Sarcoidosis Diagnosis and Detection: Summary of Clinical Practice Guidelines

Clinicians should use caution when interpreting the guidelines for the diagnosis and detection of sarcoidosis and must consider unique individual clinical circumstances when managing these patients, according to a summary published in the Annals of the American Thoracic Society.¹

The first evidence-based guideline for the diagnosis and detection of sarcoidosis was recently published by a multidisciplinary committee of experts from the American Thoracic Society.² The committee performed a systematic review of the literature focusing on the following areas of interest and provided recommendations.¹

Establishing the Initial Diagnosis

The diagnosis of sarcoidosis is based on 3 major criteria: a compatible clinical presentation, the finding of non-necrotizing granulomatous inflammation in 1 or more tissue samples, and the exclusion of alternative causes of granulomatous disease.

Lymph Node Sampling

In patients for whom there is high clinical suspicion for sarcoidosis, lymph nodes should not be sampled, while for patients presenting with asymptomatic bilateral hilar lymphadenopathy, there are no recommendations for or against obtaining a lymph node sample. For patients with suspected sarcoidosis and mediastinal and/or hilar lymphadenopathy for whom it has been determined that tissue sampling is necessary, endobronchial ultrasound-guided transbronchial needle aspiration lymph node sampling is recommended.

Screening for and Diagnosis of Extrapulmonary Disease

Baseline screening is recommended for several extrapulmonary diseases (ocular sarcoidosis, renal sarcoidosis, hepatic sarcoidosis, hypercalcemia, hematologic abnormalities, cardiac sarcoidosis) even if symptoms are not present. Recommendations are provided for the diagnostic evaluation of suspected extrapulmonary disease, specifically cardiac sarcoidosis and pulmonary hypertension.

For patients with suspected pulmonary hypertension, the committee suggested initial testing with transthoracic echocardiogram (TTE), and if the TTE is suggestive of pulmonary hypertension, to conduct right heart catheterization. For patients with extracardiac sarcoidosis and suspected cardiac involvement, the committee suggested cardiac magnetic resonance imaging (MRI) rather than a cardiac positron emission tomography or TTE to obtain diagnostic and prognostic data.

Overall, the committee found that the quality of evidence was poor in most of the literature reviewed and stressed the need for future high-quality research.¹ They concluded, “Clinicians should apply caution when interpreting the recommendations and should always consider unique individual clinical circumstances including the individual values and preferences when managing patients with sarcoidosis.”

Disclosure: Several study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors’ disclosures.

References

1. Singha A, Liao S-Y, Herman DD, et al. Summary for clinicians: Clinical practice guideline for the diagnosis and detection of sarcoidosis. Ann Am Thorac Soc. Published online September 24, 2020. doi:10.1513/AnnalsATS.202007-874CME

2. Crouser ED, Maier LA, Wilson KC, et al. Diagnosis and detection of sarcoidosis. An official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med. 2020;201(8):e26-e51.