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ID PMID Title PublicationDate abstract
31752018 Comparison of MicroRNA Expression in Tears of Normal Subjects and Sjögren Syndrome Patien 2019 Nov 1 PURPOSE: Deregulated expression of several microRNAs (miRNAs) in sera or salivary glands of patients with Sjögren syndrome (SS) has been reported. However, none have investigated miRNAs in samples that can represent lacrimal glands. We compared the miRNAs expression in the tears of SS patients and healthy controls. Moreover, we investigated the correlation between miRNAs expression and ocular staining score (OSS). METHODS: Individual tear samples were collected from 18 SS patients and 8 age-matched controls. Clinical ophthalmologic assessments included Schirmer I test, tear film breakup time (tBUT), and OSS. The expression of 43 different miRNAs in tears was measured using real-time polymerase chain reaction, and compared between the SS patients and controls. And we also compared between the three groups of control, primary SS, and secondary SS patients. The correlation between the miRNA expression and OSS was evaluated. RESULTS: The expression levels of miR-16-5p, miR-34a-5p, miR-142-3p, and miR-223-3p were significantly upregulated in patients with SS when compared with those in the control group (P < 0.05). The expression of 10 miRNAs (miR-30b-5p, miR-30c-5p, miR-30d-5p, miR-92a-3p, miR-134-5p, miR-137, miR-302d-5p, miR-365b-3p, miR-374c-5p, miR-487b-3p) was significantly downregulated in the SS patients (P < 0.05). Eight miRNAs showed statistically significant differences between the three groups of control, primary SS and secondary SS. All 14 miRNAs with significant differences in SS patients and control group were not significantly correlated with OSSs. CONCLUSIONS: The 14 differentially expressed miRNAs may be involved in the pathogenesis of SS, in particular, related to autoimmunity and neuropathy.
31205955 Tissue-Specific Autoantibodies Improve Diagnosis of Primary Sjögren's Syndrome in the Ear 2019 Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands. Due to the absence of specific clinical manifestations and biomarkers in the early stage, pSS is generally underrecognized. To elucidate the role of the tissue-specific autoantibodies (TSAs), i.e., anti-CA6, anti-SP1, and anti-PSP antibodies, we enrolled 137 pSS patients, 32 secondary Sjögren's syndrome (sSS) patients, and 127 healthy controls (HCs), whose serum and saliva samples were collected. TSA levels were detected by ELISA, and the clinical and laboratory data was reviewed from the medical records. The analysis results showed the following: (1) Compared to HCs, the serum IgA levels of anti-CA6, anti-SP1 and anti-PSP were significantly higher in pSS as well as in sSS patients, and anti-CA6 IgG was also notably higher in pSS patients. (2) The positivity of anti-CA6, anti-PSP and all the three antibodies together were significantly increased in anti-SSA-negative pSS patients. (3) The average IgM levels of anti-CA6 and anti-SP1 decreased as the disease duration extended. (4) The anti-CA6-positive patients have significantly higher levels of serum IgA, while the anti-PSP-positive group has a notably higher serum IgM level. (5) Another autoantibody specific to the salivary glands, anti-α-fodrin antibody, was elevated in TSA-positive patients, especially in the anti-CA6-positive group. (6) Preliminary detection of saliva TSAs showed that all the IgG levels of these three antibodies increased significantly in pSS patients. In conclusion, TSAs improve diagnosis of pSS in the early stage, especially in anti-SSA-negative patients, and their tissue-specific nature indicates localized salivary injury, which deserves further studies to clarify the mechanism.
31137057 Computed Tomographic Imaging in Connective Tissue Diseases. 2019 Apr Pulmonary disease represents a significant extra-articular manifestation in the majority of connective tissue diseases (CTDs). The identification, classification, and staging of pulmonary involvement are centrally important to the management of patients, aiding the prognostication of disease behavior and treatment decisions. We present a review of the high-resolution computed tomographic pulmonary features of CTD in the lung and their significance to the reporting radiologist.
