Cases Of Sjogren's Syndrome Research Articles

Sjogren's Antibodies and Neonatal Lupus: A Scoping Review.

Sjogren's syndrome (SS) is an autoimmune disease characterized by inflammation of exocrine glands. The disorder predominantly affects middle-aged women. Autoantibodies, includinganti-SS-A/Ro and anti-SS-B/La antibodies, are present in most cases of SS. These antibodies can cross the placenta and likely play a role in pregnancy complications as well as the development of neonatal lupus, resulting in congenital heart block (CHB). It is essential to monitor the fetus for CHB during pregnancy. In particular, screening with echocardiography and monitoring heart rate at home are recommended practices. Regarding medical management, hydroxychloroquineand glucocorticoids have shown promise in reducing cardiac manifestations, but further research is needed to elucidate their longer term efficacy and safety. This scoping review analyzes literature from 2001 to 2024, focusing on pregnancy outcomes among women with SS, clinical manifestations of neonatal lupus, the role of anti-SS-A/Ro and anti-SS-B/La antibodies in the development of neonatal lupus and CHB, and emphasizes the need for future research efforts to refine treatment protocols and enhance clinical care strategies for pregnant women with SS.

Prevalence and comorbidities of Sjogren's syndrome patients in the Community of Madrid: A population-based cross-sectional study.

To estimate the prevalence, sociodemographic characteristics and comorbidities of Sjogren's syndrome (SS) patients in the Community of Madrid. A population-based cross-sectional cohort of SS patients was derived from the information system for rare diseases in the Community of Madrid (SIERMA) and confirmed by a physician. The prevalence per 10,000 inhabitants among people aged ≥18years in June 2015 was calculated. Sociodemographic data and accompanying disorders were recorded. Univariate and bivariate analyses were performed. A total of 4,778 SS patients were confirmed in SIERMA; 92.8% were female, with a mean age of 64.3 (standard deviation=15.4) years. A total of 3,116 (65.2%) patients were classified as primary SS (pSS), and 1,662 (34.8%) as secondary SS (sSS). The prevalence of SS among people aged ≥18 years was 8.4/10,000 (95%Confidence interval [CI]=8.2-8.7). The prevalence of pSS was 5.5/10,000 (95%CI=5.3-5.7), and that of sSS was 2.8/10,000 (95%CI=2.7-2.9), with rheumatoid arthritis (20.3%) and systemic lupus erythematosus (8.5%) being the most prevalent associated autoimmune diseases. The most common comorbidities were hypertension (40.8%), lipid disorders (32.7%), osteoarthritis (27.7%) and depression (21.1%). The most prescribed medications were nonsteroidal anti-inflammatory drugs (31.9%), topical ophthalmic therapies (31.2%) and corticosteroids (28.0%). The prevalence of SS in the Community of Madrid was similar to the overall prevalence worldwide observed in previous studies. SS was more frequent in women in their sixth decade. Two out of every three SS cases were pSS, while one-third were associated predominantly with rheumatoid arthritis and systemic lupus erythematosus.

Detection of xerostomia, Sicca, and Sjogren\u2019s syndromes in a national sample of adults

ObjectivesTo assess the prevalence and determinants of xerostomia among adults and identify how many of the ones experiencing xerostomia have Sicca and Sjogren's syndromes.Materials and methodsThis cross-sectional study included 1405 35–74-year-old Lithuanians (51.7% response rate) from the five largest Lithuanian cities and 10 peri-urban and rural areas that were randomly selected from each of the 10 Lithuanian counties. Xerostomia was determined by the self-reported experience of dry mouth as "often" or "always". A dentist diagnosed Sicca syndrome by unstimulated whole sialometry and the Schirmer's test, and all cases were referred to a rheumatologist to confirm Sjogren's syndrome. Self-reported questionnaires collected data about the determinants.ResultsThe prevalence of xerostomia was 8.0% (n = 112), Sicca syndrome was diagnosed for 8 participants (0.60%), and Sjogren's syndrome for 2 participants (0.14%), with this being the first time it was diagnosed. Experiencing xerostomia was associated with older age (OR 1.7, 95% CI 1.1–2.6), urban residence (OR 3.3, 95% CI 1.6–5.0), presence of systemic diseases (OR 2.5, 95% CI 1.4–3.3), and the use of alcohol (OR 0.6, 95% CI 0.4–0.9). The higher proportion of participants with Sicca syndrome involved females, of older age, having systemic diseases, and using medications.ConclusionsThe prevalence of xerostomia was 8.0% and the determinants of xerostomia were older age, urban residence, systemic diseases, and absence of using alcohol. In total, 0.6% of participants had Sicca syndrome, which was more prevalent among females, older subjects, those with systematic diseases, and those using medications. Sjogren's syndrome was diagnosed in 0.14% of participants.Clinical relevanceDental clinicians need to be trained to identify potential Sjogren's syndrome cases.

