Diagnosis and Detection of Sarcoidosis

Publication Date: April 1, 2020
Last Updated: March 14, 2022

Diagnosis

Lymph Node Sampling

In patients for whom there is a high clinical suspicion for sarcoidosis (e.g., Löfgren’s syndrome, lupus pernio, or Heerfordt’s syndrome), the ATS suggests NOT sampling lymph nodes.
(C, VL)
Remarks: Patients who do not undergo lymph node sampling require close clinical follow-up.
620
For patients presenting with asymptomatic, bilateral hilar lymphadenopathy, the ATS makes no recommendations for or against obtaining a lymph node sample.
(, )
Remarks: If lymph node sampling is not obtained, close clinical follow-up is a reasonable alternative approach.
620
For patients with suspected sarcoidosis and mediastinal and/or hilar lymphadenopathy for whom it has been determined that tissue sampling is necessary, the ATS suggests endobronchial ultrasound (EBUS)-guided lymph node sampling, rather than mediastinoscopy, as the initial mediastinal and/or hilar lymph node sampling procedure. (C, VL)
620

Screening for Extrapulmonary Disease

For patients with sarcoidosis who do not have ocular symptoms, the ATS suggests a baseline eye examination to screen for ocular sarcoidosis. (C, VL)
620
For patients with sarcoidosis who have neither renal symptoms nor established renal sarcoidosis, the ATS suggests baseline serum creatinine testing to screen for renal sarcoidosis. (C, VL)
620
For patients with sarcoidosis who have neither hepatic symptoms nor established hepatic sarcoidosis, the ATS suggests baseline serum alkaline phosphatase testing to screen for hepatic sarcoidosis. (C, VL)
620
For patients with sarcoidosis who have neither hepatic symptoms nor established hepatic sarcoidosis, the ATS makes no recommendation for or against baseline serum transaminase testing. (, )
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For patients with sarcoidosis who do not have symptoms or signs of hypercalcemia, the ATS recommends baseline serum calcium testing to screen for abnormal calcium metabolism. (S, VL)
620
If assessment of vitamin metabolism is deemed necessary in a patient with sarcoidosis, such as to determine if vitamin replacement is indicated, the ATS suggests measuring both 25- and 1,25- OH vitamin levels before vitamin replacement. (C, VL)
620
The ATS suggests that patients with sarcoidosis undergo baseline complete blood cell count testing to screen for hematological abnormalities. (C, VL)
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For patients with extracardiac sarcoidosis who do not have cardiac symptoms or signs, the ATS suggests performing baseline ECG to screen for possible cardiac involvement. (C, VL)
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For patients with extracardiac sarcoidosis who do not have cardiac symptoms or signs, the ATS suggests NOT performing routine baseline transthoracic echocardiography (TTE) or 24-hour ambulatory ECG (Holter) monitoring to screen for possible cardiac involvement.
(C, VL)
Remarks: The panel recognizes the low risks attendant to the use of TTE or Holter to screen for cardiac sarcoidosis. Thus, these tests should be considered on a case-by-case basis.
620

Diagnostic Evaluation of Suspected Extrapulmonary Disease

For patients with extracardiac sarcoidosis and suspected cardiac involvement, the ATS suggests cardiac magnetic resonance imaging (MRI), rather than positron emission tomography (PET) or TTE, to obtain both diagnostic and prognostic information. (C, VL)
620
For patients with extracardiac sarcoidosis and suspected cardiac involvement who are being managed in a setting in which cardiac MRI is not available, the ATS suggests dedicated PET, rather than TTE, to obtain diagnostic and prognostic information. (C, VL)
620
For patients with sarcoidosis in whom pulmonary hypertension (PH) is suspected, the ATS suggests initial testing with TTE. (C, VL)
Remarks: “PH is suspected” refers to clinical manifestations, including exertional chest pain and/or syncope, exam findings of a prominent P2 or S4, reduced 6-minute walk distance, desaturation with exercise, reduced DLCO, increased pulmonary artery diameter relative to ascending aorta diameter (e.g., by compute tomography [CT] scan), elevated brain natriuretic factor, and/or fibrotic lung disease.
620
For patients with sarcoidosis in whom PH is suspected and a transthoracic echocardiogram is suggestive of PH, the ATS suggests right heart catheterization to definitively confirm or exclude PH. (C, VL)
620
For patients with sarcoidosis in whom PH is suspected and a transthoracic echocardiogram is NOT suggestive of PH, the need for right heart catheterization should be determined on a case-by-case basis. (, )
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Recommendation Grading

