Primary Sjögren's Syndrome: Diagnostic and Prognostic Value of Salivary Gland Ultrasonography Using a Simplified Scoring System

Abstract

Objective. To determine the usefulness and prognostic value of a simplified salivary gland ultrasonography (SGUS) scoring system in primary Sjögren's syndrome (SS). Methods. Patients with primary SS (n ‫؍‬ 105) and controls (n ‫؍‬ 57) were evaluated using a simplified SGUS scoring system. Parenchymal homogeneity in salivary glands was graded from 0 to 3, with grades 0 (normal) and 1 (mild inhomogeneity) being interpreted as normal or unspecific, and grades 2 (several rounded) and 3 (numerous or confluent hypoechoic lesions) as primary SS typical. Associations between SGUS and clinical, histologic, and laboratory disease characteristics were analyzed. Results. The characteristic hypoechoic lesions (score 2 or 3) were found in 52% of primary SS patients and in 1 (1.8%) of controls (P < 0.001). Specificity and positive predictive value of abnormal SGUS for primary SS were both 98%, sensitivity and negative predictive values were 52% and 53%, respectively. Age or disease duration did not influence the SGUS result. Dryness did not differ between normal or abnormal SGUS. However, patients with pathologic SGUS had significantly more often signs and symptoms of systemic complications, higher disease activity, and more frequently markers of lymphoma development, such as salivary gland swelling, skin vasculitis, germinal center-like structures in salivary gland biopsy findings, and CD4؉ T cell lymphopenia. Conclusion. SGUS using a simplified score for assessment of parenchyma dyshomogeneity is highly specific for primary SS and offers the advantage of identifying patients with severe disease or at risk of lymphoma. However, early disease may be missed. SGUS is easy and rapidly performed and may be considered as an item in future modified classification criteria.

Diagnostic utility of Acoustic Radiation Force Impulse (ARFI) imaging in primary Sjoegren`s syndrome

“…We agree that the visualization of parenchymatous inhomogeneity, with sensitivity of 74 % and specificity of 53 %, is a useful tool in the diagnostic setup. The lack of standardized examination protocols and the high reliance on personal physician expertise are expressed in a wide range of measured specificities (73-99 %) and sensitivities (59-94 %), which prevent reliable data acquisition, and therefore preclude comprehensive clinical application [32][33][34][35][36][37]. In concordance with recent studies, we demonstrated good intra-and inter observer reliability for ARFI imaging [38][39][40].…”

“…High-resolution B-mode ultrasound has been suggested as a non-invasive technique in the assessment of pSS. Different scoring systems have been introduced that assess parenchymatous homogeneity, echogenicity, size of the glands, and posterior glandular border [26,[32][33][34][35][36][37]. We agree that the visualization of parenchymatous inhomogeneity, with sensitivity of 74 % and specificity of 53 %, is a useful tool in the diagnostic setup.…”

Scoring hypoechogenic areas in one parotid and one submandibular gland increases feasibility of ultrasound in primary Sjögren’s syndrome

“…In the Hocevar scoring system, homogeneity and hypoechogenic areas are scored separately. On the other hand, in the scoring system used by Theander and Mandl,12 only parenchymal homogeneity is measured. However, when taking a closer look at their scoring system, in fact a combination of homogeneity and hypoechogenic areas is scored 12.…”

Inflammatory arthritis and sicca syndrome induced by nivolumab and ipilimumab

“…On ultrasound imaging, she also had discrete hypoechoic foci occupying more than 50% of her parotid and submandibular glands, changes that are commonly seen in Sjögren's syndrome. 14 One patient had a pre-existing history of severe dry eye disease, but no diagnosis of Sjögren's syndrome and no severe dry mouth symptoms. Two of the patients with sicca syndrome developed other IRAEs; pneumonitis developed in one patient and interstitial nephritis and colitis in the other.…”