Search for: rheumatoid arthritis methotrexate autoimmune disease biomarker gene expression GWAS HLA genes non-HLA genes
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30886967 | Pain, sleep and emotional well-being explain the lack of agreement between physician- and | 2018 | BACKGROUND: Clinical response and remission are defined in multiple ways and measured with different instruments, resulting in substantial variation of the proportion of patients classified as being in remission. Therefore, the agreement between patient-perceived, physician-perceived remission and clinical response and remission definitions was determined in early rheumatoid arthritis (RA) patients. And secondly, differences in clinical and patient-reported outcomes, in patients in physician-perceived remission, between patients in and not in self-perceived remission were assessed. METHODS: In 84 early RA patients, who received methotrexate and glucocorticoids, DAS44, ACR/EULAR Boolean-based remission, EULAR good and ACR70 response were determined after 12 weeks. Agreement between patient-perceived (phrased: "Would you say that, at this moment, your disease activity is as good as gone?"), physician-perceived remission (based on a visual analogue scale for global disease severity) and clinical response and remission definitions were calculated with the percentage of agreement and with kappa values (which corrects for change). In patients in physician-perceived remission, improvement in clinical and patient-reported outcomes (RAID) were compared between patients in and not in self-perceived remission. RESULTS: Agreement between the assessed outcome measures differed enormously. The agreement between physician-perceived and patient-perceived remission was 64% (kappa 0.25, p < 0.01). Physician-perceived remission had the best agreement with EULAR good response (79%), and patient-perceived remission with EULAR good and ACR70 response (both 69%). Patients not in self-perceived remission improved less on RAID components, especially on pain, sleep and emotional well-being. CONCLUSION: One-third of the early RA patients disagreed with the physician on being in remission. Those patients had less improvement on RAID components, especially on pain, sleep and emotional well-being. Together with the variability in clinical response and remission definitions, these results highlight the need to increase patient involvement in their own health care decisions. | |
29593743 | Therapeutic Potential of Invariant Natural Killer T Cells in Autoimmunity. | 2018 | Tolerance against self-antigens is regulated by a variety of cell types with immunoregulatory properties, such as CD1d-restricted invariant natural killer T (iNKT) cells. In many experimental models of autoimmunity, iNKT cells promote self-tolerance and protect against autoimmunity. These findings are supported by studies with patients suffering from autoimmune diseases. Based on these studies, the therapeutic potential of iNKT cells in autoimmunity has been explored. Many of these studies have been performed with the potent iNKT cell agonist KRN7000 or its structural variants. These findings have generated promising results in several autoimmune diseases, although mechanisms by which iNKT cells modulate autoimmunity remain incompletely understood. Here, we will review these preclinical studies and discuss the prospects for translating their findings to patients suffering from autoimmune diseases. | |
28060136 | PURTSCHER-LIKE RETINOPATHY ASSOCIATED WITH ADULT-ONSET STILL DISEASE. | 2018 Fall | PURPOSE: To describe clinical, laboratory, and funduscopic data of a patient presenting with newly diagnosed adult-onset Still disease and Purtscher-like retinopathy. METHODS: Observational case report. PATIENT: A 43-year-old man presented with a 3-week history of daily high-spiking fever, diffuse arthralgia and myalgia, sore throat, and a transient nonpruritic maculopapular rash. Two weeks from the onset of his illness, he developed a gradual decrease in visual acuity in both eyes. Fundoscopic examination showed multiple areas of retinal whitening, discrete superficial retinal hemorrhages, and few characteristic Purtscher flecken in the left eye. RESULTS: The patient was diagnosed with adult-onset Still disease. We used high-dose corticosteroid therapy with complete resolution of adult-onset Still disease, but it was ineffective for recovery of vision. CONCLUSION: Clinicians should be aware of Purtscher-like retinopathy as a severe ophthalmic complication of adult-onset Still disease. | |
