Several reports of suspected oral and ocular manifestations of coronavirus disease 2019 (COVID-19) has prompted investigations into ocular signs, symptoms, and transmission (5).11.2% of patients with COV19 infection had ocular symptoms, including ocular pain, conjunctivitis, dry eye, and floaters, meanwhile, many studies had documented oral symptoms such as dry mouth and dysgeusia in these patients. Our case reported a 39-year-old male, presented with symptoms of dry mouth and dry eye lasting more than 3 months. The patient had recovered from (PCR-confirmed) COVID-19 which lasted 10 days, 4 months ago. The physical examination was normal. Ocular findings include conjunctival hyperemia and superficial punctate keratitis. The anti-nuclear antibody (ANA) was weekly positive at 1/80. Schirmer test considered positive. He continued on 200 mg/day of hydroxychloroquine, along with tear drops until now with remission. Sicca symptoms may be a sequel of COVID-19 infection, and physicians should be aware of this sequel. The sequela of this infection is not understood, with limited data in the literature. Future prospective cohort studies are needed to reveal the impact of these features on oral health.

OBJECTIVE: This study aimed to characterize the whole phenotype of Systemic sclerosis (SSc) patients with sicca symptoms, using major salivary glands Ultrasound (SGUS) parameters, minor salivary glands biopsies (mSGB) and clinical findings, and to compare these characteristics with those from patients with Sjogren\'s Disease (SjD), and patients with sicca manifestations from other causes.
METHODS: Sixty SSc patients fulfilling the 2013 ACR/EULAR classification criteria and with subjective self-declared sicca symptoms were consecutively recruited and had SGUS and mSGB. Fifteen SSc patients without subjective sicca symptoms and 65 patients with sicca symptoms from other causes (including 37 SjD with no SSc).
RESULTS: SSc patients with subjective sicca symptoms had frequent objective clinical (up to 83 %), histological (44 % of Focus score≥1/ mm2) and US anomalies (63 % of OMERACT ≥2). 53 % patients without subjective clinical complaint also had abnormal objective tests, suggesting the existence of a sub clinical involvement of salivary glands in SSc. SjD-SSc patients had more severe glandular involvement as compared to patients with isolated SjD and isolated Sicca-SSc patients (70%, 48,6 % and 38% of patients with OMERACT ≥2 respectively) suggesting additive impact of both diseases on glandular physiology and structure.
CONCLUSIONS: SjD-SSc overlap have more severe sicca features as compared to isolated sicca-SSc and isolated SjD, suggesting a specific impact of SSc on salivary gland physiology. Further translational studies are needed to identify the underlying pathways that could serve as therapeutic targets.

UNASSIGNED: Almost one-quarter of immune checkpoint inhibitor (ICI) recipients experience sicca syndrome, while Sjögren\'s disease (SjD) is estimated at 0.3-2.5%, possibly underreported.
UNASSIGNED: This narrative review (Medline/Embase until January/31/2024) addresses the pathophysiology, incidence, demographic/clinical features, biomarkers, labial salivary gland biopsy (LSGB), fulfillment of the idiopathic SjD (iSjD) classificatory criteria, differential diagnosis, and management of sicca syndrome/SjD associated with ICIs.
UNASSIGNED: SjD associated with ICIs is underdiagnosed, since studies that performed the mandatory SjD investigation identified that 40-60% of patients with sicca syndrome associated with ICIs meet the iSjD classificatory criteria. LSGB played a fundamental role in recognizing these cases, as most of them had negative anti-Ro/SS-A antibody. Despite the finding of focal lymphocytic sialoadenitis in LSGB samples mimicking iSjD, immunohistochemical analysis provided novel evidence of a distinct pattern for sicca syndrome/SjD associated with ICIs compared to iSjD. The former has scarcity of B lymphocytes, which are a hallmark of iSjD. Additionally, patients with sicca syndrome/SjD associated with ICIs have demographical/clinical/serological and treatment response dissimilarities compared to iSjD. Dryness symptoms are more acute in the former than in iSjD, with predominance of xerostomia over xerophthalmia, and partial/complete response to glucocorticoids. Dryness symptoms in ICI-treated patients warrant prompt SjD investigation.

