Seminars in respiratory and critical care medicine
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Lung transplantation (LT) is an appropriate therapeutic option for patients with severe, fibrocystic pulmonary sarcoidosis refractory to medical therapy. Survival rates following LT for sarcoidosis are generally comparable to other indications. Timing of transplantation for patients with sarcoidosis is challenging because mortality rates are high (27 to 53%) among sarcoid patients awaiting LT. ⋯ Early referral of patients allows for timely evaluation of patients for possible listing. If the risks specific to transplantation in sarcoidosis are considered and carefully evaluated, outcomes are reasonable and match those of other diagnoses. Recurrent sarcoidosis in the lung allografts can occur but does not affect survival or risk for complications.
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Semin Respir Crit Care Med · Feb 2007
ReviewEpidemiology of sarcoidosis: recent advances and future prospects.
Sarcoidosis is by definition a disease of "unknown causes," but recent epidemiologic advances suggest that the long-standing definition of sarcoidosis may soon need to be amended. The recently completed ACCESS (A Case-Control Etiologic Study of Sarcoidosis) study was not able to definitively identify the "cause" of sarcoidosis, but yielded important findings regarding familial and environmental risks that have advanced our understanding of this disease. The HLA-DRB1 associations reported in ACCESS along with the results of two recently completed genome scans of sarcoidosis in German Caucasians and African-Americans, respectively, have further defined the genetics of sarcoidosis. ⋯ Although genetic studies of sarcoidosis in African-American populations are confounded by Caucasian admixture, this same admixture may be useful in identifying sarcoidosis genes linked with African ancestry. Case-only methods may be useful in identifying recent acute exposures linked to disease, genetic variants of risk, and gene-environment interactions. In summary, the epidemiology of sarcoidosis has a promising future that should eventually provide the answers to the etiologic origins of this complex disease.
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The treatment of sarcoidosis remains controversial. Corticosteroids remain the cornerstone of therapy, but immunosuppressive, cytotoxic, and immunomodulatory agents have emerged as viable therapeutic options for patients failing or experiencing adverse effects from corticosteroids. Published data are most extensive with methotrexate, but favorable responses have been noted with leflunomide, azathioprine, antimalarial and antimicrobial agents, and tumor necrosis factor-alpha inhibitors. This review focuses on these novel therapies for sarcoidosis, including indications for use, efficacy, toxicity, and monitoring.
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Sarcoidosis is a chronic granulomatous disorder characterized by an accumulation of activated lymphocytes, predominantly T helper cells, expressing the Th1 phenotype and macrophages at sites of disease activity. Although the cause of sarcoidosis has not been elucidated, several lines of evidence suggest that granuloma formation results from exposure to one or more antigens, eliciting a T lymphocyte response. ⋯ This article discusses in depth the key cellular elements and signals that generate and orchestrate the sarcoid granulomatous response. The precise factors inciting the sarcoid granulomatous response have not yet been identified, but chronic exposure to microbial agents, their products, or inorganic substances may be important in the pathogenesis.
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Sarcoidosis can affect any organ in the body. Frequently extrapulmonary manifestations of the disease are the major cause of morbidity. Treatment of extrapulmonary sarcoidosis often requires consideration of alternative immunosuppressive agents, topical therapy, or therapy that is not specifically directed against the granulomatous inflammation of the disease. This article reviews the clinical presentation and therapy of extrapulmonary sarcoidosis.