31079987 Intense pulsed light therapy: A promising complementary treatment for dry eye disease. 2019 Jul OBJECTIVE: To propose the Intense Pulsed Light (IPL) therapy as a helpful supplementary treatment in patients with dry eye disease. MATERIAL AND METHODS: Retrospective cross sectional design. Medical records of patients in whom dry eye disease symptoms were not satisfactorily controlled with medical therapy alone and who underwent additional IPL with at least three sessions completed. Data were analyzed before therapy and 3weeks after its completion to asses improvement. Determination of symptoms, through a visual analog scale; tear film stability, through tear Break Up Time (tBUT); measurement of tear secretion, through Schirmer Test; and ocular surface staining with Van Bijsterveld score were evaluated. SPSS software and nonparametric analysis of repeated measures were used. The study was approved by the ethics committee. RESULTS: 50 eyes from 25 subjects were reviewed. There were 9 males (36%) and 16 females (64%), with a median age of 59years (IQR 52-64). The median of the symptoms scale was 8 (IQR 8-9) and 3 (IQR 2-4) before and after the therapy respectively (P<.05). The median of BUT was 4 (IQR 3-5) and 10 (IQR 8-11), Schirmer test was 13 (IQR 12-15) and 15 (IQR 13-20), and Van Bijsterveld score was 3 (RIC 3-4) and 2 (IQR 2-3) before and after the therapy respectively (P<.05, for all measurements). CONCLUSION: IPL treatment has excellent results regarding both: dry eye disease symptoms improvement and in office objective tests such as tBUT, Schirmer test and Van Bijsterveld score; IPL could be considered as an effective adjunct for dry eye disease.
31095438 Profiling Autoantibodies against Salivary Proteins in Sicca Conditions. 2019 Jul Salivary gland dysfunction occurs in several autoimmune and immune-related conditions, including Sjögren syndrome (SS); immune checkpoint inhibitor-induced sicca (ICIS) that develops in some cancer patients and is characterized by severe, sudden-onset dry mouth; and autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED). Although subjects with these conditions present with oral dryness and often exhibit inflammatory infiltration of the salivary gland, little is known about the B-cell humoral responses directed against salivary gland protein targets. In this study, autoantibodies were evaluated against Ro52, Ro60, and La, as well as against a panel of 22 proteins derived from the salivary proteome. The tested cohort included healthy volunteers and subjects with SS, ICIS, and APECED without and with sicca. As expected, a high percentage of autoantibody seropositivity was detected against Ro52, Ro60, and La in SS, but only a few ICIS patients were seropositive for these autoantigens. A few APECED subjects also harbored autoantibodies to Ro52 and La, but only Ro60 autoantibodies were weakly associated with a small subset of APECED patients with sicca. Additional testing of the salivary panel failed to detect seropositive autoantibodies against any of the salivary-enriched proteins in the SS and ICIS subjects. However, APECED subjects selectively demonstrated seropositivity against BPI fold containing family A member 1 (BPIFA1), BPI fold containing family A member 2 (BPIFA2)/parotid salivary protein (PSP), and lactoperoxidase, 3 salivary-enriched proteins. Moreover, high levels of serum autoantibodies against BPIFA1 and BPIFA2/PSP occurred in 30% and 67% of the APECED patients with sicca symptoms, respectively, and were associated with an earlier age onset of oral dryness (P = 0.001). These findings highlight the complexity of humoral responses in different sicca diseases and provide new insights and biomarkers for APECED-associated sicca (ClinicalTrials.gov: NCT00001196; NCT00001390; NCT01425892; NCT01386437).
30620697 Connective Tissue Disorders in Childhood: Are They All the Same? 2019 Jan Systemic connective tissue disorders are characterized by the presence of autoantibodies and multiorgan system involvement. Juvenile systemic lupus erythematosus with or without associated antiphospholipid syndrome; juvenile dermatomyositis; sclerodermiform syndromes, including systemic and localized sclerodermas and eosinophilic fasciitis; mixed connective tissue disease; and Sjögren syndrome are the disorders that affect children most frequently. Diagnosis is difficult, because the clinical presentation of patients is diverse, from mild to severe disease. In addition, all organs may be affected. However, a variety of imaging techniques are now available to investigate rheumatic disease in children. These imaging modalities offer the potential for earlier diagnosis and improved assessment of therapeutic response. This article reviews the main connective tissue disorders that affect children, highlighting their key imaging features on images acquired with different diagnostic imaging modalities and correlating these features with clinical and pathologic findings, when available. (©)RSNA, 2019.