29515591 | The Potential Role of Trained Immunity in Autoimmune and Autoinflammatory Disorders. | 2018 | During induction of trained immunity, monocytes and macrophages undergo a functional and transcriptional reprogramming toward increased activation. Important rewiring of cellular metabolism of the myeloid cells takes place during induction of trained immunity, including a shift toward glycolysis induced through the mTOR pathway, as well as glutaminolysis and cholesterol synthesis. Subsequently, this leads to modulation of the function of epigenetic enzymes, resulting in important changes in chromatin architecture that enables increased gene transcription. However, in addition to the beneficial effects of trained immunity as a host defense mechanism, we hypothesize that trained immunity also plays a deleterious role in the induction and/or maintenance of autoimmune and autoinflammatory diseases if inappropriately activated. | |
29765703 | Effects of baricitinib on radiographic progression of structural joint damage at 1 year in | 2018 | BACKGROUND: Baricitinib was efficacious in a 24-week phase III study in patients with rheumatoid arthritis (RA) and an inadequate response to conventional synthetic disease-modifying anti rheumatic drugs (DMARDs) (csDMARDs) (RA-BUILD). OBJECTIVES: To evaluate radiographic progression of structural joint damage in RA-BUILD patients over 48 weeks of baricitinib treatment in the long-term extension study, RA-BEYOND. METHODS: In RA-BUILD, patients were randomised to placebo, baricitinib 2 mg or 4 mg once daily, with rescue possible from week 16. Patients completing RA-BUILD and entering RA-BEYOND continued to receive the baricitinib dose received at the end of RA-BUILD. Patients receiving placebo were switched to baricitinib 4 mg in RA-BEYOND. Joint damage was measured using the van der Heijde modified total Sharp score. To account for missing scores and scores obtained after rescue, switch or discontinuation of study drug, data were analysed using (1) linear extrapolation (LE) and (2) observed/last observation carried forward (LOCF). The observed/LOCF method used all available observed data, including after rescue or switch, with patients analysed according to original treatment assignment. RESULTS: Using LE, radiographic progression at 24 and 48 weeks was statistically significantly lower for both baricitinib 2 or 4 mg compared with placebo. Only baricitinib 4 mg demonstrated statistically significant inhibition of progressive radiographic joint damage compared with patients initially randomised to placebo using observed/LOCF at week 48. CONCLUSIONS: Once daily oral baricitinib inhibited radiographic progression of structural joint damage in patients with an inadequate response or intolerance to csDMARDs over 48 weeks. The most robust benefit was seen for the 4 mg dose. | |
29848388 | From supernatants to cytokines: a personal view on the early history of IL-1, IL-1Ra, TNF | 2018 May 30 | Long-term cell cultures developed early in the twentieth century allowed identification in their supernatants of biological mediators subsequently defined as migration factors, interferons, lymphokines, monokines, cytokines and interleukins. In rheumatology, early in the 1930s, synovial cell cultures revealed two major distinct populations, i.e. synovial fibroblasts and monocyte-macrophages. Discovery of the interstitial collagenase (MMP-1) and its role in tissue destruction, such as in rheumatoid arthritis (RA), raised the question of the cellular source for this enzyme. My personal interest in the field was driven by the lack of understanding for the link between tissue destruction and immunology. This triggered our seminal contribution to the field, establishing in 1976-79 at the Arthritis Unit (Massachusetts General Hospital, with SM Krane) that a mononuclear factor (MCF, around 15 kDa) produced by stimulated macrophage, under direct contact with activated T cells, induced large amounts of collagenase and prostaglandin E(2) (PGE(2), a bone resorbing agent) in human synovial fibroblasts from RA patients. Our original "MCF" biological observations preceded cloning, and recombinant IL-1β confirmed the biological activity of the purified natural IL-1. Following my return to Geneva in 1980 and searching for a high level of IL-1 in urine and serum of patients with high fever or Still's disease, to our surprise-"a finding of absence"-we found that IL-1 was masked by a factor of approximately 17 kDa and first presented this in 1984 at the Fourth International Lymphokine Workshop. In 1987, before IL-Ra cloning, my co-worker P Seckinger and I demonstrated first-time observation in cytokine biology that the mechanism was due to the inhibition of IL-1 binding the cell surface receptor, leading to the concept of IL-1 receptor antagonist (IL-1Ra). Having reported in 1985 that TNF/cachectin also induced collagenase and PGE(2) in human synovial cells, we found that IL-1Ra did not block TNF-α but was due to another inhibitor. As other investigators, we confirmed that this inhibitory factor was a soluble TNF receptor. The years between the 1970s and 1990s were probably the most exciting period in the field of cytokines and cytokine antagonists; it gave rise to two concepts in the cytokine field-one of the receptor antagonist, and the other of soluble receptor antagonists. | |
