Functional significance of DNA methylation: epigenetic insights into Sjögren's syndrome.

Sjögren's syndrome (SjS) is a systemic, highly diverse, and chronic autoimmune disease with a significant global prevalence. It is a complex condition that requires careful management and monitoring. Recent research indicates that epigenetic mechanisms contribute to the pathophysiology of SjS by modulating gene expression and genome stability. DNA methylation, a form of epigenetic modification, is the fundamental mechanism that modifies the expression of various genes by modifying the transcriptional availability of regulatory regions within the genome. In general, adding a methyl group to DNA is linked with the inhibition of genes because it changes the chromatin structure. DNA methylation changes the fate of multiple immune cells, such as it leads to the transition of naïve lymphocytes to effector lymphocytes. A lack of central epigenetic enzymes frequently results in abnormal immune activation. Alterations in epigenetic modifications within immune cells or salivary gland epithelial cells are frequently detected during the pathogenesis of SjS, representing a robust association with autoimmune responses. The analysis of genome methylation is a beneficial tool for establishing connections between epigenetic changes within different cell types and their association with SjS. In various studies related to SjS, most differentially methylated regions are in the human leukocyte antigen (HLA) locus. Notably, the demethylation of various sites in the genome is often observed in SjS patients. The most strongly linked differentially methylated regions in SjS patients are found within genes regulated by type I interferon. This demethylation process is partly related to B-cell infiltration and disease progression. In addition, DNA demethylation of the runt-related transcription factor (RUNX1) gene, lymphotoxin-α (LTA), and myxovirus resistance protein A (MxA) is associated with SjS. It may assist the early diagnosis of SjS by serving as a potential biomarker. Therefore, this review offers a detailed insight into the function of DNA methylation in SjS and helps researchers to identify potential biomarkers in diagnosis, prognosis, and therapeutic targets.

Sjögren's Syndrome and Devic's Disease: A Synchronised Saga.

Devic's disease, commonly known as neuromyelitis optica (NMO), is a rare relapsing autoimmune illness of the central nervous system. Occasionally, it is associated with other autoimmune diseases such as Sjögren's syndrome (SS). Dry mouth and dry eyes are two symptoms of SS, a chronic autoimmune condition marked by inflammation and dysfunction of the exocrine gland. While SS primarily affects the exocrine glands, it can also manifest with a range of extraglandular features, including neurological manifestations, as in our case where the patient initially presented with neurological symptoms and was diagnosed with NMO. Owing to the persistent relapses along with sicca symptoms that occurred late, SS was diagnosed on further evaluation. Although the association of SS with NMO is not very common, the initial presentation of SS with neurological symptoms, as in our case, is what makes it more unique.

Pervasive Sharing of Causal Genetic Risk Factors Contributes to Clinical and Molecular Overlap between Sjögren's Disease and Systemic Lupus Erythematosus.

SjD (Sjögren's Disease) and SLE (Systemic Lupus Erythematosus) are similar diseases. There is extensive overlap between the two in terms of both clinical features and pathobiologic mechanisms. Shared genetic risk is a potential explanation of this overlap. In this study, we evaluated whether these diseases share causal genetic risk factors. We compared the causal genetic risk for SLE and SjD using three complementary approaches. First, we examined the published GWAS results for these two diseases by analyzing the predicted causal gene protein-protein interaction networks of both diseases. Since this method does not account for overlapping risk intervals, we examined whether such intervals also overlap. Third, we used two-sample Mendelian randomization (two sample MR) using GWAS summary statistics to determine whether risk variants for SLE are causal for SjD and vice versa. We found that both the putative causal genes and the genomic risk intervals for SLE and SjD overlap 28- and 130-times more than expected by chance (p < 1.1 × 10-24 and p < 1.1 × 10-41, respectively). Further, two sample MR analysis confirmed that alone or in aggregate, SLE is likely causal for SjD and vice versa. [SjD variants predicting SLE: OR = 2.56; 95% CI (1.98-3.30); p < 1.4 × 10-13, inverse-variance weighted; SLE variants predicting SjD: OR = 1.36; 95% CI (1.26-1.47); p < 1.6 × 10-11, inverse-variance weighted]. Notably, some variants have disparate impact in terms of effect size across disease states. Overlapping causal genetic risk factors were found for both diseases using complementary approaches. These observations support the hypothesis that shared genetic factors drive the clinical and pathobiologic overlap between these diseases. Our study has implications for both differential diagnosis and future genetic studies of these two conditions.

