Acute transverse myelitis (TM) is a rare, acquired neuro-immune spinal cord disorder that can be idiopathic or related to a secondary disease. Clinical signs and symptoms include motor weakness, sensory alterations, and bowel or bladder dysfunction. Often TM occurs in the younger population or middle-aged adults. This patient\'s presentation is unique in the fact that he does not fall into either of these age categories. In this case, a 72-year-old male with a past medical history of hypertension and type 2 diabetes mellitus presented to the emergency department due to a five-day history of worsening weakness of the upper extremities bilaterally. In addition, the patient reported a new onset of abdominal wall numbness. The patient reported being at a theme park a few days prior, denying any injuries and only complaining of neck discomfort during the car ride home. Labs and imaging were quickly ordered for diagnostic purposes. The patient was diagnosed with TM using magnetic resonance imaging (MRI), lumbar puncture, and clinical signs. The etiology was later discovered to be due to a new diagnosis of Sjögren\'s autoimmune disease. The patient was treated with high-dose intravenous steroids for five days while being monitored for any neurologic changes. The plan was to continue steroids by mouth once discharged from the hospital. Due to poor adherence to discharge instructions, the patient was readmitted after presenting to the emergency department with worsening symptoms. Physicians need to recognize and diagnose TM quickly, as some etiologies are treatable and can prevent further damage to the spinal cord.

Complete congenital heart block (CHB), a rare and fatal bradyarrhythmia observed in children, carries significant mortality and morbidity. When congenital heart block occurs in isolation with a structurally normal heart, it prompts suspicion of an autoimmune etiology, wherein maternal antibodies are transmitted transplacentally, impacting the fetal conducting system. The manifestation of congenital complete atrioventricular block (CCAVB) can lead to complications such as dilated cardiomyopathies, arrhythmias, and fibroelastosis in certain cases. Notably, dilated cardiomyopathy is a significant prognostic factor in children diagnosed with congenital heart block. Pathological investigations have revealed the presence of antibodies, complements, and indicators of inflammation or fibrosis across the myocardium, emphasizing the shared molecular mechanisms between CCAVB and the development of dilated cardiomyopathy (DCM). This article presents the case of a one-year-old female child who presented with signs of dilated cardiomyopathy, later identified through retrospective evaluation as having autoimmune congenital heart block. The mother of the child was diagnosed with Sjogren\'s syndrome, characterized by positive anti-RO titers. Remarkably, the child remained asymptomatic for a year without the need for pacing intervention. The child\'s condition was successfully stabilized with appropriate treatment, and plans for pacemaker insertion will be considered once specific criteria are met. The onset of cardiomyopathy in a known case of CCAVB should serve as a crucial alert for prognostic considerations and the potential necessity for early-pacing intervention.

We report a case of biopsy-proven cutaneous leukocytoclastic vasculitis developing 10 days after starting verapamil and atorvastatin in a patient with long-standing Sjogren\'s syndrome. This highlights the need to monitor for this rare adverse effect.

Rowell syndrome (RS) is characterized by the presentation of lupus erythematosus (LE) with erythema multiforme (EM)-like lesions. It is thought to display a characteristic serologic pattern consisting of a \"speckled-type\" antinuclear antibody (ANA), positive anti-Ro/SSA or anti-La/SSB, or positive rheumatoid factor (RF). We report the case of a patient with subacute cutaneous lupus erythematosus (SCLE) who presented with EM-like lesions responsive to oral corticosteroids.

Primary Sjögren\'s syndrome (pSS) is a chronic inflammatory condition, which presents with symptoms of dryness, pain, fatigue and often symptoms of anxiety and depression. Health-related quality of life (HRQoL) is significantly reduced in pSS and the direct and indirect health costs of pSS are substantial. This study aims to determine how symptom burden, disease activity and demographics associate with HRQoL longitudinally over a median of 24-month follow-up period in pSS.
Longitudinal EuroQoL-5 dimension (EQ-5D)-3L data from the Newcastle pSS cohort (n = 377) were evaluated using a survival analysis strategy. Kaplan-Meier and Cox proportional hazards analysis were performed using baseline Newcastle Sjogren\'s Stratification Tool (NSST) subgroup, EULAR Sjogren\'s Syndrome Patient Reported Index (ESSPRI), EULAR Sjogren\'s Syndrome Disease Activity Index (ESSDAI), disease duration, age and sex as covariates including polypharmacy and comorbidity score, where data were available (n = 191).
Of the 377 pSS participants analysed in this study, 16% experienced a decline in HRQoL to a health state comparable to or worse than death. NSST subgroup and ESSPRI score had a significant relationship with time to \'EQ-5D event\', whereas baseline ESSDAI, age, disease duration and sex did not.
In pSS, symptom burden and to a great extent NSST subgroup, rather than systemic disease activity, has a significant relationship with HRQoL longitudinally. Improvements in symptom burden have the potential to produce significant impacts on long-term HRQoL in pSS.