30744521 Usefulness of composite indices in the assessment of joint involvement in systemic lupus e 2019 Mar Specific indices are not available to evaluate systemic lupus erythematosus (SLE) joint involvement; indeed, the application of indices validated for rheumatoid arthritis has been suggested. We evaluated the usefulness of organ specific composite indices, i.e. the Disease Activity Score on 28 joints (DAS28), Simplified Disease Activity Index (SDAI), Clinical Disease Activity Index (CDAI), and the ratio of swollen to tender joints (STR), to assess SLE joint activity by analyzing the correlation between these indices and ultrasonography (US) inflammatory status. We evaluated SLE patients with arthralgia and/or arthritis: the above-mentioned indices were calculated and the SLE Disease Activity Index 2000 (SLEDAI-2k) was applied to assess global disease activity. US of I-V metacarpophalangeal, I-V proximal interphalangeal, wrist, and knee bilateral was performed. Synovial effusion/hypertrophy and power Doppler findings were scored according to a semi-quantitative scale (0-3) to obtain an inflammatory total score (0-216). One hundred and six patients (M/F 7/99, median age 49.5 years (IQR 17.0), median disease duration 8.5 years (IQR 17.0)) were enrolled. We identified a positive correlation between US score and DAS28-CRP ( r = 0.3, p = 0.007), STR ( r = 0.42, p = 0.0005), SDAI ( r = 0.33, p = 0.02), CDAI ( r = 0.29, p = 0.03); US score reflected different levels of clinimetric joint activity. In conclusion, we suggest the ability of composite indices in detecting SLE joint inflammation and their possible real-life use.
31749743 Beyond Adaptive Mental Functioning With Pain as the Absence of Psychopathology: Prevalence 2019 Chronic pain outcomes are traditionally defined in terms of disability and illness. A definition of adaptive functioning in the context of chronic pain beyond the mere absence of negative outcomes, is the ability to flourish (i.e., experience emotional, psychological and social well-being; Keyes, 2002). We explored in two chronic pain samples the prevalence and sociodemographic, physical and psychological correlates of flourishing, and complemented this exploration with a similar examination of (being at risk for) psychopathology to help contextualize findings. Sample 1 (n = 1498) was a nationally representative sample. Subgroups included people with regular joint pain (1), regular joint pain and rheumatoid arthritis (2) and without chronic pain (3). Using chi-square tests we calculated the prevalence of both mental health outcomes and examined if people with or without chronic pain were more/less likely to flourish/at risk for psychopathology. Sample 2 (n = 238) concerned baseline data of a Randomized Controlled Trial on the effectiveness of Acceptance and Commitment Therapy for chronic pain (Trompetter et al., 2015b). We performed logistic regression analysis to identify flourishers/those at risk for depression. The Mental Health Continuum-Short Form was used to measure flourishing. The prevalence of flourishing was 34% (recurrent joint pain) and 38% (recurrent joint pain and arthritis) in sample 1, and 23% in sample 2. Compared to those without chronic pain, people with chronic pain were as likely to flourish, but more likely to be at risk for psychopathology. In sample 2, both flourishing and being at risk for depression were related foremost to psychological correlates. While engaged living was the most important correlate of flourishing, pain catastrophizing and psychological inflexibility were most important correlates of being at risk for depression. In conclusion, people with chronic pain are able to flourish. Findings suggest that positive and negative chronic pain outcomes function on two different continua, with potentially unique protective and risk factors. The Psychological Flexibility model provides pathways to explain both poor and optimal functioning in the presence of chronic pain. A better understanding of people with chronic pain who are able to flourish can be a fruitful endeavor to improve chronic pain models and interventions.