Primary Sjögren\'s syndrome (pSS), a chronic autoimmune condition, has been associated with an increased risk of several cancers. This study aims to delve into the relationship between pSS and the potential development of non-Hodgkin\'s lymphoma (NHL) utilizing an in-depth systematic review and meta-analysis approach. To thoroughly explore the topic, we conducted a thorough examination of the literature, drawing from reputable databases such as ProQuest, PubMed, Web of Science, Cochrane, and Google Scholar. Our data collection spanned until February 8, 2024, with no time limitation. Data were analyzed with Stata 14 software at a significance threshold of p < 0.05. We examined 15 cohort studies encompassing a total of 50,308 individuals from 1997 to 2023. The findings revealed a substantial link between pSS and the risk of NHL, evident across all demographics. Specifically, the standardized incidence ratio (SIR) was generally 8.78 (95% CI 5.51, 13.99), with similar trends observed in both men (SIR, 6.29; 95% CI 1.93, 20.51) and women (SIR, 9.60; 95% CI 5.89, 15.63). Additionally, the SIR (10.50 (95% CI 7, 15.75)), HR (2.82 (95% CI 1.28, 6.18)), and OR (10.50 (95% CI 3.04, 36.28)) indices further supported this association. Furthermore, the risk of non-NHL associated with pSS was noticeable across different age groups of 40-49 years (SIR, 30.13; 95% CI 14.62, 62.08), 50-59 years (SIR, 9.12; 95% CI 5.13, 16.19), and 60-69 years (SIR, 9; 95% CI 4.68, 17.32). pSS substantively augments the likelihood of NHL manifestation. It notably impacts females and those in earlier stages of adulthood with more acuity than males and older cohorts.

The frequency of antibodies to Ku varies in various autoimmune diseases. In 2019, Spielmann et al. identified two types of anti-Ku syndrome based on a hierarchical clustering analysis. Sjögren\'s syndrome occurs both in the first type of anti-Ku syndrome and in the second type. Despite the fact that increased tissue expression of Ku proteins was noted in lymphocytic cells with focal sialoadenitis of the minor salivary glands in patients with primary Sjogren\'s syndrome, only 49 cases of a combination of anti-Ku antibodies and manifestations of Sjogren\'s syndrome have been described in the literature. Some researchers examined patients for the presence of Sjogren\'s syndrome only if they had anti-Ro or anti-La antibodies, although in the literature, there are descriptions of Sjogren\'s syndrome in the presence of only isolated anti-Ku antibodies, as in our case. Literature data on glandular and extraglandular manifestations of Sjögren\'s syndrome in anti-Ku-positive patients are limited. Below, we present the first case of Sjögren\'s syndrome in combination with the first type of anti-Ku syndrome complicated by the development of mucosa-associated lymphoid tissue (MALT) lymphoma. The article also provides a systematic review of the literature on the association of Sjögren\'s syndrome with anti-Ku antibodies.

Sjögren\'s syndrome (SjS) is the most common connective tissue disease with a prevalence of 1:200. Predominantly affecting women, SjS is associated with destruction of the exocrine glands, leading to xerophthalmia and xerostomia. In over 50% of patients, there are also extraglandular manifestations, leading to multiple organ manifestations including polyneuropathies and interstitial lung disease as well as symptoms such as fatigue and arthralgia. Diagnostic procedures include biomarkers, in particular anti-SS-A/Ro antibodies, histology of salivary glands, and salivary gland sonography. There are currently no licensed immunosuppressive drugs for SjS, so current treatment is often based on off-label use of drugs. The European League Against Rheumatism (EULAR) has recently published treatment recommendations based on the prevailing organ manifestations. Several promising controlled trials with novel compounds and concepts are currently in progress.
UNASSIGNED: Das Sjögren-Syndrom ist mit einer Prävalenz von 1:200 die häufigste Kollagenose. Es betrifft hauptsächlich Frauen, geht mit einer Zerstörung der exokrinen Drüsen einher und führt so regelmäßig zu Xerophthalmie oder Xerostomie. In über 50 % der Fälle kommt es auch zu extraglandulären Manifestationen. So können neben Fatigue und Arthralgien sehr variable systemische Organmanifestationen wie beispielsweise Polyneuropathien oder interstitielle Lungenerkrankungen auftreten. Die Diagnostik umfasst Biomarker wie SS-A/Ro-Antikörper, die Histologie der Speicheldrüsen sowie die Speicheldrüsensonographie. Es gibt bislang keine zugelassene immunsuppressive Systemtherapie, sodass die Therapie i. d. R. „off label“ erfolgt. Die European League Against Rheumatism (EULAR) hat Therapieempfehlungen für die verschiedenen Organmanifestationen publiziert. Aktuell laufen mehrere vielversprechende kontrollierte Studien zu unterschiedlichen neuen Ansätzen.

