Involvement In Primary Sjogren's Syndrome Research Articles

Progressive myelopathy revealing primary Sjogren syndrome: a case report

Background: Central nervous system (CNS) involvement in primary Sjogren Syndrome (pSS) is infrequent and often poses a diagnostic dilemma due to its diverse clinical presentation. Case presentation: A 66-year-old woman presented to our department with a three-month history of progressive weakness in the lower limbs, sensory loss, and sphincter dysfunction. Spine MRI revealed an intramedullary hyperintense signal on T2 weighted images extending longitudinally and transversally from D6 to D12. Auto-immune tests, viral serologies, and metabolic workups yielded negative results. A minor salivary gland biopsy confirmed chronic grade III sialadenitis. Primary Sjogren Syndrome was diagnosed based on the 2016 ACR-EULAR classification criteria. The patient underwent treatment with glucocorticoids and cyclophosphamide, showing mild improvement after six months. Conclusion: This case highlights the significance of considering Sjogren syndrome in the differential diagnosis of progressive myelopathy. Given the severity of spinal involvement in pSS, early diagnosis and aggressive treatment from the disease’s onset are crucial.

Fibrosing interstitial lung disease in primary Sjogren syndrome.

Interstitial lung disease (ILD) represents the main pulmonary involvement in primary Sjogren syndrome (pSS). A proportion of patients with pSS develop a progressive fibrosing form of ILD, but no data are available about the prevalence of these patterns in pSS patients. Aim of this monocentric, cross-sectional study was to investigate the prevalence of fibrosing patterns in pSS patients with ILD. All consecutive patients fulfilling classification criteria for pSS with a new or previous diagnosis of ILD were enrolled in the study. Diagnosis of ILD was always performed by mean of HRCT and specific patterns were identified according to current classification criteria and divided in two groups according to the detection of a fibrotic pattern. Thirty-four pSS-ILD patients were enrolled in the study (males/females 3/31, median age 69.5 years, median pSS duration 47.5 months). Fibrotic pattern was detected in 52.9% of patients, namely: UIP (13 patients, 38.2%), fibrotic NSIP (4, 11.8%), fibrotic OP (1 2.9%) and group 2 (16 pts, 47.1%) including NSIP (6, 17.6%), OP (4, 11.8%), LIP (2, 5.9%) and unclassifiable (4, 11.8%). These patients were younger and with a shorter pSS duration at ILD diagnosis, in particular ILD diagnosis was prior or concurrent to pSS in 83.3% of cases compared to 62.5% in the group of nonfibrotic pattern (P<0.05). Our data suggest a high prevalence of this pulmonary clinical phenotype in pSS-ILD patients. Since the course of progressive fibrosing pneumonia generally results in respiratory failure, this result could be worthy of further studies.

P300 values in patients diagnosed with primary Sjogren syndrome

Purpose: Primary Sjogren syndrome (PSS) is an autoimmune disease characterized by symptoms of a dry mouth and eyes, in which other organs and systems are widely involved. Central nervous system (CNS) involvement in PSS is reported in a wide range between 2.5–60%. The reason is that the clinical picture can remain asymptomatic despite the presence of CNS involvement in the disease process. In this study, our aim was to evaluate subclinical cognitive impairment in patients with PSS by investigating P300 potential parameters.Method: Forty-three female patients with PSS (mean age: 52.6 ± 11.4 years) and 35 healthy female controls (mean age: 54.5 ± 8.09 years) were included in the study. Mini-Mental State evaluations (MMSE) and brain MRI were performed in the patient and control groups. An event-related evoked potentials test (P300) was applied to those with normal MMSE.Results: The P300 latencies of patients with PSS were significantly longer compared with the control group (p = .019). In patients with PSS, there was no difference in P300 parameters between ANA, Anti-SSA, Anti-SSB-positive and negative patients, and patients with or without sedimentation and CRP elevation. In addition, brain MRI revealed no statistically significant difference between patients with PSS with and without ischemic gliotic lesions (p=.48).Conclusion: In our study, P300 latency was also found to be significantly longer in patients who had no white matter change. We believe that prolonged P300 potential latencies without associated white matter lesions in brain imaging may be associated with subclinical CNS involvement.

Liver Diseases and Autoimmunity.

