-
Cells Mar 2021Sarcoidosis is a multi-system disease of unknown etiology characterized by the formation of granulomas in various organs. It affects people of all ethnic backgrounds and... (Review)
Review
Sarcoidosis is a multi-system disease of unknown etiology characterized by the formation of granulomas in various organs. It affects people of all ethnic backgrounds and occurs at any time of life but is more frequent in African Americans and Scandinavians and in adults between 30 and 50 years of age. Sarcoidosis can affect any organ with a frequency varying according to ethnicity, sex and age. Intrathoracic involvement occurs in 90% of patients with symmetrical bilateral hilar adenopathy and/or diffuse lung micronodules, mainly along the lymphatic structures which are the most affected system. Among extrapulmonary manifestations, skin lesions, uveitis, liver or splenic involvement, peripheral and abdominal lymphadenopathy and peripheral arthritis are the most frequent with a prevalence of 25-50%. Finally, cardiac and neurological manifestations which can be the initial manifestation of sarcoidosis, as can be bilateral parotitis, nasosinusal or laryngeal signs, hypercalcemia and renal dysfunction, affect less than 10% of patients. The diagnosis is not standardized but is based on three major criteria: a compatible clinical and/or radiological presentation, the histological evidence of non-necrotizing granulomatous inflammation in one or more tissues and the exclusion of alternative causes of granulomatous disease. Certain clinical features are considered to be highly specific of the disease (e.g., Löfgren's syndrome, lupus pernio, Heerfordt's syndrome) and do not require histological confirmation. New diagnostic guidelines were recently published. Specific clinical criteria have been developed for the diagnosis of cardiac, neurological and ocular sarcoidosis. This article focuses on the clinical presentation and the common differentials that need to be considered when appropriate.
Topics: Diagnosis, Differential; Humans; Organ Specificity; Phenotype; Sarcoidosis
PubMed: 33807303
DOI: 10.3390/cells10040766 -
The New England Journal of Medicine Sep 2021
Review
Topics: Algorithms; Biopsy; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Male; Prognosis; Sarcoidosis
PubMed: 34496176
DOI: 10.1056/NEJMra2101555 -
The Medical Clinics of North America May 2019Sarcoidosis is a multisystemic granulomatous disease that affects individuals worldwide. The lungs are most commonly involved but any organ can be involved. It has... (Review)
Review
Sarcoidosis is a multisystemic granulomatous disease that affects individuals worldwide. The lungs are most commonly involved but any organ can be involved. It has variable manifestations and clinical course. Diagnosis of sarcoidosis is based on clinicopathologic findings and the exclusion of other causes of granulomatous disease. Its hallmark is the formation of granulomas in affected organs. Immunosuppressive therapy is the cornerstone of the management of sarcoidosis and is indicated when there is evidence of symptomatic or progressive disease or when critical organs (ocular, cardiac, nervous system) are involved.
Topics: Humans; Sarcoidosis
PubMed: 30955519
DOI: 10.1016/j.mcna.2018.12.011 -
Medicina Clinica Aug 2022Sarcoidosis is a systemic autoimmune disease that is associated with the development of non-caseating granulomas. The disease especially affects the lymph nodes, lungs,... (Review)
Review
Sarcoidosis is a systemic autoimmune disease that is associated with the development of non-caseating granulomas. The disease especially affects the lymph nodes, lungs, skin and eyes. It is an infrequent but not rare disease, especially in northern Europe, the United States and India. Sarcoidosis affects more women and is diagnosed between the ages of 30 and 50. Etiopathogenically, it is closely linked to environmental factors, especially occupational exposures. Clinically, it stands out for a wide variety of presentation phenotypes (casual diagnosis, involvement of a single organ or systemic presentation). The diagnosis of sarcoidosis is complex and requires the integration of clinical, analytical, radiological, and histolopathogical data carried out by multidisciplinary clinical units. The evolution of the disease is variable, as is the indication for systemic treatment, based on the use of corticosteroids as first-line option, the use of immunosuppressants as second-line therapy, and anti-TNF agents in severe and/or refractory cases.
Topics: Adrenal Cortex Hormones; Female; Humans; Immunosuppressive Agents; Sarcoidosis; Skin; Tumor Necrosis Factor Inhibitors
PubMed: 35680449
DOI: 10.1016/j.medcli.2022.03.009 -
Immunology and Allergy Clinics of North... Aug 2023Sarcoidosis has a multitude of manifestations and affects the human body widely. Pulmonary complaints are most common; however, cardiac, optic, and neurologic... (Review)
Review
Sarcoidosis has a multitude of manifestations and affects the human body widely. Pulmonary complaints are most common; however, cardiac, optic, and neurologic manifestations carry high mortality and morbidity. Acute presentations in the emergency room can cause life-altering effects if not appropriately diagnosed and treated. Generally, less severe cases of sarcoidosis have a favorable prognosis and can be treated with steroid therapy. Resistant and more severe cases of the disease carry high mortality and morbidity. It is incredibly important to arrange specialty follow-up for these patients when needed. This review focuses on the acute presentations of sarcoidosis.