(1) Objective: To determine the diagnostic accuracy of major salivary gland ultrasonography (SGUS) in primary Sjogren\'s syndrome (SS), we used the Outcome Measures in Rheumatology Clinical Trials (OMERACT) scoring system on a large single-centre cohort of patients with sicca syndrome. (2) Method: We retrospectively collected the clinical, imaging and serological data of all the patients referred with a suspicion of SS who underwent SGUS and minor salivary glands biopsy. (3) Results: A total of 132 patients were included. The SGUS scores were correlated between the two sides (p < 0.001). The diagnostic cut-off for SS (AUROC: 0.7408) was 6 for the SGUS-global sum (sensitivity: 32.43%; specificity: 96.84%). The cut-off with the highest specificity for SS diagnosis was 7. In the patients with a final diagnosis of SS, the mean SGUS score was significantly higher (p < 0.001) than that of the non-SS patients (3.73 vs. 1.32 for the SGUS-global sum). A significant correlation was demonstrated between the SGUS scores and final SS diagnosis (p < 0.001), biopsy positivity (p < 0.001), ANA positivity (p = 0.016), Ro-SSA positivity (p = 0.01), and gland fibrosis (p = 0.02). (4) Conclusions: SGUS, using the OMERACT scoring system, has moderate sensitivity and high specificity for the diagnosis of SS. The scoring showed a strong and direct correlation with all the clinical hallmarks of SS diagnosis, such as the positivity of a labial salivary gland biopsy, ANA and Ro-SSA statuses, and salivary gland fibrosis. Because of its high specificity, a SGUS-global score > 6 could be therefore employed for the diagnosis of SS in the case of ANA negativity or the unavailability of a biopsy.

UNASSIGNED: Sjogren\'s syndrome (SS) is an autoimmune disease that mainly affects the salivary and lacrimal glands and further leads to dry mouth and eyes. In recent years, knowledge about the treatment of SS is developing rapidly. This study aims to assess research progress on SS treatment using a bibliometric approach and to identify research hotspots and emerging trends in this area.
UNASSIGNED: The publications related to the treatment of SS were retrieved from the Science Citation Index Expanded (SCI-E) database. The following search terms were used to extract document data: TS=(Sjogren* OR Sicca*) AND TS= (Treat* OR Therap* OR Disease Management). Articles and review articles published in English from 1900 to 2022 were selected. After the manual screening, the publication data were exported to a plain text file and applied for cooperative network analysis, keyword analysis, and reference co-citation analysis by using CiteSpace.
UNASSIGNED: A total of 2038 publications were included in the analysis from 571 journals by 9063 authors. The annual number of published studies and times cited showed an overall upward trend since 1992. There was a degree of national/regional collaboration in this area, but direct collaboration between institutions and authors was still lacking. The country with the highest number of publications was in the United States, followed by China and Japan. Five SS-related treatments as the research hotspots were summarized by analyzing keywords and references, including immunosuppressive and anti-inflammatory therapy, regenerative therapy, gene therapy, surgical treatment, and symptomatic treatment. Among them, B cells, T cells, mucosal-associated invariant T (MAIT) cells, mesenchymal stem cells (MSCs), rituximab, belimumab, cell-target therapy, and immunosuppressive and anti-inflammatory therapy were emerging trends in this field.
UNASSIGNED: This study conducted a data-based and objective introduction to the treatment of SS from a fresh perspective. An analysis of the intellectual bases, research hotspots, and emerging trends in the field will contribute to future research and treatment decisions, which will ultimately benefit SS patients.