Central nervous system (CNS) involvement in Sjogren\'s syndrome (SS) has a broad spectrum of presentations. We present a 33-year-old with sudden onset, rapidly progressive quadriplegia, severe dysarthria, bilateral facial palsy, bulbar palsy, and hypernatremia. The MRI of the brain revealed hyperintensity in the central pons diffusion-weighted imaging, T2-weighted imaging, and fluid-attenuated inversion recovery (FLAIR) without abnormal contrast enhancement, consistent with central pontine myelinolysis. However, there was no antecedent history of hyponatremia with rapid correction. The patient responded excellently to sodium correction and pulse methylprednisolone therapy and was erroneously diagnosed as idiopathic hypernatremic osmotic demyelination. One year later, she presented with vague constitutional symptoms, renal tubular acidosis type-1 (distal), hypokalemia with associated myopathy. Subsequent testing for anti-Sjögren\'s-syndrome-related antigen A (SSA)/Ro autoantibodies and a biopsy of the minor salivary gland established the diagnosis of primary Sjogren syndrome (pSS). Remission was achieved with oral prednisolone after her discharge. Neurological signs can be the initial presentation that precedes the classical systemic manifestations of multisystem autoimmune disorders like pSS. In the event of osmotic demyelination, when antecedent hyponatremia with rapid correction is not there, we suggest evaluating for possible autoimmune etiology.

The salivary gland tumors are rare entities and the majority of these are benign. However, there are some entities such as prior neck radiation, certain infections, and systemic diseases which should raise the clinical suspicion for a malignant lesion. Patients with Sjogren syndrome are at increased risk for a salivary gland neoplasm, specifically non-Hodgkin lymphoma. While clinical findings play an important role in the initial workup, imaging plays a critical role in the diagnosis and management. This case describes a patient with Sjogren syndrome who presented with a left face mass where imaging was able to confidently diagnose her with a suspicious parotid neoplasm with lymphoma as the favored diagnosis. After histological evaluation, she was diagnosed with primary parotid mucosa-associated lymphoid tissue (MALT) non-Hodgkin lymphoma after which she went on to non-operative management.

Patients suffering from autoimmune diseases appear to be at greater risk for developing infections with the influenza virus compared to healthy controls due to their immunosuppressive treatment, suggesting the importance of vaccination. Within this literature review, we highlight the importance, efficacy, and safety of influenza vaccination in individuals with autoimmune diseases, including systemic lupus erythematosus (SLE), Sjogren syndrome (SS), rheumatoid arthritis (RA), and inflammatory bowel disease (IBD) in both vaccinated and unvaccinated individuals. Overall, vaccination is generally well tolerated by SLE patients and the literature recommends the inactivated influenza vaccine to SLE patients according to the recommendations and schedules for the general population and annually against seasonal influenza viruses. While the data are still unclear in patients with SS, there does seem to be a general consensus to vaccinate these individuals to prevent harmful risks of influenza disease. In patients with RA and IBD, vaccination efficacy with the inactivated influenza vaccine should be determined on a case-by-case basis, taking patient therapy into account. In light of the current pandemic and global coronavirus disease 2019 (COVID-19) crisis, it is crucial to emphasize the safety and immunogenicity of influenza vaccination in vulnerable individuals suffering from autoimmune diseases. Public health measures are recommended to protect these individuals with vaccinations, keeping in mind the possibility of the multiple COVID-19 vaccines that are currently available.

T follicular helper (Tfh) cells are a critical component of adaptive immunity and assist in optimal Ab-mediated defense. Multiple effector functions of Tfh support germinal center B cell survival, Ab class switching, and plasma cell maturation. In the past 2 decades, the phenotype and functional characteristics of GC Tfh have been clarified allowing for robust studies of the Th subset including activation signals and environmental cues controlling Tfh differentiation and migration during an immune response. A unique, 2-step differentiation process of Tfh has been proposed but the mechanisms underlying transition between unstable Tfh precursors and functional mature Tfh remain elusive. Likewise, newly identified transcriptional regulators of Tfh development have not yet been incorporated into our understanding of how these cells might function in disease. Here, we review the signals and downstream transcription factors that shape Tfh differentiation including what is known about the epigenetic processes that maintain Tfh identity. It is proposed that further evaluation of the stepwise differentiation pattern of Tfh will yield greater insights into how these cells become dysregulated in autoimmunity.

This study is a systematic review of the literature which seeks to estimate the expected treatment outcomes of a patient with Sjogren\'s syndrome (SS) undergoing therapeutic sialendoscopy.
Systematic Review.
PubMed, Scopus, and Cochrane library databases were used to search for studies published as of August 2020 regarding the treatment outcomes of SS with sialendoscopy. The key search terms included \"Sjogren\'s syndrome\" and \"sialendoscopy.\" Only studies in the English language involving more than one human patient were included. PRISMA guidelines were followed in study inclusion and data extraction. The primary outcome assessed was improvement in patient symptoms.
Six studies met criteria and were analyzed in this review, including 125 patients undergoing sialendoscopy of parotid and/or submandibular glands as well as 25 controls. Of these patients, 90% were female with an age range of 18 to 79 years. There was significant diversity in outcome reporting tools. The outcomes of symptom improvement were pooled qualitatively based on improvement noted in each study. Outcomes were defined as partial improvement if the measured outcomes improved and complete improvement if measured outcomes resolved entirely. Despite the limited number of studies on this topic, this meta-analysis suggests that a similar study of therapeutic sialendoscopy could expect to provide at least temporary improvement of symptoms 90% to 99% of the time.
This review provides support for the application of sialendoscopy in the treatment of SS salivary disease. Larger studies with consistent outcome reporting tools and control groups are needed to validate these results and provide a consistent therapy protocol. Laryngoscope, 131:1474-1481, 2021.