29227497 | Dietary fruits and arthritis. | 2018 Jan 24 | Arthritis is a global health concern affecting a significant proportion of the population and associated with reduced quality of life. Among the different forms of arthritis, osteoarthritis (OA) and rheumatoid arthritis (RA) are the most common and lacking a definite cure in the affected individuals. Fruits, such as berries and pomegranates are rich sources of a variety of dietary bioactive compounds, especially the polyphenolic flavonoids that have been associated with antioxidant, anti-inflammatory and analgesic effects. Emerging research demonstrates a protective role of fruits and their polyphenols in pre-clinical, clinical and epidemiological studies of OA and RA. In this context, commonly available fruits, such as blueberries, raspberries and strawberries, and pomegranates have shown promising results in reducing pain and inflammation in experimental models and in human clinical studies of arthritis. There is also some evidence on the role of specific fruit polyphenols, such as quercetin and citrus flavonoids in alleviating RA symptoms. These emerging data deserve further investigation in rigorous scientific studies to determine the mechanisms, dosing and selection of fruits and fruit extracts in arthritis management. | |
29359591 | Bone marrow mesenchymal stem cells suppress IL-9 in adjuvant-induced arthritis. | 2018 Feb | Interleukin-9 (IL-9) has been shown to be upregulated in rheumatoid arthritis (RA). The exact role of IL-9 has not yet been effectively studied. Mesenchymal stem cells (MSCs) have shown a promising immunomodulatory role towards repairing cartilage and restoring joint function. One of the key problems influencing the therapeutic efficacy of stem cell therapy is the poor cell survival following transplantation. This is attributed to oxidative and inflammatory stresses at the injured sites. Hesperidin (Hsd), a flavanone present in citrus fruits, has been studied as potential therapeutic agents that have anti-oxidant and anti-inflammatory activities. The objective of this study is to evaluate the therapeutic paracrine action of bone marrow MSCs on the IL-9 level in adjuvant-induced arthritis (AIA) and the enhancement effect of Hsd on transplanted MSCs. Articular tissue inflammation and cartilage damage were assessed by histological scoring. Antinuclear autoantibodies, tumour necrosis factor-alpha (TNF-α), IL-9, IL-4, interferon gamma (IFN-δ), and transforming growth factor-beta1 (TGF-β1), as well as malondialdehyde (MDA), glutathione (GSH), and superoxide dismutase (SOD) levels, were assessed in spleen tissue homogenates after treatment with MSCs either alone or combined with Hsd for 4 weeks in an AIA rat model. Results of this study confirmed that MSCs decreased IL-9 levels in AIA and provide novel insights into the application of Hsd on MSC-based treatments. Highlights Adjuvant-induced arthritis (AIA) is one of the most widely used models that has a great similarity to rheumatoid arthritis (RA). Few studies in recent years have estimated IL-9 in rheumatic diseases and it remains an understudied cytokine. For the first time, bone marrow mesenchymal stem cells (MSCs) therapy has a vital role in splenocytes IL-9 level and further studies are required. Combined therapy of MSCs with antioxidants as hesperidin (Hsd) can alleviate oxidative stress and enhance stem cells immunomodulatory action. | |