In this report, we present a patient with metastatic non-small cell lung cancer who developed Sjögren\'s syndrome secondary to immune checkpoint inhibition. This patient had a typical clinical presentation as well as biochemical signature, developing only 18 months after the start of treatment with PD-1 inhibition (pembrolizumab).

BACKGROUND: Ultrasound examination of the salivary glands (SG) is a quick and noninvasive method to detect and semiquantitatively estimate typical changes in the large SG in Sjögren\'s syndrome (SS). The differential diagnosis of SS is difficult because several diseases and adverse effects of treatment have a similar clinical picture as SS with sicca syndrome and can even induce alterations in the SG (mimic diseases). Hence, for a long time an SG biopsy was regarded as the diagnostic procedure of choice, especially in SS‑A negative patients, whereas the significance of SD sonography is still controversially discussed.
OBJECTIVE: Comparison of typical and atypical changes for SS in the salivary glands in ultrasound and associated histological sections.
METHODS: This article describes six patient cases with antibody positive or negative SS with and without typical SS ultrasound patterns, SS-associated lymphoma, sarcoidosis and IgG4-associated disease. The findings of the sonographic examination of the parotid glands and the associated histology of the SD are explained and put into context.
RESULTS: The SSA antibody positive patients with SS show a typical sonographic pattern with hypoechoic foci, especially if the disease has been present for a long time. This pattern can help support the diagnosis of SS. The ultrasound patterns of the mimic diseases sometimes differ significantly from the typical patterns of pSS. The histological examination of the SG helps to corroborate the diagnosis but low histological focus scores, in particular, require a critical synopsis of the clinical, serological and imaging findings.
CONCLUSIONS: Both salivary gland ultrasound and the histological examination of SG biopsies are justified in the diagnostics and differential diagnosis of SS and sicca syndrome.
UNASSIGNED: HINTERGRUND: Der Ultraschall der Speicheldrüsen (SD) ist eine schnell durchführbare und nichtinvasive Methode, Sjögren-Syndrom(SS)-typische Veränderungen der großen SD zu detektieren und semiquantitativ einzuschätzen. Die Differenzialdiagnose des SS ist komplex, da zahlreiche Krankheiten und Therapienebenwirkungen ein dem SS ähnliches klinisches Bild mit Sicca-Syndrom und z. T. Speicheldrüsenveränderungen verursachen können („Nachahmer-Erkrankungen“). Lange Zeit galt daher die SD-Biopsie, besonders bei SS-A-negativen Patienten, als Diagnostik der Wahl, während der Stellenwert der SD-Sonographie auch heute noch kontrovers diskutiert wird.
UNASSIGNED: Es erfolgt eine Gegenüberstellung SS-typischer und -untypischer Veränderungen der Speicheldrüsen im Ultraschall und dazugehöriger histologischer Schnitte.
METHODS: Sechs Patientenfälle mit Antikörper-positivem bzw. -negativem SS mit und ohne SS-typischen Ultraschallbefund, SS-assoziiertem Lymphom, Sarkoidose sowie IgG4-assoziierter Erkrankung werden beschrieben. Die Befunde der sonographischen Untersuchung der Parotisdrüsen sowie die dazugehörige Histologie der SD werden erläutert und in Kontext gesetzt.
UNASSIGNED: SS-A-Antikörper-positive Patienten mit SS weisen v. a. bei länger bestehender Erkrankung ein typisches sonographisches Muster mit echoarmen Foci auf. Dieses Muster kann die Diagnose eines SS untermauern helfen. Die Ultraschallmuster der Nachahmer-Erkrankungen unterscheiden sich teils deutlich von primäres Sjögren-Syndrom(pSS)-typischen Mustern. Die histologische Untersuchung der SD hilft bei der Diagnosefindung, jedoch bedürfen gerade niedrige histologische Fokus-Scores einer kritischen Zusammenschau der klinischen, serologischen und bildgebenden Befunde.
CONCLUSIONS: Sowohl der Speicheldrüsenultraschall als auch die histologische Aufarbeitung der Speicheldrüsenbiopsien haben eine Berechtigung in der SS-Diagnostik und Differenzialdiagnose des Sicca-Syndroms.