Sjögren syndrome: looking forward to the future

Abstract

Primary Sjögren’s syndrome (pSS) is a heterogeneous disease characterised by a wide spectrum of manifestations that vary according to the different stages of the disease and among different subsets of patients. The aim of this qualitative literature review is to summarise the recent advances that have been reported in pSS, ranging from the early phases to the established disease and its complications. We analysed the diagnostic, prognostic, and management aspects of pSS, with a look into future clinical and research developments. The early phases of pSS, usually antedating diagnosis, allow us to investigate the pathophysiology and risk factors of the overt disease, thus allowing better and timely patient stratification. Salivary gland ultrasound (SGUS) is emerging as a valid complementary, or even alternative, tool for histopathology in the diagnosis of pSS, due to a standardised scoring system with good agreement and performance. Other promising innovations include the application of artificial intelligence to SGUS, ultrasound-guided core needle biopsy, and a wide array of novel diagnostic and prognostic biomarkers. Stratifying pSS patients through the integration of clinical, laboratory, imaging, and histopathological data; differentiating between activity-related and damage-related manifestations; and identifying patients at higher risk of lymphoma development are essential steps for an optimal management and individualised treatment approach. As new treatment options are emerging for both glandular and systemic manifestations, there is a need for a more reliable treatment response evaluation. pSS is a complex and heterogeneous disease, and many distinct aspects should be considered in the different stages of the disease and subsets of patients. In recent years, efforts have been made to improve our understanding of the disease, and certainly in the coming years, some of these novelties will become part of our routine clinical practice, thus improving the management of pSS patients.

Does a steroid dementia syndrome really exist? A brief narrative review of what the literature highlights about the relationship between glucocorticoids and cognition

“…23 In some pSS patients, treatment with GCs is mandatory. 24 Some drugs may cause or exacerbate cognitive impairment (Table 1). Long-acting benzodiazepines and anti-cholinergic drugs are the most common ones.…”

Sjögren Syndrome: New Insights in the Pathogenesis and Role of Nuclear Medicine

“…The goal of SS is to be able to stratify pSS through the integration of clinical, laboratory, histopathology, and imaging data; thus, it could be possible to identify which patients have clinical manifestations related to inflammatory activity or sequelae; the possibility to identify which patients are on the risk of lymphoma development it is also important. Because new treatment strategies are emerging, there is also evident the need for new probes for a more reliable treatment response evaluation [ 43 ].…”

Usefulness of ultrasound guided core needle biopsy of the parotid gland for the diagnosis of primary Sjögren's syndrome

“…Patients' clinical and laboratory data (including age, gender, disease duration, previous unstimulated sialometry and Schirmer's tests, and evidence of serum antibodies, as well as antinuclear, anti-Ro/SSA and anti-La/SSB antibodies) were collected from medical charts. The presence of lymphoma development risk factors for patients with pSS was noted at the time of participants' biopsy procedures (glandular swelling, lymphadenopathy, cryoglobulinaemia, a serum monoclonal component, rheumatoid factor, low serum C4 and leucopoenia) (15,17).…”

“…Open surgical biopsy of the parotid gland is a safe technique when performed by expert surgeons, but presents some disadvantages, such as the need for expert surgeons themselves, an operating surgery room and possible adverse effects, which may justify its limited application up to now. In recent years, sonographic evaluation of the parotid and submandibular glands has attracted increased interest, allowing the assessment of the glandular parenchyma and the detection of salivary gland abnormalities (16,17). For years, ultrasound-guided core needle biopsy (US-guided CNB) has been an established method for diagnosis of breast lumps or masses in other body parts (18).…”