29907975 | Metabolite assignment of ultrafiltered synovial fluid extracted from knee joints of reacti | 2019 Jan | Currently, there are no reliable biomarkers available that can aid early differential diagnosis of reactive arthritis (ReA) from other inflammatory joint diseases. Metabolic profiling of synovial fluid (SF)-obtained from joints affected in ReA-holds great promise in this regard and will further aid monitoring treatment and improving our understanding about disease mechanism. As a first step in this direction, we report here the metabolite specific assignment of (1) H and (13) C resonances detected in the NMR spectra of SF samples extracted from human patients with established ReA. The metabolite characterization has been carried out on both normal and ultrafiltered (deproteinized) SF samples of eight ReA patients (n = 8) using high-resolution (800 MHz) (1) H and (1) H─(13) C NMR spectroscopy methods such as one-dimensional (1) H CPMG and two-dimensional J-resolved(1) H NMR and homonuclear (1) H─(1) H TOCSY and heteronuclear(1) H─(13) C HSQC correlation spectra. Compared with normal SF samples, several distinctive (1) H NMR signals were identified and assigned to metabolites in the (1) H NMR spectra of ultrafiltered SF samples. Overall, we assigned 53 metabolites in normal filtered SF and 64 metabolites in filtered pooled SF sample compared with nonfiltered SF samples for which only 48 metabolites (including lipid/membrane metabolites as well) have been identified. The established NMR characterization of SF metabolites will serve to guide future metabolomics studies aiming to identify/evaluate the SF-based metabolic biomarkers of diagnostic/prognostic potential or seeking biochemical insights into disease mechanisms in a clinical perspective. | |
30218288 | Red blood cell distribution width as a potential predictor of survival of pulmonary arteri | 2019 Feb | Pulmonary arterial hypertension (PAH) is a severe complication and leading cause of mortality in patients with primary Sjogren's syndrome (pSS). This study was to investigate the overall survival rates and the utility of red blood cell distribution width (RDW) as a potential prognostic factor of pSS-PAH. This cohort study retrospectively enrolled 55 patients with pSS-PAH who were followed up at the Department of Rheumatology of Peking Union Medical College Hospital (PUMCH) between August 2007 and May 2017. The patients were stratified according to the level of RDW (≤ 15.0 and > 15.0%). Baseline demographics, laboratory results, pulmonary function conditions, hemodynamic assessments, and treatment regimens were analyzed. Cox proportional hazards regression analysis was used to identify whether RDW level is a factor related to adverse outcome. A total of 55 patients were recruited, with an average age of 38.9 ± 9.3 years. Fifty-four were female (98.2%), and the average duration at the time of PAH diagnosis was 25.5 ± 33.2 months. Higher RDW levels were found in patients who deceased in follow-up (13.8 ± 2.6 vs 16.5 ± 1.6%, p = 0.003) and with higher NYHA classes (13.8 ± 1.8 vs 16.5 ± 2.9%, p < 0.001). Patients with RDW > 15% had a significantly worse overall survival than patients with RDW ≤ 15% (3-year survival rate 59.5 vs. 88.7% log-rank p = 0.015). Cox regression analysis identified RDW > 15% as a prognostic factor for adverse outcome (HR 1.786, 95% CI 1.137-2.803, p = 0.012). RDW can serve as a potential negative prognostic factor of pSS-PAH. | |
29902805 | Evidence for the Detection of Subclinical Retinal Involvement in Systemic Lupus Erythemato | 2018 | BACKGROUND: Retinal involvement in systemic lupus erythematosus (SLE) and Sjögren syndrome (SS) may be subclinical and thus underdiagnosed. OBJECTIVES: We aimed at evaluating morphological and functional visual abnormalities in a cohort of SLE and SS patients in the absence of an overt clinical visual impairment. We also investigated potential associations between retinal disorders and disease activity, organ involvement, and treatment with steroid and/or hydroxychloroquine. METHODS: The study comprised 42 SLE and 36 primary SS patients and 76 healthy controls (HC). Ophthalmological examination, standard automated perimetry, spectral-domain optical coherence tomography, and fundus perimetry were performed. RESULTS: Retinal thickness of the posterior pole was not different between SLE and HC groups, but it was reduced in the SS group compared with both the HC and the SLE group. In SLE and SS patients, mean defect and pattern standard deviation by standard automated perimetry were higher than in HC. Visual field index values were lower in both SLE and SS patients than in HC. SLE patients with nephritis displayed increased mean defect and pattern standard deviation and reduced visual field index values compared to patients without nephritis. In SLE and SS patients, fundus perimetry differential sensitivity was reduced, and mean defect values were higher than in HC. Disturbances in fundus perimetry in the SLE group were more prevalent in steroid-naïve patients and in SS patients who received a cumulative hydroxychloroquine dose > 1,000 g. CONCLUSIONS: Functional eye impairment was demonstrated in SLE patients, possibly associated with kidney involvement. In SLE, corticosteroids might exert a protective role. Morphological alterations and functional impairment were detected in SS patients, which may be linked to hydroxychloroquine toxicity. | |