The Diagnosis, Treatment, and Clinical Sequelae of Sjogren's Syndrome in a Pediatric Patient: A Case Report.

Sjogren's Syndrome is a chronic multisystem autoimmune condition where lymphocytes attack exocrine glands. Although this condition occurs in pediatric populations, it is often a missed diagnosis or diagnosis made after significant disease progression, frequently leading to extensive investment of time and resources. This case study follows a six-year-old African American female who, after an extensive medical course, was ultimately diagnosed with Sjogren's Syndrome. This case study intends to increase awareness of the potential abnormal presentations of this connective tissue disease in special populations, specifically school-aged pediatric patients. Even with the rarity of this condition in the pediatric population, physicians should keep Sjogren's Syndrome on their differential diagnosis when a patient presents with atypical or non-specific autoimmune-like symptoms. The presentation of children can be more severe than anticipated in an adult. A rapid, multi-disciplinary approach must be implemented to improve the prognosis of pediatric patients with Sjogren's Syndrome.

Time interval between the onset of connective tissue disease symptoms and first contact with a rheumatologist: results from the German National Database of collaborative arthritis centers.

The long-term outcome of connective tissue diseases is associated with the time from symptom onset to diagnosis. To understand gaps in care, we determine whether the length of time between symptom onset and first presentation to a rheumatologist has changed in Germany in recent decades. We analyzed data on patients diagnosed with connective tissue diseases (n = 19,662) collected from the German National Database of the Regional Cooperative Rheumatology Centers. We reviewed the onset of relevant symptoms listed at first presentations from 1993 to 2018 and performed a quantitative analysis of the intervals until first presentation to a rheumatologist. We compared time intervals and performed a linear mixed regression model with random effects to identify associated factors. Although the interval between the onset of symptoms and first presentation to a rheumatologist has diminished since 1980 for all connective tissue diseases, there has been no relevant improvement during the past 2 decades. The interval between symptoms and presentation increases with patients age for all connective tissue diseases (e.g., Systemic sclerosis; for each 10-year-increment of patients age: ß 0.41, CI 0.38; 0.44). Among those diagnosed with systemic sclerosis, the mean interval was 1.5 years (95% CI 1.1; 1.8) for male patients and 2.6 years (95% CI 2.4; 2.8) for females. Patients presenting with different degrees of disease severity on their first visits and with different educational levels had similar mean intervals between symptoms and first presentation regardless of their final diagnoses. Over the past 2 decades, the time to first consultation with a rheumatologist has not continued to improve in Germany, but has stagnated at the same level. Selected patient subgroups, such as older patients with suspected connective tissue diseases and female patients with suspected systemic sclerosis, are at risk to present late and may in particular benefit from an earlier referral to a rheumatologist.

A Rare Case of Breast Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma With Amyloid Deposition in a Patient With Sjogren's Syndrome.

Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) of the breast with amyloid deposits is a very rare cause of breast malignancy. Patients who carry a diagnosis of Sjogren's syndrome (SS) have a 5-10% lifetime risk of developing non-Hodgkin lymphoma with MALT lymphoma as the most common histologic subtype. Our case highlights the importance of routine screening mammography in the early detection of such unusual malignancies, and further interventions needed to diagnose and appropriately manage breast MALT lymphoma.

Case report: disease mechanisms and medical management of calcium nephrolithiasis in rheumatologic diseases.