Overview

Title

Diagnosis and Detection of Sarcoidosis

Authoring Organization

American Thoracic Society

Publication Month/Year

April 1, 2020

Last Updated Month/Year

November 22, 2024

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Document Objectives

The purpose of this clinical practice guideline is to make recommendations that address uncertainties that are commonly confronted by clinicians relating to the diagnosis and detection of sarcoidosis.

Target Patient Population

Patients with suspected or confirmed pulmonary sarcoidosis.

Target Provider Population

Pulmonary, rheumatology, or other clinicians who manage patients with suspected or confirmed pulmonary sarcoidosis.

PICO Questions

  1. Should Lymph Node Sampling Be Performed in a Patient Presenting with Asymptomatic Bilateral Hilar Lymphadenopathy?

  2. Should Patients with Suspected Sarcoidosis and Mediastinal and/or Hilar Lymphadenopathy, for Whom It Has Been Determined That Tissue Sampling Is Necessary, Undergo EBUS-guided Lymph Node Sampling or Mediastinoscopy as the Initial Mediastinal and/or Hilar Lymph Node Sampling Procedure?

  3. Should Patients with Sarcoidosis Who Do Not Have Ocular Symptoms Undergo Screening for Ocular Sarcoidosis by Routine Eye Examination?

  4. Should Patients with Sarcoidosis Who Do Not Have Renal Symptoms Undergo Screening for Renal Sarcoidosis by Routine Serum Creatinine Testing?

  5. Should Patients with Sarcoidosis Who Do Not Have Hepatic Symptoms Undergo Screening for Hepatic Sarcoidosis by Routine Transaminase and Alkaline Phosphatase Testing?

  6. Should Patients with Sarcoidosis Who Do Not Have Symptoms or Signs of Hypercalcemia Undergo Screening for Abnormal Calcium Metabolism by Routine Serum Calcium and Vitamin D Testing?

  7. Should Patients with Sarcoidosis Undergo Screening for Hematological Abnormalities by Routine Complete Blood Cell Count Testing?

  8. Should Patients with Sarcoidosis Who Do Not Have Cardiac Symptoms or Signs Undergo Routine Screening for Cardiac Sarcoidosis using ECG, TTE, or 24-Hour Ambulatory ECG Monitoring?

  9. Should Patients Who Are Suspected of Having Cardiac Sarcoidosis Undergo Cardiac MRI, TTE, or PET as an Initial Imaging Test?

  10. Should Patients with Sarcoidosis Who Are Suspected of Having PH Undergo TTE?

Inclusion Criteria

Male, Female, Adult, Older adult

Health Care Settings

Ambulatory

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Diagnosis, Assessment and screening

Diseases/Conditions (MeSH)

D012507 - Sarcoidosis

Keywords

pulmonary hypertension, sarcoidosis, tuberculosis, cardiac sarcoidosis, rare lung disease, Lymp nodes, endobronchial ultrasound biopsy

Source Citation

Crouser, E, Maier, L, Wilson, K, Bonham, ., Morgenthau, A, & Patterson, K. et al. (2020). Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med, 201(8), e26-e51. doi: 10.1164/ rccm.202002-0251st

Supplemental Methodology Resources

Data Supplement