29526328 | [Gastroparesis may be the cause of unexplained dyspepsia in patients with primary Sjögren | 2018 Jun | INTRODUCTION: Upper digestive symptoms may be present in up to 50% of patients with primary Sjögren syndrome (pSS). We report a retrospective cohort of gastroparesis in a population of pSS presenting unexplained dyspepsia. Delayed gastric emptying was defined by a gastric emptying time above 113min or by a retention percentage at 4h more than 10% on scintigraphy. RESULTS: Eleven patients with primary Sjögren syndrome and gastroparesis were included in a retrospective study. Every patients were women of age 48±18y. The average time of gastric emptying was 725,18±704,45min. 64% of patients had abdominal pain or gastric heaviness. A central or peripheral neurologic involvement was described in respectively 9 and 27% of cases. The diagnostic delay of gastroparesis was higher than 24 months. CONCLUSION: In primary Sjögren syndrome, gastroparesis should be suspected in case of unexplained dyspepsia, and a scintigraphy performed to prove the diagnosis. A neurologic involvement could explain gastroparesis, but prospective studies are needed for a better understanding of this disorder. | |
29241348 | The Voice of Autoimmunity: Antisynthetase Syndrome Manifesting as Vocal Fold Bamboo Nodes. | 2018 Feb | OBJECTIVES: To describe a case of vocal fold bamboo nodes leading to the diagnosis of antisynthetase syndrome, a rare autoimmune disorder. To highlight the link between these laryngeal lesions and autoimmunity. METHODS: A case of vocal fold bamboo nodes in a patient with long-standing interstitial lung disease is presented. The presence of these characteristic lesions prompted a rheumatologic workup that led to the diagnosis of a rare autoimmune disorder. RESULTS: The patient was ultimately diagnosed with antisynthetase syndrome, a rare condition characterized by inflammatory myositis and interstitial lung disease. She was treated with steroids and immunosuppressive agents with improvement in her symptoms and clinical findings. CONCLUSIONS: Vocal fold bamboo nodes are pathognomonic signs of autoimmunity. Management consists primarily of medical treatment of the underlying systemic disorder. Intralesional steroid injection or phonomicrosurgical excision may be considered for refractory cases. | |
28585263 | Combined interventional sialendoscopy and intraductal steroid therapy for recurrent sialad | 2018 Feb | OBJECTIVES: To evaluate the effectiveness of interventional sialendoscopy alone or combined with outpatient intraductal steroid irrigations in patients with sialadenitis due to Sjögren's syndrome (SS). DESIGN: A pilot therapeutic study. SETTING: ENT Clinics, Universities of Milan and Pavia. STUDY POPULATION: We included 22 patients with SS of whom 12 underwent interventional sialendoscopy followed by intraductal steroid irrigations (group A), and 10 interventional sialendoscopy alone (group B). OUTCOMES MEASURES: The following outcome measures were considered and recorded before and after the therapeutic intervention: (i) number of episodes of glandular swelling, (ii) cumulative prevalence of patients with glandular swelling assessed by the specific domain, the EULAR SS Disease Activity Index (ESSDAI), (iii) severity of pain by means of a 0-10 pain visual analogue scale (VAS), (iv) severity of xerostomia and other disease symptoms assessed by the EULAR SS Patient Reported Index (ESSPRI) and the Xerostomia Inventory questionnaire. RESULTS: The postoperative reduction in the mean number of episodes of glandular swelling was 87% (95% CI: 77-93) and 75% (95% CI: 47%-88%) in the groups A and B, respectively. The percentage of patients with glandular swelling decreased from 41.7% to 0.0% in the group A and from 30.0% to 0.0% in the group B, respectively. Most of the patients experienced a subjective clinical improvement documented by the statistically significant reductions in the postoperative mean pain VAS (group A P<.001; group B P=.004), Xerostomia Inventory (P<.001 and P=.003) and ESSPRI scores (P<.001 and P=.008). Interventional sialendoscopy followed by outpatient intraductal steroid irrigations was more effective than interventional sialendoscopy alone, when pain VAS, Xerostomia Inventory and ESSPRI scores before and after treatment were analysed together using the multivariate Hotelling T(2) test (P=.0173). CONCLUSIONS: This pilot study confirms that interventional sialendoscopy with steroid duct irrigation significantly reduces the number of painful episodes of sialadenitis and improves the subjective sensation of oral dryness and other disease symptoms in patients with SS. The study results also suggest that the improvement is greater when interventional sialendoscopy is combined with a cycle of outpatient steroid ductal irrigations. Larger controlled randomised studies are certainly needed to confirm these preliminary data. | |