BACKGROUND: Nephrolithiasis as a feature of rheumatologic diseases is under recognized. Understanding presenting features, diagnostic testing is crucial to proper management. CASE PRESENTATION: A 32 year old woman with a history of recurrent complicated nephrolithiasis presented to a rheumatologist for a several month history of fatigue, dry eyes, dry mouth, arthralgias. She had a positive double-stranded DNA, positive SSA and SSB antibodies. She was diagnosed with Systemic Lupus erythematosus (SLE) and Sjogren's syndrome and was started on mycophenalate mofetil. Of relevance was a visit to her local emergency room 4 years earlier with profound weakness with unexplained marked hypokalemia and a non-anion gap metabolic acidosis. Approximately one year after that episode she developed flank pain and nephrocalcinosis. She had multiple issues over the ensuing years with stones and infections on both sides. Interventions included extracorporeal shockwave lithotripsy as well as open lithotomy and eventual auto-transplantation of left kidney for recurrent ureteric stenosis. 24 h stone profile revealed marked hypocitraturia, normal urine calcium, normal urine oxalate and uric acid. She was treated with potassium citrate. Mycophenolate was eventually stopped due to recurrent urinary tract infections and she was started on Belimumab. Because of recurrent SLE flares, treatment was changed to Rituximab (every 6 months) with clinical and serologic improvement. Her kidney stone frequency gradually improved and no further interventions needed although she continued to require citrate repletion for hypocitraturia. CONCLUSIONS: Nephrolithiasis can be a prominent and even presenting feature in Sjogrens syndrome as well as other rheumatologic diseases. Prompt recognition and understanding disease mechanisms is important for best therapeutic interventions for kidney stone prevention as well as treatment of underlying bone mineral disease.

Immunoglobulin G4-Related Disease: A Case Report.

Immunoglobulin G4-related disease (IgG4 RD) has a fair prognosis but its diagnosis has been difficult due to the condition's wide range of clinical manifestations, limited awareness among common practitioners, and various differentials. Here, we present a case of an elderly male who presented with recurrent dental caries, recurrent sinusitis, persistent dry mouth, and dry eyes along with bilateral parotid gland enlargement without any lymphadenopathy. The patient was evaluated further and found to have elevated levels of IgG4 and on histopathological examination of the parotid gland showed lymphocytic infiltrate with germinal centers without any granulomatous lesions and IgG4-positive plasma cells on immunohistochemistry (IHC). The patient was diagnosed with IgG4 RD and was started on corticosteroids, after which there was a symptomatic improvement.

Primary Sjögren's Syndrome Presenting as Cerebral Venous Thrombosis: A Rare Case.

Sjogren's syndrome is a late-onset, slowly progressing autoimmune disease characterized by the destruction of the exocrine glands by lymphocytic infiltration, resulting in dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca). Sjögren's syndrome may be associated with various autoimmune diseases, including systemic lupus erythematosus, rheumatoid arthritis, and systemic sclerosis. We report a case of a 34-year-old female who delivered a live baby 20 days ago. She presented in a postictal state after two episodes of tonic-clonic movements of limbs with altered sensorium with a history of headache for seven days. Further evaluation revealed that the subject had a history of multiple abortions and grittiness in her eyes. MRI showed signs of infarction in the left parietal lobe and magnetic resonance venography (MRV) suggested cavernous venous thrombosis. After an unwavering effort to rule out alternate causes, the rare correlation between primary Sjogren's syndrome and cerebral venous thrombosis was considered. Additional investigations were performed, which showed the patient to be positive for Anti SS-A (Ro52), Anti SS-B (La), and anti-centromere antibodies. The patient gradually improved with anti-edema measures and steroids and was discharged by day nine. We present this case to emphasize the neurological manifestation of Sjogren's syndrome, which may present as cerebral venous thrombosis.

Adverse post-operative events of salivary gland biopsies: A systematic review and meta-analysis.

