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Showing PubMed Articles, all 15 from Seminars in respiratory and critical care medicine
The Etiologic Role of Infectious Antigens in Sarcoidosis Pathogenesis.
Sarcoidosis is a disease of unknown etiology, characterized pathologically by noncaseating granulomas that most commonly involve the lung, skin, lymph nodes, and eyes. Syndromes with similar pathological and immunologic features to sarcoidosis such as chronic beryllium disease, hypersensitivity pneumonitis, and tuberculosis illustrate that granulomatous diseases may or may not have an infectious etiology. Although the etiology of sarcoidosis remains unknown, recent molecular, genetic, and immunologic studie...
Review: Role of Genetics in Susceptibility and Outcome of Sarcoidosis.
Sarcoidosis develops in genetically predisposed individuals that are exposed to unknown antigens. There is a statistically significant increased risk for the disease among family members of sarcoidosis patients, and the disease differs in different ethnic groups. It is a genetically complex disease, with many genes contributing, both as risk factors but also with an influence on the disease course. The strongest genetic associations with sarcoidosis are found within the major histocompatibility complex (MHC...
Immunologic Response of Sarcoidosis.
Sarcoidosis is a chronic granulomatous disorder characterized by an accumulation of T lymphocytes, macrophages, and immune-effector cells within affected organs. The pathogenesis of sarcoidosis involves a complex interplay between immune and nonimmune cells, with myriad cytokines and chemokines involved in the orchestration and evolution of the granulomatous/fibrotic process. The etiology of sarcoidosis is not known, but a granulomatous response to antigen(s) is likely. Genetic polymorphisms influence the s...
Diagnostic Modalities in Sarcoidosis: BAL, EBUS, and PET.
Advances have been made in minimally invasive diagnostic procedures in sarcoidosis, including bronchoalveolar lavage (BAL), endobronchial ultrasonography-guided transbronchial needle aspiration (EBUS-TBNA), and positron emission tomography (PET). Several independent groups found almost identical predictive values of the CD4:CD8 ratio in BAL for the diagnosis of sarcoidosis. A CD4:CD8 ratio greater than 3.5 shows a high specificity of 93 to 96% for sarcoidosis, but the sensitivity is low (53 to 59%). EBUS-TB...
Assessing Pulmonary Disease and Response to Therapy: Which Test?
Because 75% of deaths attributable to sarcoidosis occur due to progressive respiratory failure, the staging of pulmonary disease and the accurate identification of changes in disease severity with time are both an essential part of clinical management. Historically, pulmonary function tests (PFTs) and chest radiographic appearances have been applied to both goals. Several additional investigations have been proposed as markers of active disease, including gallium scanning, positron emission tomographic (PET...
Neurosarcoidosis: Diagnosis and Management.
Sarcoidosis is a multisystemic inflammatory granulomatous disease that affects both the central and peripheral nervous system. The neurological manifestations depend on the areas of the neuroaxis affected. In the brain, patients with neurosarcoidosis have leptomeningeal and intraparenchymal infiltration of granulomas that leads to, for example, cranial nerve palsies, basal meningitis, and endocrine dysfunction. It can cause peripheral neuropathies such as mononeuritis multiplex and sensorimotor polyneuropat...
Cardiac involvement is undeniably one of the most challenging issues in sarcoidosis. Although autopsy studies reveal heart lesions in 20 to 30% of sarcoid patients, fewer than 5% suffer from clinical disease. Cardiac sarcoidosis (CS) has a predilection for the myocardium, but the pericardium and endocardium may also be affected. CS manifestations are various and most frequently include the following: (1) aberrations of atrioventricular or intraventricular conduction, either silent or symptomatic; (2) ventri...
Sarcoidosis is a systemic disease with skin manifestations. Skin manifestations are classified as nonspecific if they are not characterized by granulomatous inflammation and specific if the lesions have granulomas histologically. Erythema nodosum is the most common nonspecific skin manifestation, and it portends a good prognosis. Specific skin lesions have a varied clinical appearance, although often they can be distinguished by their yellow translucent character. Despite the potential variable appearance,...
Ocular disease occurs in approximately a third of sarcoidosis patients. The rate of disease varies around the world, with Japanese sarcoidosis patients having ocular disease in more than 70% of cases. If untreated, ocular disease can lead to permanent visual impairment, including blindness. The most common manifestation is uveitis, with anterior involvement often being self-limiting, whereas posterior involvement can be chronic. The diagnosis of ocular sarcoidosis in patients with known sarcoidosis usually...
Rheumatologic Manifestations of Sarcoidosis.
Sarcoidosis is a systemic, clinically heterogeneous disease characterized by the development of granulomas. Any organ system can be involved, and patients may present with any number of rheumatologic symptoms. There are no U.S. Food and Drug Administration-approved therapies for the treatment of sarcoidosis. Diagnosing sarcoidosis becomes challenging, particularly when its complications cause patients' symptoms to mimic other conditions, including polymyositis, Sjögren syndrome, or vasculitis. This review...
Calcium and Vitamin D in Sarcoidosis: How to Assess and Manage.
The synthesis of vitamin D is altered by the granulomatous inflammation of sarcoidosis leading to increased production of 1, 25-dihydroxyvitamin D. Mounting evidence suggests that vitamin D is an immunomodulating hormone that inhibits both antigen presentation by cells of the innate immune system, and the cytokine release and proliferation of Th1 cells. These and other extraskeletal health benefits have led to an increase in vitamin D assessment and pharmacological supplementation in the general population....
Quality of Life in Sarcoidosis: Assessment and Management.
This review focuses on current knowledge of the QOL (quality of life) and HS (health status) of sarcoidosis patients. The QOL and HS of sarcoidosis patients are impaired and symptoms are a cause of this impairment. Fatigue appears to be the major problem in sarcoidosis patients. QOL is a concept that concerns patients' evaluation of their functioning in a wide range of domains, but always the physical, psychological, and social domain. HS concerns patients' physical, psychological, and social functioning. T...
Sarcoidosis-Associated Pulmonary Hypertension: Assessment and Management.
Pulmonary hypertension (PH) is a recognized complication of sarcoidosis, with increased morbidity and poor prognosis. Sarcoidosis-associated pulmonary hypertension (SAPH) is typically seen in advanced cases, with pulmonary fibrosis, destruction and obliteration of the pulmonary vasculature, and chronic hypoxemia. PH can, however, occur in the absence of pulmonary fibrosis, suggesting alternative pathophysiological mechanisms. Diverse processes may coexist in the pathogenesis of SAPH, and there is an overlap...
Sarcoidosis is an immune-mediated systemic syndrome of unknown etiology. The treatment of the granulomatous inflammation in sarcoidosis is thus dependent on nonspecific suppression of the immune response. Although steroid-sparing approaches have moved to front-line therapy for many other immune-mediated inflammatory diseases, corticosteroids remain the initial treatment of choice for most patients with sarcoidosis due in large part to the clinical variability and high rates of spontaneous remission. Given t...