Pericarditis as an initial manifestation of Sjögren syndrome in a child

Abstract Introduction: Primary Sjögren syndrome (SS) is a chronic systemic autoimmune disease characterized by lymphocytic inflammation of exocrine glands and epithelia in multiple sites. SS is usually difficult to diagnose particularly in children because SS is rare for this age group, and various clinical signs and symptoms, without typical sicca symptoms, are shown as initial manifestations in pediatric SS patients. There have been no reports on cases of pediatric primary SS with symptomatic pericarditis being the initial manifestation. We report a case of primary SS in a pediatric patient who developed symptomatic pericarditis. Patient concerns: An 11-year-old girl presented with a 2-day history of fever, and chest and left shoulder pains. The pain was exacerbated when she was in a supine position. Diffuse ST-segment elevations on the electrocardiogram, presence of pericardial friction sounds, exacerbation of diastolic pericardial effusion on the echocardiogram, and continuous chest pain indicated acute symptomatic pericarditis. Diagnosis: The results of the blood antibody test, salivary gland scintigram, and salivary gland biopsy led to the diagnosis of SS. Also, this patient has no hypocomplementemia, and classification criteria of American College of Rheumatology of systemic lupus erythematosus and mixed connective tissue disease were not fulfilled. Intervention: Prednisolone therapy relieved her symptoms. Subclinical Hashimoto's thyroiditis, a well-known complication of primary SS, was observed; however, prescriptions were not needed. Outcomes: The clinical signs and symptoms of systemic lupus erythematosus and mixed connective tissue disease were not observed when prednisolone was tapered. During the 1-year treatment and follow-up period, the patient had no symptom recurrence. Conclusions: In pediatric primary SS patients, clinically silent heart changes are common, but obvious heart involvement is extremely rare. Unusual initial symptoms, such as symptomatic pericarditis, should not be missed in childhood-onset primary SS.

IS SJÖGREN'S SYNDROME ANOTHER POSSIBLE AUTOIMMUNE COMPLICATION OF CHIKUNGUNYA FEVER?

Chikungunya (CHIK) fever is a tropical arbovirosis characterized by sudden fever, headache, rash, and severe arthralgia. The disease has been suggested to act as a trigger for several autoimmune disorders, especially rheumatoid arthritis. This abstract reports 2 cases of Sjogren syndrome (SS) following CHIK infection. Both patients were female and presented with fever, arthralgia, rash, and arthritis during CHIK outbreak in Recife, Brazil. After several months, they developed severe sicca symptoms and 1 of them presented with parotid enlargement. Clinical salivary gland biopsy and serologic examination rendered the diagnosis of SS for both patients. No systemic features of SS were noted. New evidence has pointed to the development of autoimmune inflammatory musculoskeletal diseases following CHIK fever, such as rheumatoid arthritis, spondylitis, and systemic lupus as a sequel of the infection. Nevertheless, SS has rarely been associated with CHIK and this association should be better evaluated.

Pulmonary and Bronchiolar Involvement in Sjogren's Syndrome.

Sjogren's syndrome (SS) is a chronic autoimmune disease characterized by mononuclear cells (principally lymphocytes) infiltrating exocrine glands (e.g., salivary and lacrimal glands), leading to destruction of exocrine epithelial cells and dryness of mucosal surfaces. Cardinal symptoms are dry eyes (xerophthalmia) and dry mouth (xerostomia). Extraglandular sites are affected in 30 to 40% of cases of SS (particularly neurological, kidneys, skin, and lungs). B cell hyperactivity, autoantibody production, and hypergammaglobulinemia are cardinal features of SS. Primary SS is not associated with other autoimmune diseases. However, SS can complicate diverse autoimmune disorders (particularly systemic lupus erythematosus, rheumatoid arthritis, and scleroderma); this form is termed "secondary SS." Pulmonary involvement is usually not a dominant feature of SS, but may be severe in some cases. In this review, we discuss specific tracheal, bronchiolar, and pulmonary complications of SS including xerotrachea, bronchiolitis, bronchiectasis, interstitial lung disease, nonspecific interstitial pneumonia, usual interstitial pneumonia, lymphoid interstitial pneumonia, organizing pneumonia, acute fibrinous and organizing pneumonia, pulmonary cysts, pleural effusions, pulmonary amyloidosis, and bronchus- or lung-associated lymphomas.

