-modifying agents. This review contains ­1 highly rendered figure, 7 tables, and 82 references.

In 2013, the International Encephalitis Consortium proposed clinical criteria for acute encephalitis consisting of 24 hours of altered level of consciousness, lethargy, or personality change and at least three additional supportive features. Although viruses are the most common cause of acute encephalitis, not all encephalitides are acute, viral, or even infectious. Chronic encephalitis can be pathologically similar to acute encephalitis, but the causative agents and clinical manifestations vary. Management of encephalitis is largely supportive; however, for many common encephalitides primary preventive approaches exist. This module reviews the epidemiology, manifestations, diagnosis, management, and prevention of various encephalitides, including herpes family encephalitis (herpes simplex virus, varicella-zoster virus, cytomegalovirus, human herpesvirus 6), arbovirus encephalitis (West Nile virus, eastern equine encephalitis virus, tick-borne encephalitis virus, Japanese encephalitis virus), other encephalitides associated with viruses (influenza virus, human immunodeficiency virus, John Cunningham virus, rabies virus), encephalitides associated with bacteria (Mycoplasma pneumonia, Listeria monocytogenes), and autoimmune encephalitis (acute disseminated encephalomyelitis, paraneoplastic and other autoimmune encephalitides, immune reconstitution inflammatory syndrome). Tables include the International Encephalitis Consortium’s supportive feature of encephalitis, differential diagnosis for magnetic resonance imaging (MRI) findings in the patient with suspected encephalitis, diagnostic considerations for triaging workup of infection-associated encephalitis, differential diagnosis of arboviral infections by location of travel or residence, and preventive strategies for select infectious encephalitis. Figures include MRIs showing patients with herpes simplex encephalitis, varicella-zoster virus, eastern equine encephalitis, HIV, Listeria monocytogenes, acute disseminated encephalomyelitis, acute hemorrhagic leukoencephalitis, and immune reconstitution inflammatory syndrome. This review contains 8 highly rendered figures, 5 tables, and 78 references.

This review focuses on the primary category of Sjögren syndrome (SS), a chronic inflammatory condition that is defined by the presence of dry eyes (keratoconjunctivitis sicca) or dry mouth (xerostomia) in the absence of other rheumatologic diseases. SS may also have extraglandular manifestations in the form of pulmonary, renal, gastrointestinal, and neurologic diseases that can cause significant morbidity and increased mortality and is distinct from other connective tissue diseases. Although the etiology of primary SS is unknown, genome-wide association studies are continuing to reveal that susceptibility to the disease is based on genetic predisposition; patients with primary SS have been identified with several non–major histocompatibility complex genetic polymorphisms that are statistically associated with increased disease susceptibility. In a recent study, blood CD4+ T cells from patients with primary SS were shown to differ in their patterns of DNA methylation compared with healthy controls and demonstrated that many genes involved in lymphocyte activation and the immune response were poised for transcription. Treatment of primary SS mainly involves relief of symptoms and prevention of long-term disease complications. Although biologic therapies have been studied, the results so far have been either negative or inconclusive. This review contains 5 highly rendered figures, 5 tables, and 89 references.

Women with disabilities face substantial barriers to obtaining health care services. They are more likely than nondisabled women to smoke cigarettes, to be obese or overweight, to be physically inactive, and to report depressive symptoms, anxiety, and fears. Women with disabilities are often stigmatized as being asexual and experience higher rates of domestic partner and in-home abuse. Physical, communication, and attitudinal barriers within the health care system may prevent women with disabilities from receiving the care they need. This module discusses the definition of disability, prevalence and demographics of disability among US women, health conditions and risk factors, and general considerations in caring for women with disabilities. Selected health issues in caring for women with disabilities, including reproductive and parental rights, menstruation and fertility, contraception, preconception counseling, and menopause, are also discussed. Tables review components of the International Classification of Functioning, Health and Disability, examples of disability definitions, disability indicators using National Health Interview Survey data, health conditions causing chronic mobility disability for women ages 18 to 49, comorbid health conditions reported among women with and without disability, mental health conditions reported by women with and without disability, examples of women’s experiences with inaccessible medical equipment, examples of communication accommodations, and accessible labeling of prescription drug containers. Figures include the International Classification of Functioning, Health and Disability model of disability and graphs showing the percentage of women with basic action difficulties (BADs); with one or more BADs; with complex action limitations; with BADs by racial and ethnic groups; with indicated level of education and income among women with and without BADs; with indicated level of self-reported overall health among women with BADs; by smoking status among women with and without BADs; in body mass index category among women with and without BADs; with indicated physical activity level among women with and without BADs; with indicated heath care service use and lacking health insurance among women with and without BADs; and receiving Pap smears, mammography screening, and colon cancer screening among women with and without BADs. This review contains 12 highly rendered figures, 9 tables, and 86 references.