Topics: Humans; Sarcoidosis; Prognosis
PubMed: 37394261
DOI: 10.1016/j.iac.2022.10.011 -
Internal and Emergency Medicine Apr 2018Sarcoidosis is a systemic granulomatous lung disease of unknown origin affecting people of any age, mainly young adults. The disease is extremely heterogeneous with an... (Review)
Review
Sarcoidosis is a systemic granulomatous lung disease of unknown origin affecting people of any age, mainly young adults. The disease is extremely heterogeneous with an unpredictable clinical course. Different phenotypes have been identified: an acute syndrome can be distinguished from subacute and chronic variants. About 20% of patients are chronically progressive and may develop lung fibrosis. Sarcoidosis usually involves the lungs and thoracic lymph nodes, although the skin, eyes, bones, liver, spleen, heart, upper respiratory tract and nervous system can also be affected. No reliable indicators of clinical outcome are available, and there is no single serological biomarker with demonstrated unequivocal diagnostic and prognostic value. Diagnosis requires histological confirmation although a presumptive diagnosis may be acceptable in special conditions. This review examines the diagnostic approach to sarcoidosis involving a multidisciplinary team of specialists in which the internist has the task of identifying all pulmonary and extrapulmonary localizations of the disease and of managing complications and comorbidities.
Topics: Humans; Lung; Sarcoidosis; Tomography, X-Ray Computed
PubMed: 29299831
DOI: 10.1007/s11739-017-1778-6 -
Emergency Medicine Clinics of North... Feb 2022Sarcoidosis has a multitude of manifestations and affects the human body widely. Pulmonary complaints are most common; however, cardiac, optic, and neurologic... (Review)
Review
Sarcoidosis has a multitude of manifestations and affects the human body widely. Pulmonary complaints are most common; however, cardiac, optic, and neurologic manifestations carry high mortality and morbidity. Acute presentations in the emergency room can cause life-altering effects if not appropriately diagnosed and treated. Generally, less severe cases of sarcoidosis have a favorable prognosis and can be treated with steroid therapy. Resistant and more severe cases of the disease carry high mortality and morbidity. It is incredibly important to arrange specialty follow-up for these patients when needed. This review focuses on the acute presentations of sarcoidosis.
Topics: Humans; Sarcoidosis
PubMed: 34782085
DOI: 10.1016/j.emc.2021.08.012 -
Journal of Internal Medicine Jun 2023Sarcoidosis is characterized by noncaseating granulomas which form in almost any part of the body, primarily in the lungs and/or thoracic lymph nodes. Environmental... (Review)
Review
Sarcoidosis is characterized by noncaseating granulomas which form in almost any part of the body, primarily in the lungs and/or thoracic lymph nodes. Environmental exposures in genetically susceptible individuals are believed to cause sarcoidosis. There is variation in incidence and prevalence by region and race. Males and females are almost equally affected, although disease peaks at a later age in females than in males. The heterogeneity of presentation and disease course can make diagnosis and treatment challenging. Diagnosis is suggestive in a patient if one or more of the following is present: radiologic signs of sarcoidosis, evidence of systemic involvement, histologically confirmed noncaseating granulomas, sarcoidosis signs in bronchoalveolar lavage fluid (BALF), and low probability or exclusion of other causes of granulomatous inflammation. No sensitive or specific biomarkers for diagnosis and prognosis exist, but there are several that can be used to support clinical decisions, such as serum angiotensin-converting enzyme levels, human leukocyte antigen types, and CD4 Vα2.3+ T cells in BALF. Corticosteroids remain the mainstay of treatment for symptomatic patients with severely affected or declining organ function. Sarcoidosis is associated with a range of adverse long-term outcomes and complications, and with great variation in prognosis between populations. New data and technologies have moved sarcoidosis research forward, increasing our understanding of the disease. However, there is still much left to be discovered. The pervading challenge is how to account for patient variability. Future studies should focus on how to optimize current tools and develop new approaches so that treatment and follow-up can be targeted to individuals with more precision.
Topics: Male; Female; Humans; Sarcoidosis; Bronchoalveolar Lavage Fluid; Lung; Granuloma; CD4-Positive T-Lymphocytes
PubMed: 36872840
DOI: 10.1111/joim.13629 -
The New England Journal of Medicine Nov 2007
Review
Topics: Adrenal Cortex Hormones; Adult; Child; Female; Heart Diseases; Humans; Lung Transplantation; Male; Nervous System Diseases; Sarcoidosis; Skin Diseases
PubMed: 18032765
DOI: 10.1056/NEJMra071714 -
Clinics in Liver Disease May 2019Hepatic granulomas are a common finding in systemic sarcoidosis, but most patients remain asymptomatic. Elevated alkaline phosphatase is the most common sign of hepatic... (Review)
Review
Hepatic granulomas are a common finding in systemic sarcoidosis, but most patients remain asymptomatic. Elevated alkaline phosphatase is the most common sign of hepatic sarcoidosis (HS). Lacking a specific diagnostic test, the diagnosis of HS is one of exclusion. Therapy may be indicated in a minority of patients to control symptoms, but the effects of therapy in the natural history of HS are unknown.
Topics: Adrenal Cortex Hormones; Cholagogues and Choleretics; Diagnosis, Differential; Humans; Liver Diseases; Liver Transplantation; Sarcoidosis; Ursodeoxycholic Acid
PubMed: 30947880
DOI: 10.1016/j.cld.2018.12.012