Based on the principal clinical and pathological features, autoimmune diseases can be broadly divided into localized (involving only a single organ or tissue) and systemic (involving multiple organs). The liver is a lymphoid organ that not only participates in the immune response, but also a target of autoimmune reactions. Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are three major types of autoimmune diseases localized in the liver or autoimmune liver diseases, whereas granulomatous diseases such as sarcoidosis and connective tissue diseases such as systemic lupus erythematosus, rheumatoid arthritis and primary Sjogren syndrome (PSS) are the major systemic autoimmune diseases with liver involvement. Over the past decade, advances have been achieved in the diagnosis and treatment of autoimmune liver diseases and liver involvement in systemic autoimmune diseases.1–5 Accordingly, in this issue of Journal of Clinical Translational Hepatology, the aim of the review articles is to update the knowledge and current management of autoimmune liver diseases and liver involvement in systemic autoimmune diseases. AIH is a chronic inflammatory liver disease of unknown cause, with an estimated prevalence of 0.5–1.0 per 100,000; it is more common in women than in men. There have been extensive studies on this important disease, and remission of the disease is now achievable by immunosuppression; however, the prognosis is very poor if the patients are left untreated. In this issue, Kapila et al. present the clinical manifestations of AIH, update pathogenesis of liver-directed immune injury and new concepts in the understanding of immune tolerance, and finally provide insight into the development of novel therapeutic approaches.6 Sarcoidosis is a systemic, granulomatous disease that usually affects multiple organs including the liver. The estimated prevalence of sarcoidosis is 2–60 per 100,000. So far, little is unknown about its etiology. Liver involvement in sarcoidosis is very common. In this issue, Tadros et al. introduce the epidemiology and clinical spectrum of hepatic sarcoidosis of the disease and explore the underlying mechanism for liver injury in hepatic sarcoidosis.7 Moreover, they provide state-of-the-art knowledge on the diagnosis, differential diagnosis (with PBC and PSC) and treatment of the disease. PSS is a systemic autoimmune disorder with secretory gland dysfunction. The estimated prevalence is 0.1–3.3%, with significant female preponderance, and it is reportedly associated with increased risk of cancers. Liver involvement is one of the commonly reported extraglandular manifestations of PSS, and two main causes of liver disease, namely chronic viral infections and autoimmune liver diseases contribute to liver involvement in PSS. Diagnosis of the causes of liver involvement in PSS is very important but challenging. In this issue, Briton-Zeron et al. 8 specifically describe the causes, and provide the diagnostic strategy accordingly. There is no doubt that the new data provided in this issue will enrich our understanding and improve clinical treatment of autoimmune liver diseases and liver involvement in systemic autoimmune diseases. This is consistent with the objective of Journal of Clinical Translational Hepatology to deliver recent advances in pathogenetic mechanisms, and developments in basic, translational and clinical studies.

Primary Sjogren′s Syndrome presenting with Interstitial lung disease and Myositis

Sjogren's syndrome (SS) is an autoimmune disease, is the most common autoimmune disease following rheumatoid arthritis but remains undiagnosed in more than half. The hallmark histopathological features of SS are periepithelial lymphocytic infiltration of the affected organs. Antibodies to SSA (Ro) and SSB (La) are characteristically associated with SS. The reported frequency of pulmonary involvement in primary SS varies widely, ranging from 9% to 75% and consists of various forms of small airway diseases and interstitial lung diseases (ILD). We are reporting a case of primary SS presenting with ILD and myositis.

Neurological involvement in Primary Sjogren Syndrome: A case report

Introduction: Primary Sjogren Syndrome (PSS) is an autoimmune mediated inflammatory disease, which may affect, aside from exocrine glands, other organs/systems, including the nervous system (both peripheral and central). CASE: The authors hereby report a case of a 67year old woman, admitted due to hypoesthesia of the left foot, which progressively ascended to the infra-mammary region within a period of 7days, weakness of both legs, constipation and xerophtalmia. Neurological examination showed diminished strength, grade 3 of both legs; ataxia; extensor plantar reflexes;and hypoesthesia from D5–D9.

Renal involvement in primary Sjogren syndrome of childhood: case report and literature review

Renal tubular acidosis (RTA) is common in adults with primary Sjogren syndrome (pSS) but to date this condition has only been identified in 12 pediatric cases of pSS. Here we present the case of a 13-year-old, otherwise asymptomatic girl in whom the search for the etiology of incidentally found nephrocalcinosis led to diagnosis of distal RTA and nephrogenic diabetes insipidus secondary to SS-associated tubulointerstitial nephritis. Immunosupressive treatment and alkali/electrolyte supplementation resulted in stable renal function over the 6-year follow-up. A review of the literature focuses on two aspects of pSS: (1) the difficulties in diagnosing pSS in childhood and (2) clinical-pathological features, treatment and outcome of renal tubulointerstitial disease in childhood pSS. SS should be considered in older children, particularly females with otherwise unexplained RTA. A careful search for other renal dysfunctions is necessary, and renal biopsy may be of value in assessing the extent of renal damage and the need for immunomodulatory therapy.

Primary Sjogren’s syndrome: cognitive symptoms, mood, and cognitive performance

To investigate the relationships between self-reported cognitive abilities, psychological symptoms and neuropsychological outcomes in PSS. Patients with Primary Sjogren's syndrome (PSS) and healthy controls completed a comprehensive neuropsychometric battery and questionnaires: the Centers for Epidemiological Scale-Depression, the Profile of Fatigue-mental domain (Prof-M) for cognitive symptoms, Fatigue Severity Scale, and the Short-Form McGill Pain Questionnaire. Female patients with PSS (N = 39) were similar to controls (N = 17) in estimated premorbid intellectual function, age and education. Depression (P = 0.002), cognitive symptoms (P = 0.001), fatigue (P = 0.000003), and pain (P = 0.024) scores were greater in the patient group. Patients with PSS demonstrated inferior performance relative to controls in psychomotor processing (P = 0.027) and verbal reasoning (P = 0.007). Patients with PSS with and without depression had similar performance on multiple tests, but depressed patients had significantly lower scores for executive function (P = 0.041). Cognitive symptoms correlated with verbal memory (P = 0.048), whereas pain correlated with executive function measures (Stroop, P = 0.017) and working memory (Trails B, P = 0.036). In the regression model, depression and verbal memory were independent predictors that accounted for 61% of the variance in cognitive symptoms. The Prof-M is a simple self-report measure which could be useful in screening PSS subjects who may benefit from detailed psychometric evaluation. Our results are consistent with the hypothesis that depression and verbal memory impairment are overlapping but independent aspects of neural involvement in PSS. While pain and depression are significant confounders of cognitive function in PSS, this study suggests that impaired verbal reasoning ability in PSS is not attributable to pain or depression.