Rheumatoid arthritis (RA) is among the most common forms of chronic inflammatory arthritis. It affects approximately 1% of adults and is two to three times more prevalent in women than in men. There are no specific laboratory tests for RA; diagnosis depends on a constellation of signs and symptoms that can be supported by serology and radiographs. The disease evolves over many years as a consequence of repeated environmental stress causing inflammation and immune activation followed by a breakdown of tolerance in individuals with a specific genetic background. This review describes the definition of RA; its etiology, including genetics, infections, the role of smoking and citrullination of proteins, and epigenetic mechanisms; and its pathogenesis, including synovial histopathology, bone and cartilage damage, adaptive and innate immunity, and the role of cytokines and intracellular signaling. Tables include the 1987 American Rheumatism Association criteria for the classification of RA and the 2010 American College of Rheumatology/European League Against Rheumatism classification for RA. Figures show citrullinated proteins in airway cells, a section of a proliferative synovium from a patient with a classic RA, and scalloped regions of erosion at the junction between a proliferative inflamed rheumatoid synovium and the bone. This review contains 3 highly rendered figures, 2 tables, and 71 references.

Chest wall and neuromuscular diseases encompass a broad spectrum of illnesses that affect the mechanics of breathing. This chapter reviews the physiology of the respiratory system and the impact of these diseases. A brief discussion of various chest wall and neuromuscular diseases is included. The approach to the evaluation of a patient with suspected chest wall or neuromuscular disease, including key aspects of the history, physical examination, and diagnostic testing, is discussed. Respiratory care, including ventilation, cough, swallowing, and sleep, is described. Tables outline neuromuscular diseases affecting the respiratory system, causes of diaphragm weakness and paralysis, and a comprehensive approach to respiratory care in chest wall and neuromuscular disease. Figures include illustrations of the anatomy of the respiratory system and contraction of the diaphragm, pressure measures above and below the diaphragm, radiographic images of chest wall diseases and measurement of the Cobb angle, a computed tomographic scan of a patient with pectus excavatum, a graph showing patterns of pulmonary function testing in chest wall and neuromuscular disease, and photos of mouthpiece ventilation and a mechanical insufflator-exsufflator device. Videos show a fluoroscopic sniff test demonstrating unilateral diaphragm paralysis and the use of a mechanical insufflator-exsufflator device. This review contains 9 highly rendered figures, 2 videos, 3 tables, and 40 references.

This review examines the most commonly used hemodynamic monitoring devices in the intensive care unit. After a brief review of some important issues in hemodynamic monitoring, a variety of monitoring systems are considered, including arterial catheters, pulmonary artery catheters (PACs), less invasive hemodynamic monitors, central venous oxygen saturation (ScvO2) monitors, and point-of-care (POC) echocardiography. For each system, the basic operating principles, indications and limitations of use, complications, key issues in data interpretation, and evidence regarding utility in patient care are reviewed. Figures depict the distinction between correlation and agreement, a representative Bland-Altman plot, a square wave test, PAC waveforms, principles of pulmonary artery occlusion pressure measurement, measuring pulmonary artery occlusion pressure at end-exhalation, right atrial and ventricular pressure waveform, pulmonary artery pressure waveform, potential pulmonary artery occlusion waveforms, west zones of the lung and the pulmonary artery occlusion pressure measurements, and POC echocardiography. Tables outline landmarks during insertion of the PAC from the internal jugular and subclavian vein insertion sites; indications, contraindications, and complications of PAC insertion; a comparison of less invasive hemodynamic monitors; reasons for decreased mixed or ScvO2; and indications and contraindications for transesophageal echocardiography. This review contains 12 highly rendered figures, 7 tables, and 95 references.