OBJECTIVES: The study aimed to perform a systematic review and meta-analysis of the complications following major and minor salivary gland biopsy. MATERIALS AND METHODS: Observational studies assessing postoperative complications of minor salivary gland biopsy and indexed at Medline/PubMed, EMBASE, Cinahl, LILACS, or Scopus were selected. This review was registered under the protocol number: CRD42020211169. The level of significance considered was 0.05, and the R software (The R Foundation) was used for the meta-analysis. RESULTS: Twenty-seven studies reporting 3208 patients were included in this review. The combined prevalence of postsurgical complications was 11% (95% CI, 8 to 13%, p = 0.01). The percentage of the combined prevalence of neurological complications was 3% (95% CI, 1-6%, p = 0.01). The surgical technique did not influence the frequency of overall and neurological complications. CONCLUSION: Minor salivary gland biopsies are a safe and predictable procedure that should be performed on the lower lip. Postoperative complications are more common than previously reported, but permanent complaints are uncommon.

Fibromyalgia, Sjogren's & depression: linked?

Health care has become increasingly fragmented, partly due to advancing medical technology. Patients are often managed by various specialty teams when presenting with symptoms that could be manifestations of different diseases. Approximately one third of them are referred to specialists, at over half for outpatient appointments. Fatigue, pain, depression, dry mouth, headaches, and arthralgia are common complaints and frequently require referral to specialist physicians. Differential diagnoses include fibromyalgia (FM), Sjogren's syndrome (SS), and depression. Evaluations involve various sub-specialist especially physicians like those practicing pain management, rheumatology, and psychiatry. Thresholds for referring vary. Patients sometime feel lost in a 'medical maze'. Disagreement is frequent between specialties regarding management. Each discipline has its own diagnostic and treatment protocols and there is little consensus about shared decision-making. Communication between doctors could improve continuity. There are many differences and similarities in the pathophysiology, symptomatology, diagnosis, and treatment of fibromyalgia, Sjogren's syndrome, and depression. Understanding the associations between fibromyalgia, Sjogren's syndrome and depression should improve clinical outcome via a common holistic approach.

Refractory Sjogrens syndrome: Is parotidectomy justified?

Primary and secondary Sjogrens syndrome (SS) is the classification used, according to the American-European Consensus Group Criteria. Salivary and lacrimal gland dysfunctions are the usual hallmark of the disease, but the involvement of other exocrine glands and extraglandular manifestations of the disease do occur. In rare cases, few patients are refractory to the conventional therapy and due to the sudden increase in size of a mass and the esthetic and psychological concerns of a "cancerous growth," the surgical treatment modalities have to be modified. There is a significant lack of contemporary literature on the indications for surgery in refractory SS, and the option should be given in patients with esthetic concerns, risk of malignancy, and to improve the overall quality of life.

Assessing health-related quality of life in primary Sjögren's syndrome-The PSS-QoL.

OBJECTIVES: To develop a questionnaire for the assessment of health-related quality of life (HRQL) in primary Sjögren's syndrome (PSS), and to test its psychometric properties. METHODS: Based on the concepts of a previous qualitative study, a questionnaire for the assessment of HRQL in PSS (PSS-QoL) was developed. Psychometric testing of PSS-QoL was performed after revising the first draft with feedback of patients (n = 6) and clinicians (n = 4). Convergent construct validity was assessed by correlating the score with the EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI), EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) and Euro-QoL 5D (EQ-5D). Reliability was examined by asking patients to complete the questionnaire twice 1-2 weeks apart. An English Version of the PSS-QoL was developed by using standard methodology with forward and back translation. RESULTS: Out of the 75 PSS patients, 91% were female, mean (±SD) age was 58.5 ± 12.5 years. PSS-QoL consists of 25 questions and can be divided into two main categories: physical (discomfort and dryness) and psychosocial. The internal consistency of the PSS-QoL revealed a Crohnbach's α of 0.892. Strong and moderate correlations were found between the PSS-QoL and ESSPRI (corrcoeff = 0.755) and EQ. 5D-pain/discomfort (corrcoeff = 0.531). Reproducibility of the PSS-QoL was high, yielding an ICC of 0.958 (95% CI: 0.926-0.981). CONCLUSIONS: The PSS-QoL is the first specific tool for the assessment of patients' HRQL in PSS and showed good psychometric properties. It may serve as a novel patient-reported outcome measure in future clinical studies.

