What are the guideline sources, guidelines and references for Sarcoidosis ?
Guideline sources The following summarized guidelines for the evaluation and management of sarcoidosis are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023), the European Respiratory Society (ERS 2022; 2021), the European League Against Rheumatism (EULAR 2022), the American Thoracic Society (ATS 2020), the American Heart Association (AHA/HRS/ACC 2019), the American College of Chest Physicians (ACCP 2018), and the British Thoracic Society (BTS/TSANZ/ITS 2008). 1 2 3 4 5 6 7 8 Guidelines 1. Diagnostic investigations Initial laboratory tests: As per ATS 2020 guidelines, consider obtaining a complete blood cell count in patients with sarcoidosis, in order to screen for hematological abnormalities. C Show 2 more As per BTS 2008 guidelines, recognize that serum angiotensin-converting enzyme level has only a limited role in diagnosis and does not contribute to monitoring patients with pulmonary sarcoidosis when added to serial lung function and imaging. B https://web.pathway.md/diseases/recP054VjSPdfVohc 1/5 6/24/23, 3:05 PM Sarcoidosis Pathway Evaluation for pulmonary hypertension: consider obtaining a TTE in patients with sarcoidosis in whom pulmonary hypertension is suspected. C Show 2 more Evaluation for cardiac involvement: consider obtaining a baseline ECG to screen for possible cardiac involvement in patients with extracardiac sarcoidosis, even in the absence of symptoms or signs of cardiac disease. C Show 3 more Evaluation for renal sarcoidosis: consider obtaining baseline serum creatinine in patients with sarcoidosis, in order to screen for renal sarcoidosis. C Evaluation for hepatic sarcoidosis: consider obtaining baseline serum ALP in patients with sarcoidosis, in order to screen for hepatic sarcoidosis. C Evaluation for ocular involvement: consider obtaining a baseline eye examination in patients with sarcoidosis (even in the absence of ocular symptoms), in order to screen for ocular sarcoidosis. C Screening for chronic infections: screen for HBV infection in all patients eligible for treatment with conventional synthetic, biological, or targeted synthetic DMARDs, immunosuppressants, or corticosteroids (according to dose and duration). B Show 3 more 2. Diagnostic procedures BAL fluid analysis: recognize that typical BAL cellular profiles may allow a diagnosis of sarcoidosis with greater confidence, if the diagnosis is uncertain after clinical assessment and HRCT. B Lymph node biopsy: consider not performing lymph node sampling in patients for whom there is a high clinical suspicion for sarcoidosis (e.g., Lofgren's syndrome, lupus pernio, or Heerfordt's syndrome). D Show 2 more Lung biopsy: As per ERS 2022 guidelines: Consider performing transbronchial lung cryobiopsy in patients with undiagnosed ILD, whether deemed eligible or ineligible for surgical lung biopsy, if obtaining histopathological data is indicated. C Consider performing step-up surgical lung biopsy in patients with undiagnosed ILD and a non- informative transbronchial lung cryobiopsy, if obtaining histopathological data is indicated. C As per BTS 2008 guidelines, perform endobronchial biopsy samples in addition to transbronchial lung biopsy in patients with suspected sarcoidosis, since they are frequently positive, are associated with low morbidity and increase the diagnostic yield. B https://web.pathway.md/diseases/recP054VjSPdfVohc 2/5 6/24/23, 3:05 PM Sarcoidosis Pathway 3. Medical management Management of pulmonary sarcoidosis: As per ERS 2021 guidelines, initiate corticosteroids to improve and/or preserve FVC and quality of life in untreated patients with major involvement from pulmonary sarcoidosis at higher risk of future mortality or permanent disability. B Show 2 more As per ACCP 2018 guidelines, do not use routine ICSs for the treatment of chronic cough in patients with pulmonary sarcoidosis. D As per BTS 2008 guidelines, do not initiate treatment for pulmonary sarcoidosis in patients with asymptomatic stage I disease D or asymptomatic stage II or III disease with mildly abnormal lung function and stable disease, because of the high rates of spontaneous remission. D Show 7 more Management of cardiac sarcoidosis: As per ERS 2021 guidelines, initiate corticosteroids (with or without other immunosuppressive agents) in patients with evidence of functional cardiac abnormalities, including heart block, dysrhythmias or cardiomyopathy. B As per ACC 2019 guidelines: Consider performing permanent pacing, with additional defibrillator capability if needed and meaningful survival of > 1 year is expected, in patients with cardiac sarcoidosis and second- degree Mobitz type II