The risk of Sjogren's syndrome in the older adults with gout: A medicare claims study.

In the absence of previous studies, our objective was to assess whether gout was associated with an increase or decrease in the risk of Sjogren's Syndrome (SS) in older adults, 65 years or older. We used the 5% Medicare claims from 2006-2012. A multivariable Cox regression model assessed the association of gout with incident SS adjusting for age, sex, race, Charlson-Romano comorbidity index, and the use of medications for cardiovascular diseases (statins, beta-blockers, diuretics, ACE-inhibitors) and gout (allopurinol, febuxostat). Hazard ratios (HR) and 95% confidence intervals (CI) were calculated. There were 3,186 new cases of SS in the study cohort with crude incidence rates of SS of 30/100,000 person-years in patients without gout and 49/100,000 person-years in patients with gout. Multivariable-adjusted analyses showed that gout was independently associated with a higher hazard ratio of SS of 1.73 (95% CI, 1.45, 2.06). Sensitivity analyses that substituted continuous Charlson-Romano comorbidity index score with categorized score (model 2) or individual comorbidities plus three common cardiovascular diseases (hypertension, hyperlipidemia, and coronary artery disease; model 3), confirmed the main study findings with minimal attenuation of hazard ratio, 1.70 (95% CI, 1.43, 2.02) and 1.48 (95% CI, 1.25, 1.77), respectively. Younger age, female sex, White race and higher comorbidity score were associated with a higher hazard of SS. Gout was associated with more than 1.7-fold higher risk of incident SS in adults 65 years or older. This finding needs to be reproduced and the underlying mechanisms for this association need further study.

Trismus with facial/neck swelling: An atypical presentation of Sjogren's syndrome

Background: Sjögren's syndrome (SS) is an autoimmune disorder affecting exocrine glands & commonly associated with other autoimmune conditions. We present an unusual case of SS and the diagnostic challenges associated with it.

Catatonia and Psychosis as Manifestations of Primary Sjögren's Syndrome.

Primary Sjögren’s syndrome (SS) is a chronic, systemic autoimmune disease, most commonly presenting with sicca symptoms—xerostomia and xerophthalmia. Up to one-half of affected individuals also develop extra-glandular involvement. Early diagnosis is important to prevent further complications. This paper describes an atypical case of SS in a 21-year-old woman who presented first with neuropsychiatric symptoms and fever. She was diagnosed and treated for a presumed psychotic episode. However, because she remained febrile, a thorough investigation resulted in the challenging diagnosis of SS.LEARNING POINTSPrimary Sjögren’s syndrome (SS) may be an under-recognized cause of central nervous system and neuropsychiatric involvement.Neuropsychiatric manifestations of primary SS can be variable.Patients may be treated incorrectly if the diagnosis is not rapidly established, so a clinical history and thorough examination are very important.

Lymphoepithelial Sialadenitis Involving HIV-Infected and Sjogren Syndrome Patients: A Cytologic Study.

Lymphoepithelial salivary gland cysts are rarely seen in autoimmune diseases particularly Sjogren syndrome as well as in HIV for which medical management is advocated. To study the morphology of these cysts, correlate with the disease process and assess the final outcome. Case series. Fine needle aspiration clinic. HIV-infected and autoimmune disease patients with lymphoepithelial cysts. Antiretroviral therapy for HIV-patients and anti-inflammatory drugs for Sjogren syndrome. Three HIV-infected patients (two children and one adult) and three middle aged female patients presented with parotid and submandibular cysts, two of which were bilateral along with submandibular (one each in the HIV and the autoimmune group). In the adult HIV-patient, the cyst was found at the inception of the disease while the other pediatric HIV-patients just crossed a decade. Of the other three cases of Sjogren syndrome, two were primary and one, secondary to rheumatoid arthritis. All the cysts regressed completely with treatment of the respective diseases which was confirmed by ultrasonograms. Lymphoepithelial cysts are produced by release of serous secretion by the acinar and ductal cells within the epithelial islands in the process of their destruction. Possibly, antibody mediated increased secretion in the initial stages also plays a role. Lymphoepithelial cysts of HIV patients may occur in the course of treatment, not necessarily in the beginning, though it resolves spontaneously. Lymphoepithelial cysts of primary or secondary Sjogren syndrome may be repressed sufficiently by anti-inflammatory/immunosuppressant treatment.

A Case of Idiopathic Retroperitoneal Fibrosis Associated With Sjögren's Syndrome.

