Ultrasound and Bioptic Investigation of Patients with Primary Sjögren's Syndrome

Abstract

Primary Sjögren's syndrome (pSS) is a chronic and heterogeneous disorder characterized by a wide spectrum of glandular and extra-glandular features. The hallmark of pSS is considered to be the immune-mediated involvement of the exocrine glands and B-cell hyperactivation. This leads pSS patients to an increased risk of developing lymphoproliferative diseases, and persistent (>2 months) major salivary gland enlargement is a well-known clinical sign of possible involvement by B cell lymphoma. Better stratification of the patients may improve understanding of the mechanism underlying the risk of lymphoproliferative disorder. Here, we summarize the role of different imaging techniques and a bioptic approach in pSS patients, focusing mainly on the role of salivary gland ultrasonography (SGUS) and a US-guided core needle biopsy (Us-guided CNB) as diagnostic and prognostic tools in pSS patients with persistent parotid swelling.

Keywords: Sjögren’s syndrome; US-guided core needle biopsy; biopsy; histopathology; lymphoma; salivary gland; salivary swelling; ultrasonography.

Figure 1

Examples of clinical parotid swelling (a) and appearance of mucosa–associated lymphoid tissue (MALT) lymphoma by ultrasound (b). Red arrow: Parotid swelling visible after cutaneous inspection.

Figure 2

Histological picture showing a marginal zone lymphoma of the MALT type in a parotid gland characterized by diffuse coalescence of centrocyte-like lymphocytes with lymphoepithelial lesions (H&E, 200×).