29644928 | Salivary gland ultrasound is linked to the autoimmunity profile in patients with primary S | 2020 Jan | OBJECTIVE: Salivary gland ultrasound (SGU) is a reliable technique for assessing the salivary glands in patients with primary Sjögren’s syndrome (pSS). The aim of this study was to elucidate the relationship between SGU findings and autoimmunity in patients with pSS. METHODS: Patients with pSS underwent an SGU assessment. The patients were classified into three groups according to their autoimmunity profile: the complete positive group (positive rheumatoid factor, antinuclear antibodies, and anti-Ro/anti-La antibodies), the partial seropositive group (positivity of at least one autoantibody but not all), and the seronegative group. RESULTS: In total, 93 patients were evaluated. Eighty-six (92.5%) were female, and their median age was 49.5 years. The median disease duration was 12.3 years. Pathological SGU findings were present in 32 (34.4%) patients [25 of 36 (78.1%) in the complete positive group and 7 of 44 (21.9%) in the partial positive group]. Patients with pathological SGU findings had a shorter disease duration and slightly higher European League Against Rheumatism Sjögren’s syndrome disease activity index. CONCLUSIONS: The autoimmunity profile and pathological SGU findings are strongly associated with each other in patients with pSS. However, the disease duration does not seem to be related to pathological SGU findings. | |
30391023 | Synaptotagmin-1 overexpression under inflammatory conditions affects secretion in salivary | 2019 Feb | Sjögren's syndrome (SS) is an autoimmune exocrinopathy associated with severe secretory alterations by disruption of the glandular architecture integrity, which is fundamental for a correct function and localization of the secretory machinery. Syt-1, PI(4,5)P(2) and Ca(2+) are significant factors controlling exocytosis in different secretory cells, the Ca(2+) role being the most studied. Salivary acinar cells from SS-patients show a defective agonist-regulated intracellular Ca(2+) release together with a decreased IP3R expression level, and this condition may explain a reduced water release. However, there are not reports where Syt-1, PI(4,5)P(2) and Ca(2+) in acinar cells of SS patients had been studied. In the present study, we analyzed the expression and/or localization of Syt-1 and PI(4,5)P(2) in acinar cells of labial salivary gland biopsies from SS-patients and control individuals. Also, we evaluated whether the overexpression of Syt-1 and the loss of cell polarity induced by TNF-α or loss of interaction between acinar cell and basal lamina, alters directionality of the exocytosis process, Ca(2+) signaling and α-amylase secretion in a 3D-acini model stimulated with cholinergic or β-adrenergic agonists. In addition, the correlation between Syt-1 protein levels and clinical parameters was evaluated. The results showed an increase of Syt-1 mRNA and protein levels, and a high number of co-localization points of Syt-1/STX4 and PI(4,5)P(2)/Ezrin in the acinar basolateral region of LSG from SS-patients. With regard to 3D-acini, Syt-1 overexpression increased exocytosis in the apical pole compared to control acini. TNF-α stimulation increased exocytic events in the basal pole, which was further enhanced by Syt-1 overexpression. Additionally, altered acinar cell polarity affected Ca(2+) signaling and amylase secretion. Overexpression of Syt-1 was associated with salivary gland alterations revealing that the secretory dysfunction in SS-patients is linked to altered expression and/or localization of secretory machinery components together with impaired epithelial cell polarity. These findings provide a novel insight on the pathological mechanism implicated in ectopic secretory products to the extracellular matrix of LSG from SS-patients, which might initiate inflammation. | |