After presenting with nonspecific symptoms, a 52-year-old female was found to have a 3.4 × 2.6 cm retroperitoneal mass resulting in hydronephrosis on abdominal computed tomography scan. After extensive workup including surgical debulking, excisional biopsy, and autoimmune panel, she was found to have idiopathic retroperitoneal fibrosis (IRF) with negative staining for immunoglogulin G4 (IgG4). Two years after presentation, the patient developed dyspnea, cough, and xerostomia. Repeat anti-Sjögren Syndrome A and anti-Sjögren Syndrome B titers and labial gland biopsy confirmed the diagnosis of concomitant Sjögren's syndrome (SS). The patient was treated with mycophenolate mofetil with improvement in her symptoms. Most cases of retroperitoneal fibrosis are associated with IgG4-related disease or other autoimmune disease. To our knowledge, this is only the second reported case of SS associated with IRF. Because symptoms of IRF are nonspecific, there is often a delay in diagnosis resulting in end-organ disease such as ureteral obstruction or hydronephrosis. Although IRF is uncommon, it should be considered in patients presenting with abdominal or flank pain, especially in patients with concomitant autoimmune disorders. Early recognition of disease can prevent end-organ damage and, as more cases are diagnosed, its relationship to SS may be elucidated leading to further advances in treatment and surveillance.

English

Sjogren syndrome is an autoimmune exocrinopathy with various extra glandular manifestations. Cryoglobulinemia, small vessel vasculitis has been reported in 5-10% of patients with primary Sjogren syndrome. It may be associated with a higher prevalence of extra glandular disease in Sjogren patients. Thrombotic microangiopathy (TMA), a potentially lethal condition characterized by microvascular thrombosis due to endothelial injury, is rare in SS. Its most characteristic histological finding is intravascular and intraglomerular fibrin thrombi. The association of Sjogren syndrome, cryoglobulinemia and thrombotic microangiopathy has not been previously reported. We report the first case of Sjogren syndrome with these three manifestations, occurring in combination.

Sjögren Syndrome Complicated by Mucosa-Associated Lymphoid Tissue Lymphoma and Lymphocytic Interstitial Pneumonia.

Sjögren syndrome (SS) is an autoimmune disease with exocrine glands dysfunction and multiorgan involvement. It is associated with increased risk of lymphoproliferative disorders, especially B-cell marginal zone lymphoma. While the role of F-18 Fluorodeoxyglucose position emission tomography/computed tomography (F-18 FDG PET/CT) for evaluation of lymphoma has been established, its use in patients with a chronic history of SS to evaluate for possible lymphoproliferative disorders or multiorgan involvement is limited. We present a case of chronic SS in which F-18 FDG PET/CT demonstrated FDG avid intraparotid and cervical lymph nodes pathologically proven to be mucosa-associated lymphoid tissue lymphoma. In addition, the patient had bibasilar cystic changes consistent with lymphocytic interstitial pneumonia.

Psoriasis and connective tissue diseases: What about Sjogren syndrome?

Psoriasis (Ps) is an autoimmune chronic skin disease with a high prevalence in the general population. Connective tissue diseases (CTD) are also autoimmune disorders of unknown cause with highly variable clinical expression. The combination of CTD and psoriasis is uncommon and has rarely been described. We report two cases of CTD associated with psoriasis: a case of systemic lupus erythematosus (SLE) and a case of Sjogren syndrome (SS)s.

Co-development of autoimmune hepatitis and Sjögren's syndrome triggered by the administration of herbal medicines

Autoimmune hepatitis (AIH) has been reported in association with Sjögren's syndrome (SS). Drug-induced AIH has been rarely reported. A rare case of the co-development of AIH and SS in a 53-year-old woman after the consumption of herbal medicines is described. After admission, the patient complained of dryness in her mouth, and she was subsequently diagnosed with SS, which had not been detected previously. The patient's bilirubin and aminotransferase levels initially decreased following conservative management; however, they later began to progressively increase. A diagnosis of AIH was made based on the scoring system proposed by the International Autoimmune Hepatitis Group. The patient was administered a combination of prednisolone and azathioprine, and the results of follow-up liver-function tests were found to be within the normal range. This is an unusual case of AIH and SS triggered simultaneously by the administration of herbal medicines.