30905461 The serum SA levels are significantly increased in sepsis but decreased in cirrhosis. 2019 Most of proteins in human blood circulation are glycoproteins with one or more covalently linked N- or O-linked glycans. Sialic acid (SA) generally occurs as the terminal monosaccharide on the glycans. SA in glycoproteins modulates a wide range of physiological and pathological processes and has been routinely measured in hospital since 1950s. Increased serum SA levels have been associated with different types of cancers. However, a systematic comparison of the serum SA levels in different types of human diseases has not been reported. In current study, 160,537 clinical lab test results of serum SA levels from healthy individuals and patients with 64 different types of diseases during the past 5 years in our hospital were retrieved and analyzed. Based on the mean (SD), median, and p (-Log(10)p) values, we found that patients suffering 55 different types of cancer and noncancer diseases such as sepsis, pancreatitis, bone cancer, rheumatoid arthritis, pancreatic cancer, and encephalitis had significantly (p<0.05, -Log(10)p>1.30) increased median serum SA levels whereas patients suffering hepatic encephalopathy, cirrhosis, renal cyst, and hepatitis had significantly decreased median serum SA levels compared to that of healthy controls. Moreover, the greatest increase in the mean (SD) and -Log(10)p values was observed in sepsis and pancreatitis, respectively, but not in cancers. Thus, the regulations of serum SA levels were much more complicated than previously assumed. Understanding the molecular mechanisms behind these observations would make serum SA a useful biomarker to facilitate personalized diagnosis and treatment for patients with different diseases.
31540835 [Drug-induced Sweet's syndrome related to hydroxychloroquine: About 2 cases]. 2020 Apr INTRODUCTION: Hydroxychloroquine is widely prescribed in systemic lupus erythematosus. Dermatologic adverse drug reactions are rare but can mimic a disease specific manifestation of lupus. Exceptionally, Sweet's syndrome, or acute febrile neutrophilic dermatosis, may be drug-induced. CASE REPORTS: Two patients aged 31 and 42 years were treated with hydroxychloroquine for systemic lupus and Sjogren's syndrome, respectively. Three weeks after starting treatment, they had a febrile, purple and erythematous papular rash of the trunk and limbs. There was a biological inflammatory syndrome and skin biopsy disclosed an infiltrate of the dermis rich in neutrophils. Lesions regressed after stopping hydroxychloroquine and introducing systemic corticosteroid therapy. Allergologic tests discussed the differential diagnosis with a delayed-type hypersensitivity reaction. CONCLUSION: We report two exceptional cases of drug-induced Sweet's syndrome related to hydroxychloroquine treatment in autoimmune rheumatic diseases.
31464670 Sicca/Sjögren's syndrome triggered by PD-1/PD-L1 checkpoint inhibitors. Data from the Int 2019 May OBJECTIVES: To analyse the worldwide occurrence of sicca/Sjögren's (SS) syndrome associated with the use of immune checkpoint inhibitors (ICI) in patients with cancer. METHODS: The ImmunoCancer International Registry (ICIR) is a Big Data-Sharing multidisciplinary network composed by 40 specialists in Rheumatology, Internal Medicine, Immunology and Oncology from 18 countries focused on the clinical and basic research of the immune-related adverse events (irAEs) related to cancer immunotherapies. For this study, patients who were investigated for a clinical suspicion of SS after being exposed to ICI were included. RESULTS: We identified 26 patients (11 women and 15 men, with a mean age at diagnosis of 63.57 years). Underlying cancer included lung (n=12), renal (n=7), melanoma (n=4), and other (n=3) neoplasia. Cancer immunotherapies consisted of monotherapy (77%) and combined regimens (23%). In those patients receiving monotherapy, all patients were treated with PD-1/PD-L1 inhibitors (nivolumab in 9, pembrolizumab in 7 and durvalumab in 4); no cases associated with CTLA-4 inhibitors were identified. The main SS-related features consisted of dry mouth in 25 (96%) patients, dry eye in 17 (65%), abnormal ocular tests in 10/16 (62%) and abnormal oral diagnostic tests in 12/14 (86%) patients. Minor salivary gland biopsy was carried out in 15 patients: histopathological findings consisted of mild chronic sialadenitis in 8 (53%) patients and focal lymphocytic sialadenitis in the remaining 7 (47%); a focus score was measured in 5 of the 6 patients (mean of 1.8, range 1-4). Immunological markers included positive ANA in 13/25 (52%), anti-Ro/ SS-A in 5/25 (20%), RF in 2/22 (9%), anti-La/SS-B in 2/25 (8%), low C3/C4 levels in 1/17 (6%) and positive cryoglobulins in 1/10 (10%). Classification criteria for SS were fulfilled by 10 (62%) out of 16 patients in whom the two key classificatory features were carried out. Among the 26 patients, there were only 3 (11%) who presented exclusively with sicca syndrome without organ-specific autoimmune manifestations. Therapeutic management included measures directed to treat sicca symptoms and therapies against autoimmune-mediated manifestations (glucocorticoids in 42%, second/third-line therapies in 31%); therapeutic response for systemic features was observed in 8/11 (73%). No patient died due to autoimmune involvement. CONCLUSIONS: Patients with Sjögren's syndrome triggered by ICI display a very specific profile different from that reported in idiopathic primary SS, including more frequent occurrence in men, a higher mean age, a predominant immunonegative serological profile, and a notable development of organ-specific autoimmune involvement in spite of the poor immunological profile. The close association found between sicca/Sjögren's syndrome and primarily PD-1 blockade requires further specific investigation.
