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      Management of extrapulmonary sarcoidosis: challenges and solutions.

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          Abstract

          Sarcoidosis is a chronic multisystem disease of unknown etiology characterized by noncaseating granulomas that most often involves the lungs, but frequently has extrapulmonary manifestations, which might be difficult to treat in individual patients.

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          Sarcoidosis.

          Dominique Valeyre, Antje Prasse, Hilario Nunes … (2014)
          Sarcoidosis is a systemic disease of unknown cause that is characterised by the formation of immune granulomas in various organs, mainly the lungs and the lymphatic system. Studies show that sarcoidosis might be the result of an exaggerated granulomatous reaction after exposure to unidentified antigens in individuals who are genetically susceptible. Several new insights have been made, particularly with regards to the diagnosis and care of some important manifestations of sarcoidosis. The indications for endobronchial ultrasound in diagnosis and for PET in the assessment of inflammatory activity are now better specified. Recognition of unexplained persistent disabling symptoms, fatigue, small-fibre neurological impairment, cognitive failure, and changes to health state and quality of life, has improved. Mortality in patients with sarcoidosis is higher than that of the general population, mainly due to pulmonary fibrosis. Predicted advances for the future are finding the cause of sarcoidosis, and the elucidation of relevant biomarkers, reliable endpoints, and new efficient treatments, particularly in patients with refractory sarcoidosis, lung fibrosis, and those with persistent disabling symptoms. Copyright © 2014 Elsevier Ltd. All rights reserved.
          Article 0 comments Cited 301 times – based on 0 reviews     Review now
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            ATS/ERS/WASOG statement on sarcoidosis. American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and other Granulomatous Disorders.

            G W Hunninghake, U Costabel, M Ando … (1999)
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              Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone.

              Yoshikazu Yazaki, Mitsuaki Isobe, Michiaki Hiroe … (2001)
              Cardiac involvement is an important prognostic factor in sarcoidosis, but reliable indicators of mortality risk in cardiac sarcoidosis are unstudied in a large number of patients. To determine the significant predictors of mortality and to assess the efficacy of corticosteroids, we analyzed clinical findings, treatment, and prognosis in 95 Japanese patients with cardiac sarcoidosis. Twenty of these 95 patients had cardiac sarcoidosis proven by autopsy; none of these patients had received corticosteroids. We assessed 12 clinical variables as possible predictors of mortality by Cox proportional hazards model in 75 steroid-treated patients. During the mean follow-up of 68 months, 29 patients (73%) died of congestive heart failure and 11 (27%) experienced sudden death. Kaplan-Meier survival curves showed 5-year survival rates of 75% in the steroid-treated patients and of 89% in patients with a left ventricular ejection fraction > or = 50%, whereas there was only 10% 5-year survival rate in autopsy subjects. There was no significant difference in survival curves of patients treated with a high initial dose (> 30 mg) and a low initial dose (> or = 30 mg) of prednisone. Multivariate analysis identified New York Heart Association functional class (hazard ratio 7.72 per class I increase, p = 0.0008), left ventricular end-diastolic diameter (hazard ratio 2.60/10 mm increase, p = 0.02), and sustained ventricular tachycardia (hazard ratio 7.20, p = 0.03) as independent predictors of mortality. In conclusion, the severity of heart failure was one of the most significant independent predictors of mortality for cardiac sarcoidosis. Starting corticosteroids before the occurrence of systolic dysfunction resulted in an excellent clinical outcome. A high initial dose of prednisone may not be essential for treatment of cardiac sarcoidosis.
              Article 0 comments Cited 139 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (iso-abbrev): Ther Clin Risk Manag
                Title: Therapeutics and clinical risk management
                Publisher: Informa UK Limited
                ISSN: 1176-6336
                ISSN (Print): 1176-6336
                Publication date (Electronic): 2016
                Volume: 12
                Affiliations
                [1 ] Department of Molecular Biosciences, University of Kansas, Lawrence, KS, USA.
                [2 ] Department of Nephrology and Rheumatology, University Medical Center Göttingen, Göttingen, Germany.
                [3 ] Division of Pulmonary, Critical Care, Sleep and Allergy, University of Illinois at Chicago, Chicago, IL.
                [4 ] Division of Rheumatology, University of Illinois at Chicago, Chicago, IL.
                [5 ] Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, University of Miami Miller School of Medicine, Miami, FL.
                [6 ] Department of Medicine, Michigan State University College of Osteopathic Medicine, East Lansing, MI.
                [7 ] Department of Gastroenterology, Hepatology and Nutrition, University of Buffalo, Buffalo, NY.
                [8 ] New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, Louisiana State University Health Sciences Center, New Orleans, LA.
                [9 ] Department of Medicine, University Medical Center of Cincinnati, Cincinnati, OH, USA.
                [10 ] Division of Pulmonary, Critical Care, Sleep and Allergy, University of Illinois at Chicago, Chicago, IL; Division of Rheumatology, University of Illinois at Chicago, Chicago, IL.
                Article
                Publisher Item ID: tcrm-12-1623
                DOI: 10.2147/TCRM.S74476
                PMC ID: 5106225
                PubMed ID: 27853374
                SO-VID: 501ae2e9-8cb9-4d1e-962e-f484aa6ab38d
                History

                Keywords: chronic granulomatous diseases,diagnostic tests,immunosuppressive agents,sarcoidosis
                Data availability:
                Keywords: chronic granulomatous diseases, diagnostic tests, immunosuppressive agents, sarcoidosis

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