Sj�gren's Syndrome Complicated with Thrombotic Thrombocytopenic Purpura: A Case Report and Literature Review

The association of Sjogren's syndrome (SS) with thrombotic thrombocytopenic purpura (TTP) is rare, but it carries a high death rate and can be easily misdiagnosed. We report a case of TTP with SS and the related literature is reviewed. A previously healthy 72-year-old woman presented with an acute onset of marked thrombocytopenia and then dizziness, fatigue, fever and fainting. Schirmer's test was bilaterally positive, associated with positive Rose Bengal corneal staining. Radionuclide imaging of the glands showed abnormalities. The presence of anti-SSA and anti-SSB antibodies confirmed the diagnosis of SS. The patient was treated successfully with plasma exchange therapy, plasma infusion, glucocorticoid, and immunosuppressive agents. Her condition has remained stable after 6 months of follow-up. Only a few cases of SS complicated with TTP have been described in the literature. Rheumatologists should pay attention to this special condition because it is rare and has a high mortality rate. Keywords Sjogren's syndrome, Thrombotic thrombocytopenic purpura, Autoimmune

Detection of minor salivary glands affected in Sjogren’s syndrome by Raman spectroscopy

Sjogren's syndrome(SS) is defined as a slowly processing autoimmune inflammatory exocrinopathy affecting the salivary and lachrymal glands. Due to the difficulty in reaching a diagnosis in some SS cases, there are many criteria published for classification and diagnosis. Among the most of criteria, lip biopsy plays an important role in clinical diagnosis, but it is an invasive and complex method which always causes patients' pain and discomfort. Raman spectroscopy is a noninvasive and real-time vibrational spectroscopic technique applied in the clinical detection and diagnosis of diseases. The diagnostic sensitivity and specificity are both high. The hypothesis is to detect the minor salivary glands affected in SS by the noninvasive Raman spectroscopy instead of lip biopsy. Therefore, Raman spectroscopy may increase the diagnostic sensitivity and specificity, meanwhile prevent patients from pain caused by biopsy.

Hodgkin lymphoma as a complication of primary Sjögren’s syndrome

Sjögren's syndrome (SS) is a chronic autoimmune disease that is characterized by lymphocytic infiltration of the exocrine glands, mainly the salivary and lachrymal glands, usually manifesting with xerostomia and xerophthalmia. Around 50% of patients with primary SS develop systemic complications, lymphoma being the most feared of these. The majority of these neoplasias originate from B cells and are of the non-Hodgkin type. We describe here a rare case of SS in which the patient developed a Hodgkin lymphoma. We also review the literature on this subject.

Patterns of anti-nuclear antibodies in patients with primary biliary cirrhosis and primary Sjögren syndrome

To investigate the anti-nuclear antibody (ANA) patterns in the diagnosis of primary biliary cirrhosis (PBC) and primary Sjögren syndrome (SS). Serum samples from 61 cases of PBC, 28 cases of primary SS, and 11 cases of overlap syndrome of PBC and SS were collected to detect the ANA with indirect immunofluorescence staining, and the difference in ANA patterns was analyzed between the PBC and SS patients. Antinuclear antibodies were detected in 85.2% of the PBC patients, with the following hierarchy of specificities: 37.7% being rim-like, 21.3% being discrete speckled, 18.0% being speckled, 8.2% being multiple nuclear dots, and 4.9% being anti-lamin; 89.3% of the primary SS patients was ANA positive with speckled antibody, only one of them presented discrete speckles (3.6%); and all of the PBC patients overlapped with SS (100%) were positive in ANA, and their contexture of ANAs was similar to those of the PBC patients: 45.5% being rim-like, 18.2% being discrete speckled, 18.2% being speckled, 18.2% being anti-lamin, and 9.1% being multiple nuclear dots. Rim-like pattern and multiple nuclear dots were statistically significant for the diagnosis of PBC, compared with primary SS (chi(2) = 14.236, 3.781, P < 0.01, < 0.05). The ANA patterns of PBC are different from those of primary SS. Among them, rim-like pattern and multiple nuclear dots are highly specific nuclear patterns of PBC and may be useful in diagnosing individuals without anti-mitochondrial antibodies.

Juvenile dermatomyositis with Sjögren’s syndrome

Juvenile dermatomyositis (JDM) is a rare disease, and Sjögren's syndrome (SS) is unusual in adolescents. We report the first case of biopsy-proven JDM and SS with pulmonary involvement. A 15-year-old adolescent boy presented with recurrent parotid gland hypertrophy, severe muscle weakness, pronounced skin rash and widespread lymphadenopathy. JDM was diagnosed by clinical examination, elevated muscle enzymes, electromyography and muscle biopsy; SS was diagnosed by xerostomia, anti-Ro (SS-A) positivity and histopathological analysis of salivary gland tissue. This case illustrates a systematic approach which we feel is especially important in the younger patient with a more plastic immune system.