30203448 | Rheumatoid-nodule-like cutaneous granuloma associated with recombinase activating gene 1-d | 2018 Dec | Cutaneous granulomas without detectable infectious etiology rarely occur in children and adults with primary immunodeficiency disorders. These cutaneous granulomas are primarily seen in combined variable immunodeficiency, ataxia-telangiectasia, and severe combined immunodeficiency (SCID) and can emulate the reaction patterns seen in sarcoidosis and granuloma annulare. To date, the literature has described only six cases of non-infectious cutaneous granulomas in SCID. We report an unusual case of cutaneous granuloma, mimicking a sarcoma, in a 40-year old male with recombinase activating gene 1-deficient SCID, who presented with a slow-growing globus mass over the lateral aspect of the right elbow. There was heterogeneous enhancement on MRI, which was concerning for neoplasm but no malignancy was found on frozen or permanent sections. GMS, PAS with diastase, and AFB stains, as well as microbiology cultures, were negative. An AE1/AE3 stain was negative and a CD163 stain highlighted histiocytes. No infectious etiology was identified and histopathology revealed palisaded granulomatous dermatitis, most closely resembling a rheumatoid nodule. Although cutaneous manifestations have been reported in nearly half of primary immunodeficiency disorder cases, non-infectious cutaneous granulomas are exceedingly rare in SCID. To our knowledge, this is the first case report of cutaneous palisaded granulomatous dermatitis mimicking a rheumatoid nodule in a major joint. | |
29787840 | Cutaneous features and diagnosis of primary Sjögren syndrome: An update and review. | 2018 Oct | Sjögren syndrome (SS) is an autoimmune connective tissue disorder (CTD) that principally affects the lacrimal and salivary glands. Although SS is 1 of the 3 most common autoimmune CTDs alongside systemic lupus erythematosus and progressive systemic sclerosis, it is the least researched CTD overall. SS poses a particular diagnostic challenge because it shares multiple clinical and immunologic features with other CTDs. However, there are some characteristic cutaneous clinical features that can precede the well-known sicca symptoms by years. By familiarizing themselves with these clinical features and having a high suspicion for SS, dermatologists can play an important role in the early diagnosis and treatment of this disease. | |
30724137 | Influence of Hormones on Sjögren's Syndrome. | 2018 | Sjögren's syndrome (SS) is an immune system oral disorder that is characterized generally by dry mouth and eyes. In this review, SS classification, presentation and pathogenesis are briefly discussed. Moreover, the epidemiology of SS regarding sex, age and association with other complications are also presented. This review also addresses the interactions between endocrine axes and SS, and the important findings up to regarding hormone treatment of this syndrome. The main hormones discussed in this review includes Adrenocorticotropic hormone (ACTH), Follicle-stimulating hormone (FSH), Luteinizing hormone (LH), Thyroid-stimulating hormone (TSH), and prolactin. | |
29606976 | Association of weight loss with improved disease activity in patients with rheumatoid arth | 2018 | OBJECTIVE: To evaluate the association between weight loss and rheumatoid arthritis (RA) disease activity. METHODS: We conducted a retrospective cohort study of RA patients seen at routine clinic visits at an academic medical center, 2012-2015. We included patients who had ≥2 clinical disease activity index (CDAI) measures. We identified visits during follow-up where the maximum and minimum weights occurred and defined weight change and CDAI change as the differences of these measures at these visits. We defined disease activity improvement as CDAI decrease of ≥5 and clinically relevant weight loss as ≥5 kg. We performed logistic regression analyses to establish the association between improved disease activity and weight loss and baseline BMI category (≥25 kg/m(2) or <25 kg/m(2)). We built linear regression models to investigate the association between continuous weight loss and CDAI change among patients who were overweight/obese at baseline and who lost weight during follow-up. RESULTS: We analyzed data from 174 RA patients with a median follow-up of 1.9 years (IQR 1.3-2.4); 117 (67%) were overweight/obese at baseline, and 53 (31%) lost ≥5 kg during follow-up. Patients who were overweight/obese and lost ≥5 kg had three-fold increased odds of disease activity improvement compared to those who did not (OR 3.03, 95%CI 1.18-7.83). Among those who were overweight/obese at baseline, each kilogram weight loss was associated with CDAI improvement of 1.15 (95%CI 0.42-1.88). Our study was limited by using clinical data from a single center without fixed intervals for assessments. CONCLUSION: Clinically relevant weight loss (≥5 kg) was associated with improved RA disease activity in the routine clinical setting. Further studies are needed for replication and to evaluate the effect of prospective weight loss interventions on RA disease activity. |