Prevalencia y significado clínico de los anticuerpos anti-SS-A(RO) en pacientes con enfermedades reumáticas autoinmunes sistémicas / Prevalence and clinical significance of anti-SS-A(RO) antibodies in patients with systemic autoimmune rheumatic diseases

Por la importancia diagnóstica que tiene la detección de los distintas especificidades de anticuerpos que permite distinguir síndromes reumáticos que se sobreponen en el plano clínico, se exploró su frecuencia en un grupo de 4 693 pacientes con enfermedades reumáticas autoinmunes sistémicas (SARDs) en el periodo entre el 10 de junio del 2010 al 10 de junio del 2016. Fueron estudiados con ANA screen, ANA combi e IMMUNOBLOTTING. Solo fueron positivos 277 (5,9 %), 250 del sexo femenino y 27 del sexo masculino. Existió una importante prevalencia de reactividad contra los anticuerpos anti-SS-A con 140 pacientes (50 %), seguido de los antinucleosoma con 97 (35 %) y los DNA ds con 72 (25 %), en el resto de los anticuerpos no existieron hallazgos importantes. Este estudio sugiere que para los pacientes con manifestaciones clínicas de enfermedades reumáticas autoinmunes sistémicas es necesario y útil la utilización de estas pruebas que, junto con la información clínica y en algunos casos histológica, puede ayudar a realizar un diagnóstico más preciso.

Due to the diagnostic importance of the detection of different antibody specificities that allows us to distinguish rheumatic syndromes that clinically overlap; we studied its prevalence in a group of 4 693 patients with systemic autoimmune rheumatic diseases In the period between June 10, 2010 and June 10, 2016. For that purpose we used ANA Screen and ANA Combi. 277 (5 %) were female and 250 male. There was a significant prevalence of anti-SS-A antibodies140 (50 %) followed by antinucleosome 97 (35 %) and DNAs of 72 (25 %), no significant results were obtained with the rest of the other antibodies. Our results suggest the usefulness of these tests in patients with clinical manifestations of systemic autoimmune rheumatic diseases together with the clinical and histological information that could help to make an accurate diagnosis.

[Coexistence of sarcoidosis and primary Sjögren syndrome: a clinical analysis and literature review].

Four patients with coexistence of sarcoidosis and primary Sjögren syndrome (pSS) were retrospectively analyzed.All patients were female, who were referred to our department mainly because of respiratory symptoms.Positive antinuclear antibody(ANA) was detected in 2 patients and anti-Sjögrens syndrome A (SSA) antibody positive in 1 patient.All patients presented specific histologic patterns of both sarcoidosis and pSS.Publications related to coexistence of these two diseases were reviewed.Forty-one patients were finally included in the analysis, among whom 37 confirmed patients were from literature search.There were 37 women and 4 men.The main clinical features presentation were xerophthalmia in 40, xerostomia in 38, hilaradenopathies in 28, interstitial lung disease in 15, respiratory symptoms in 13.The main immunologic data were positive ANA in 23, SSA antibody in 19, anti-Sjögrens syndrome B antibody in 10 and rheumatoid factor in 12.All patients presented specific histologic patterns of both diseases.Patients with both sarcoidosis and pSS of ten represent multisystemic involvement and positive immunologic parameters, as well as the dual expression of specific histologic characteristics.