31420813 Effectiveness and safety of abatacept for the treatment of patients with primary Sjögren' 2020 Jan Sjögren's syndrome is an autoimmune disease characterized by inflammation of the exocrine glands. The disease can be primary or secondary (if it is associated with another autoimmune disease). In Barring symptom management, there is no established treatment. To evaluate the effectiveness and safety of abatacept as a treatment of primary Sjögren's syndrome over the course of 24 months. Eleven patients with primary Sjögren's syndrome from the Rheumatology Department of Universidade Santo Amaro, Sao Paulo, Brazil were enrolled for a prospective observational study. Eligible participants were diagnosed according to the 2002 American-European consensus criteria and had a score greater than 3 on the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI). Participants received intravenous abatacept for 24 months at a weight-adjusted dose of 500 mg for patients weighing < 60 kg and 750 mg for those weighing > 60 kg. The outcomes were ESSDAI activity index, non-stimulated salivary flow rate, ocular dryness (Schirmer test, tear film break-up time, and ocular staining score), SF-36 questionnaire, and Fatigue domain of the FACIT (Functional Assessment of Chronic Illness Therapy) index. There was a statistically significant reduction in ESSDAI index and improvement of salivary flow. One subscale of the SF-36 index-emotional role functioning-showed improvement. There was no change in ocular parameters or in the FACIT index. In this sample of 11 patients with primary Sjögren's syndrome, abatacept therapy improved xerostomia and systemic disease activity.Key Points• Abatacept is safe and effective for the treatment of primary Sjögren's syndrome.• Abatacept can improve salivary flow and ESSDAI index in this patient population.
31329136 Radiomics-Based Assessment of Primary Sjögren's Syndrome From Salivary Gland Ultrasonogra 2020 Mar Salivary gland ultrasonography (SGUS) has shown good potential in the diagnosis of primary Sjögren's syndrome (pSS). However, a series of international studies have reported needs for improvements of the existing pSS scoring procedures in terms of inter/intra observer reliability before being established as standardized diagnostic tools. The present study aims to solve this problem by employing radiomics features and artificial intelligence (AI) algorithms to make the pSS scoring more objective and faster compared to human expert scoring. The assessment of AI algorithms was performed on a two-centric cohort, which included 600 SGUS images (150 patients) annotated using the original SGUS scoring system proposed in 1992 for pSS. For each image, we extracted 907 histogram-based and descriptive statistics features from segmented salivary glands. Optimal feature subsets were found using the genetic algorithm based wrapper approach. Among the considered algorithms (seven classifiers and five regressors), the best preforming was the multilayer perceptron (MLP) classifier (κ = 0.7). The MLP over-performed average score achieved by the clinicians (κ = 0.67) by the considerable margin, whereas its reliability was on the level of human intra-observer variability (κ = 0.71). The presented findings indicate that the continuously increasing HarmonicSS cohort will enable further advancements in AI-based pSS scoring methods by SGUS. In turn, this may establish SGUS as an effective noninvasive pSS diagnostic tool, with the final goal to supplement current diagnostic tests.