AV block, high-grade AV block, or third-degree AV block. C Consider performing permanent pacing, with additional defibrillator capability if needed and meaningful survival of > 1 year is expected, without further observation for reversibility in patients with second-degree or third-degree AV block associated with cardiac sarcoidosis. C Management of cutaneous sarcoidosis: Consider initiating oral corticosteroids to reduce skin lesions in patients with cutaneous sarcoidosis and cosmetically important active skin lesions unable to be controlled by local treatment. C Consider adding infliximab to reduce skin lesions in patients with cutaneous sarcoidosis treated with corticosteroids and/or other immunosuppressive agents and have continued cosmetically important active skin disease. C Management of neurosarcoidosis: Consider adding methotrexate in patients with neurosarcoidosis treated with corticosteroids and have continued disease. C Consider adding infliximab in patients with neurosarcoidosis treated with corticosteroids and a second-line agent (methotrexate, azathioprine, mycophenolate mofetil) and have continued disease. C Management of sarcoidosis-related fatigue: Consider offering a pulmonary rehabilitation progra and/or inspiratory muscle strength training for 6-12 weeks in patients with sarcoidosis with troublesome fatigue. C https://web.pathway.md/diseases/recP054VjSPdfVohc 3/5 6/24/23, 3:05 PM Sarcoidosis Pathway Consider initiating dexmethylphenidate or armodafinil for 8 weeks, after consideration of a pulmonary exercise or rehabilitation program, to test its effect on fatigue and tolerability in patients with sarcoidosis with troublesome fatigue not related to disease activity. C 4. Preventative measures Routine immunizations: consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications. C Show 17 more Prophylaxis for Pneumocystis jirovecii pneumonia: consider administering prophylaxis against P. jirovecii pneumonia in patients initiating high-dose corticosteroids, especially in combination with immunosuppressants and depending on the risk-benefit ratio. C Likelihood Ratios Pertinent positives The following findings increase the probability of sarcoidosis in adults. -1 Finding LR+ Value Increased serum angiotensin converting enzyme activity (> 54 nmol/mL/min) 3.6 Pertinent negatives The following findings decrease the probability of sarcoidosis in adults. -1 Finding LR- Value serum angiotensin converting enzyme activity not increased ( 54 nmol/mL/min) 0.5 References 1. Anne R Bass, Eliza Chakravarty, Elie A Akl et al. 2022 American College of Rheumatology Guideline for Vaccinations in Patients With Rheumatic and Musculoskeletal Diseases. Arthritis Rheumatol. 2023 Jan 4. Online ahead of print. Open 2. Robert P Baughman, Dominique Valeyre, Peter Korsten et al. ERS clinical practice guidelines on treatment of sarcoidosis. Eur Respir J. 2021 Dec 16;58 6 2004079. Open 3. Dani l A Korevaar, Sara Colella, Markus Fally et al. European Respiratory Society guidelines on transbronchial lung cryobiopsy in the diagnosis of interstitial lung diseases. Eur Respir J. 2022 Nov 10;60 5 2200425. Open https://web.pathway.md/diseases/recP054VjSPdfVohc 4/5 6/24/23, 3:05 PM Sarcoidosis Pathway 4. Elliott D Crouser, Lisa A Maier, Kevin C Wilson et al. Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2020 Apr 15;201 8):e26- e51. Open 5. B Bradley, H M Branley, J J Egan Irish Thoracic Society) et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax. 2008 Sep;63 Suppl 5:v1 58. Open 6. Fred M Kusumoto, Mark H Schoenfeld, Coletta Barrett et al. 2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2019 Aug 20;140 8):e382-e482. Open 7. Surinder S Birring, Joanne E Kavanagh, Richard S Irwin et al. Treatment of Interstitial Lung Disease Associated Cough: CHEST Guideline and Expert Panel Report. Chest. 2018 Oct;154 4 904 917. Open 8. George E Fragoulis, Elena Nikiphorou, Mrinalini Dey et al. 2022 EULAR recommendations for screening and prophylaxis of chronic and opportunistic infections in adults with autoimmune inflammatory rheumatic diseases. Ann Rheum Dis. 2022 Nov 3;ard-2022 223335. Open 9. Michael Glikson, Jens Cosedis Nielsen, Mads Brix Kronborg et al. 2021 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy. Eur Heart J. 2021 Sep 14;42 35 3427 3520. Open 10. West SG. Current management of sarcoidosis I pulmonary, cardiac, and neurologic manifestations. Curr Opin Rheumatol. 2018 May;30 3 243 248. Open 11. Expert Panel on Neurological Imaging, Vikas Agarwal, Lubdha M Shah et al. ACR Appropriateness Criteria Myelopathy: 2021 Update. J Am Coll Radiol. 2021 May;18 5S S73 S82. Open https://web.pathway.md/diseases/recP054VjSPdfVohc 5/5