Effects of periodontal treatment on primary sjogren's syndrome symptoms

Abstract The aim of this longitudinal prospective study was to evaluate the effects of periodontal treatment on the clinical, microbiological and immunological periodontal parameters, and on the systemic activity (ESSDAI) and subjective (ESSPRI) indexes in patients with primary Sjögren’s Syndrome (pSS). Twenty-eight female patients were divided into four groups: pSS patients with or without chronic periodontitis (SCP, SC, respectively), and systemically healthy patients with or without chronic periodontitis (CP, C, respectively). Periodontal clinical examination and immunological and microbiological sample collection were performed at baseline, 30 and 90 days after nonsurgical periodontal treatment (NSPT). Levels of interleukin IL-1β, IL-8 and IL-10 in saliva and gingival crevicular fluid (GCF) were evaluated by ELISA, as well as the expression of Porphyromonas gingivalis (Pg), Aggregatibacter actinomycetemcomitans, (Aa) Tannerella forsythia (Tf), and Treponema denticola (Td), by qPCR. Systemic activity and pSS symptoms were evaluated by ESSDAI and ESSPRI. NSPT resulted in improved periodontal clinical parameters in both SCP and CP groups (p>0.05). Pg, Aa, and Tf levels decreased after NSPT only in CP patients (p<0.05). Significantly greater levels of IL-10 in GCF were verified in both SCP and CP groups (p<0.05). SCP patients showed increased salivary flow rates and decreased ESSPRI scores after NSPT. In conclusion, NSPT in pSS patients resulted in improved clinical and immunological parameters, with no significant effects on microbiological status. pSS patients also showed increased salivary flow and lower ESSPRI scores after therapy. Therefore, it can be suggested that NSPT may improve the quality of life of pSS patients.

Alteration in cellular turnover and progenitor cell population in lacrimal glands from thrombospondin 1-/- mice, a model of dry eye.

The purpose of this study was to investigate the changes that occur in the lacrimal glands (LGs) in female thrombospondin 1 knockout (TSP1-/-) mice, a mouse model of the autoimmune disease Sjogren's syndrome. The LGs of 4, 12, and 24 week-old female TSP1-/- and C57BL/6J (wild type, WT) mice were used. qPCR was performed to measure cytokine expression. To study the architecture, LG sections were stained with hematoxylin and eosin. Cell proliferation was measured using bromo-deoxyuridine and immunohistochemistry. Amount of CD47 and stem cell markers was analyzed by western blot analysis and location by immunofluorescence microscopy. Expression of stem cell transcription factors was performed using Mouse Stem Cell Transcription Factors RT2 Profiler PCR Array. Cytokine levels significantly increased in LGs of 24 week-old TSP1-/- mice while morphological changes were detected at 12 weeks. Proliferation was decreased in 12 week-old TSP1-/- mice. Three transcription factors were overexpressed and eleven underexpressed in TSP1-/- compared to WT LGs. The amount of CD47, Musashi1, and Sox2 was decreased while the amount of ABCG2 was increased in 12 week-old TSP1-/- mice. We conclude that TSP1 is necessary for maintaining normal LG homeostasis. Absence of TSP1 alters cytokine levels and stem cell transcription factors, LG cellular architecture, decreases cell proliferation, and alters amount of stem cell markers.

Hepatitis Delta Virus Detected in Salivary Glands of Sjögren's Syndrome Patients and Recapitulates a Sjögren's Syndrome-Like Phenotype in Vivo.