30832451 Association between Periodontal Disease and Subsequent Sjögren's Syndrome: A Nationwide P 2019 Mar 3 Xerostomia (dry mouth) is the cardinal symptom of Sjögren's syndrome (SS), which is an autoimmune disease involving the exocrine glands and other organs. Xerostomia may predispose patients to periodontal disease (PD) and an association between SS and PD has been reported. This association may be bidirectional; therefore, we conducted this study to investigate the risk of SS in patients with PD using data from the National Health Insurance Research Database of Taiwan. A total of 135,190 patients were enrolled in our analysis. In all, 27,041 patients with PD were matched by gender, age, insured region, urbanization and income, with cases and controls in a 1:4 ratio. Both groups were followed and the risks of SS were calculated by Cox proportional hazards regression. Finally, 3292 (2.4%) patients had newly diagnosed SS. Patients with PD had a significantly higher risk of subsequent SS (903 (3.3%) vs. 2389 (2.2%), adjusted hazard 1.47, 95% confidence interval: 1.36⁻1.59). In conclusion, patients with PD had an approximately 50% increased risk of subsequent SS. Physicians should be aware of the symptoms and signs of SS in patients with PD.
30379721 Tear Meniscus and Corneal Sub-basal Nerve Plexus Assessment in Primary Sjögren Syndrome a 2019 Feb PURPOSE: To evaluate lower tear meniscus and corneal sub-basal nerve plexus in primary Sjögren's syndrome (pSS) and Sicca syndrome patients. METHODS: Cross-sectional study of 116 patients with Sicca syndrome associated with pSS and not associated with Sjögren's syndrome (non-SS Sicca) and 20 normal control subjects. Tear meniscus height and area were measured using anterior segment optical coherence tomography; corneal sub-basal nerve plexus density, length, and tortuosity were evaluated using in vivo confocal microscopy. Data analysis was performed using IBM-SPSS Statistics 24.0. RESULTS: Corneal sub-basal nerve plexus density and length were significantly lower, and tortuosity was significantly higher in pSS and non-SS Sicca groups than in normal control subjects (P < 0.001; P = 0.018, respectively). Corneal sub-basal nerve plexus presented a strong association with Schirmer test I and tear breakup time. Cutoff values of sub-basal nerve plexus density (36.5 nerve/mm) and length (12.5 mm/mm) presented 80.2% to 81.9% sensitivity and 85% specificity for detecting Sicca syndrome patients. No significant differences were found between the 3 groups regarding tear meniscus height and area. CONCLUSIONS: Corneal sub-basal nerve plexus in vivo confocal microscopy may be a useful tool in the assessment of dry eye disease in Sicca syndrome, complementing the information provided by the conventional modalities used in dry eye disease evaluation.
31624648 The Role of Ultrasound in Assessing Hand Joints and Tendons in Psoriatic Arthritis. 2019 Apr INTRODUCTION: Psoriatic arthritis (PsA) is an inflammatory arthritis, usually involving peripheral joints, associated with cutaneous psoriasis, in the absence of rheumatoid factor and anti-citrullinated protein antibodies. MATERIAL AND METHODS: The study included 28 patients diagnosed with psoriatic arthritis according to CASPAR criteria, presenting pain or swelling of the small joints of the hands and wrists, hospitalized in the Department of Rheumatology of the Emergency Clinical County Hospital of Craiova during 2016-2018. RESULTS: In the 28 patients, a total of 840 joints were examined clinically and by ultrasound. From the clinical point of view, out of the total, 32 joints were swollen while 59 joints were tender. Dactylitis was evidenced in 20/280 examined digits upon physical examination. MUS revealed the presence of synovial proliferation in 108/840 sites (12.85%), with grade 1 synovitis in 4 joints (3.7%), grade 2 in 94 joints (87.03%) and grade 3 in 10 joints (9.25%). Out of 280 examined fingers 5.71% presented dactylitis, the third digit being the most commonly targeted. Grade 2 tenosynovitis was found in 40 of the examined tendons, whereas 8 tendons presented with grade 3 tenosynovitis. The most frequently involved were the flexor tendons of the digits (38 out of 40 tendons with inflammatory changes), followed by the extensor carpi ulnaris and flexor carpi radialis tendons. CONCLUSIONS: Ultrasound has proven its utility in assessing joint and tendon inflammation to a higher extent that clinically expected. Thus, MUS examination of hand joints may aid the clinician in diagnosing PsA, in evaluating the prognosis and the course of treatment.
32699627 A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis. 2020 Jun Autoimmune disorders such as rheumatoid arthritis or Sjögren's syndrome can be associated with impaired renal acid excretion. Only few cases of patients with primary biliary cirrhosis (PBC) and distal renal tubular acidosis (dRTA) have been described. Here, we present the case of a 60-year-old woman with PBC and dRTA. Her kidney biopsy showed an absence of markers of acid-secretory Type A intercalated cells (A-ICs) and expression of aquaporin-2, a marker of principal cells, in all cells lining the collecting duct. Moreover, the serum of the patient contained antibodies directed against a subset of cells of the collecting duct. Thus, PBC-related autoantibodies may target acid-secretory A-ICs and thereby impair urinary acidification.
32254801 Bibliographic review on the state of the art of strontium and zinc based regenerative ther 2019 Mar 28 Musculoskeletal disorders such as osteoporosis and rheumatoid arthritis are responsible for more than 25k deaths every year in the European Union and constitute a chronic burden on the individuals who suffer from this condition. There is no medical cure for these diseases and there are many therapies applied which have limited effectiveness and severe side effects over time. Regenerative therapies are being studied as a potential treatment for musculoskeletal diseases and are known for their upgrading effects of the natural healing processes carried out in the human body. It is believed that both strontium and zinc play an essential role in bone and cartilage tissue formation, which has led many scientists to study the effect of including these elements to promote tissue formation and inhibit its resorption. In this review, a deep analysis is undertaken of the most relevant developments in strontium and zinc based regenerative therapies that have occurred in the last five years, taking into consideration only those studies reporting significant progress towards real clinical applications. This review brings up to date the state of the art of strontium and zinc based regenerative therapies as it is believed that both have a promoting effect on tissue formation and have an essential role inhibiting resorption in musculoskeletal disorders.
31732938 T Helper 17 Cells as Pathogenic Drivers of Periodontitis. 2019 T helper 17 (Th17) cells were first described as a T helper subset involved in the pathogenesis of experimental autoimmune inflammation. Since then, these cells have been described as orchestrators of immunopathology in several human inflammatory conditions including psoriasis, rheumatoid arthritis, and inflammatory bowel disease. More recently, the crucial role of Th17 cells in the regulation of immunity and protection of barrier sites has been unveiled. In the present work, we review the available evidence regarding Th17 cells in health and disease with a focus on the oral mucosa and their role in periodontitis pathogenesis. Recent mechanistic studies in animal models have demonstrated that interleukin-17A (IL-17A) and Th17 cells are critical mediators for alveolar bone destruction during periodontal inflammation. Observations in a cohort of patients with naturally occurring impaired Th17 cell differentiation supported these findings. However, interventional studies are needed to conclusively implicate Th17 cells in the immunopathogenesis of human alveolar bone and tissue destruction that characterize periodontitis.
31495525 [Pyoderma gangrenosum and systemic lupus erythematosus: A rare association]. 2020 Jan INTRODUCTION: Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that is traditionally associated with systemic disorders such as chronic inflammatory bowel diseases, rheumatoid arthritis and malignant hematologic disorders. Its association with systemic lupus erythematosus (SLE) is rare and not well known. We report a case of this association with a review of the literature. CASE REPORT: A 43-year-old female patient, followed for 4 years for SLE, presented a deep ulceration of the anterior face of the left thigh with inflammatory borders, an ulcerated nodule of the right shoulder and four small ulcerations of the back of the right hand. The biopsy of the ulceration of the left thigh concluded to PG. The patient was treated by corticosteroids with complete healing of lesions. CONCLUSION: The prognosis of lupus does not seem to be aggravated by PG and the treatments of a SLE flare are usually enough for treating associated PG.