BACKGROUND: Low-level, chronic viral infections have been suspect in the development of select autoimmune diseases, including primary Sjögren's syndrome (pSS). Multiple studies have shown stimulation of antiviral response pathways in pSS tissues suggestive of a viral infection. Yet, with this data in hand, a causal link between a viral infection and development of pSS had not been identified. Therefore, a study was designed to further define the viral landscape within pSS-affected salivary gland tissue to identify potential viral-mediated triggers in the pathogenesis of this autoimmune disease. METHODS: A viral microarray was utilized to measure viral transcripts present in salivary gland tissue from patients diagnosed with pSS compared to healthy controls. Murine models of salivary gland localized HDV antigen expression were developed to evaluate the capacity of a chronic HDV signature to trigger the development of a pSS-like phenotype. RESULTS: Through this analysis, two distinct viral profiles were identified, including the increased presence of hepatitis delta virus (HDV) in 50% of pSS patients evaluated. Presence of HDV antigen and sequence were confirmed in minor salivary gland tissue. Patients with elevated HDV levels in salivary gland tissue were negative for detectible hepatitis B virus (HBV) surface antigen and antibodies to HBV or HDV. Expression of HDV antigens in vivo resulted in reduced stimulated saliva flow, increase in focal lymphocytic infiltrates, and development of autoantibodies. CONCLUSION: Identification of HDV in pSS patients and induction of a complete pSS-like phenotype in vivo provides further support of a viral-mediated etiopathology in the development of pSS.

Manifestaciones bucales del síndrome de Sjögren. Presentación de un caso / Oral manifestations of the Sjögrens syndrome. Presentation of a case

El síndrome de Sjögren es una enfermedad autoinmunitaria, caracterizada por la infiltración linfoplasmocitaria de las glándulas exocrinas con destrucción epitelial, provocando un síndrome seco. El objetivo de esta presentación de caso fue profundizar en el conocimiento de la enfermedad, ya que constituye la clave para su diagnóstico oportuno. Se presentó a una paciente femenina de 54 años, con antecedentes de lupus eritematoso sistémico. El interrogatorio, el examen clínico general y bucal, los estudios de laboratorio e histológico, así como la sospecha clínica de la enfermedad, permitieron realizar el diagnóstico del síndrome de Sjögren asociado a lupus eritematoso sistémico. En esta paciente concurrieron las siguientes manifestaciones bucales: xerostomía, halitosis, sensación de sed constante, dificultades para la deglución y para hablar, labios secos y pálidos, lengua depapilada, ardor bucal, intolerancia al uso de la prótesis, queilitis angular y candidiasis bucal. El manejo de la enfermedad tiene un enfoque multidisciplinario; el estomatólogo cumple un papel esencial en su diagnóstico y tratamiento, lo cual permite mejorar la calidad de vida de estos pacientes. (AU)

The Sjögrens syndrome is an auto-immune disease, characterized by the limpho-plasmocytic infiltration of the exocrine glands with epithelial destruction, provoking a dry syndrome. The objective of this case presentation was deepening in this disease knowledge, because it is the key for its opportune diagnosis. We presented a female patient, aged 54 years, with antecedents of systemic lupus erythematosus. The anamnesis, oral and general examination, histological and laboratory studies, and also the clinical suspicion, allowed arriving to the diagnosis of Sjögrens syndrome associated to systemic lupus erythematosus. This patient showed the following oral manifestations: xerostomia, halitosis, sensation of constant thirst, difficulties for deglutition and speaking, dry and pale lips, depapillated tongue, oral burning, intolerance to prosthesis usage, angular cheilitis and oral candidiasis. The disease management has a multidiscipline approach; the dentist plays an essential role in the diseases diagnosis and treatment, allowing improving the life quality of these patients. (AU)

Case-based discussion: Lymphocytic interstitial pneumonia a rare presentation in an immunocompetent adult male.

Lymphocytic interstitial pneumonia (LIP) is a rare form of interstitial lung disease usually associated with other systemic diseases; however, idiopathic cases are being reported. As per recent ATS/ERS 2013 guidelines, diagnostic criteria of clinical, radiological and histopathological for LIP is same as 2002 except some cystic changes on HRCT chest. Many cases diagnosed in the past as LIP now turn out to be NSIP; therefore as per new ATS/ERS classification whenever anybody report a case of LIP, NSIP should always be kept in mind as differential diagnosis. Here we present a case of LIP in an immunocompetent adult male presented with history of persistent dry cough and breathlessness on exertion, confirmed on HRCT chest and histopathologically, treated successfully with steroids.