Search for: rheumatoid arthritis methotrexate autoimmune disease biomarker gene expression GWAS HLA genes non-HLA genes
| ID | PMID | Title | PublicationDate | abstract |
|---|---|---|---|---|
| 29390329 | Primary Sjögren syndrome that initially presented with repeated hypergammaglobulinemic pu | 2017 Dec | RATIONALE: Purpura is a common dermatologic manifestation in Sjögren syndrome (SS). When a patient presents with sicca symptoms, the diagnosis of SS is not difficult. PATIENT CONCERNS: Here, we reported a case of a 52-year-old Chinese woman who initially presented with nonpalpable purpura on both lower extremities, and these lesions had developed soon after prolonged sitting. In the past 2 years, she had repeated cutaneous nonpalpable purpura 4 times. She had no sicca symptoms, dry eyes, or dry mouth. DIAGNOSES: Combining the laboratory findings, Schirmer test, and labial gland biopsy, primary SS was confirmed. INTERVENTIONS: The patient was placed on a trial of hydroxychloroquine (200 mg once daily). OUTCOMES: The purpura on both lower extremities had faded at the sixth day after onset and at the third day after hydroxychloroquine treatment. LESSONS: These case was not easy to diagnosis primary SS because she had no sicca symptoms. A patient with primary SS who initially presented with recurrent purpura associated with prolonged sitting. Prolonged sitting had been a possible aggravating factor for the cutaneous purpura of this patient with primary SS. | |
| 29130141 | Sjögren's syndrome initially presented as thrombotic thrombocytopenic purpura in a male p | 2018 May | Thrombotic thrombocytopenic purpura (TTP) is a potentially lethal multisystem disorder which could be caused by autoimmune diseases. However, the concomitant occurrence of TTP and Sjögren's syndrome (SS) is an extremely uncommon scenario, especially in male patients. A 56-year-old Chinese male was admitted for the appearance of diffuse ecchymosis. Then he gradually developed transient slurred speech, progressive confusion, agitation, extremity weakness, and fever. Laboratory investigations suggested anemia, thrombocytopenia, significantly increased lactic dehydrogenase, schistocytes in peripheral blood smear, and a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 (ADAMTS13) activity deficiency with high inhibitor titers. TTP was thus diagnosed. The patient also had positive anti-nuclear antibody, anti-SSA, and anti-SSB; however, anti-double stranded DNA (dsDNA) was negative. These drove us to perform ocular and dental sicca evaluation and the finial diagnosis was TTP secondary to SS. Plasma exchange and corticosteroid therapy were effective to control TTP. Cyclophosphamide was subsequently added when the platelet count was stable. The total duration of corticosteroid and cyclophosphamide was 8 and 6 months, respectively. The patient recovered without relapse at 1-year follow-up. To our knowledge, this was the first case of SS initially presented as TTP in a male patient. The case also elucidated the importance of autoantibody screen in the workup of TTP and the benefits of adjunctive immunosuppressive therapy in relapse prevention. | |
| 28914368 | Macrophage activation syndrome in Still's disease: analysis of clinical characteristics an | 2017 Dec | Macrophage activation syndrome (MAS) is a reactive form of hemophagocytic lymphohistiocytosis, complicating Still's disease, both in paediatric and adult patients. In this work, we aimed to investigate clinical picture and outcome of Still's disease patients developing MAS. We performed a retrospective analysis of patients, both paediatrics and adults, affected by Still's disease attending our department. During the follow-up, each patient was investigated for MAS occurrence and possible predictors, clinical and laboratory factors, were analysed. We evaluated 50 patients affected by Still's disease, 21 paediatric and 29 adult patients. Ten patients experienced MAS (five adult and five paediatric patients) and its development significantly reduced the survival rate when compared with patients without this complication (p < 0.0001). The analysis of possible predictors showed that high-value systemic score (p = 0.03) and high levels of serum ferritin (p = 0.002) were independently associated with an increased likelihood of MAS. MAS occurrence significantly reduced survival rate in both paediatric and adult patients affected by Still's disease. The high levels of serum ferritin and an elevated systemic score, at the time of diagnosis, were significantly associated with MAS. | |
| 28122643 | Identification of potential saliva and tear biomarkers in primary Sjögren's syndrome, uti | 2017 Jan 25 | BACKGROUND: There is a long-lasting need for non-invasive, more accurate diagnostic techniques when evaluating primary Sjögren's syndrome (pSS) patients. Incorporation of additional diagnostics involving screening for disease-specific biomarkers in biological fluid is a promising concept that requires further investigation. In the current study we aimed to explore novel disease biomarkers in saliva and tears from pSS patients. METHODS: Liquid chromatography-mass spectrometry (LC-MS) was performed on stimulated whole saliva and tears from 27 pSS patients and 32 healthy controls, and salivary and tear proteomic biomarker profiles were generated. LC-MS was also combined with size exclusion chromatography to isolate extracellular vesicles (EVs) from both fluids. Nanoparticle tracking analysis was conducted on joint fractions from the saliva and tears to determine size distribution and concentration of EVs. Further EV characterisation was performed by immunoaffinity capture of CD9-positive EVs using magnetic beads, detected by flow cytometry. The LC-MS data were analysed for quantitative differences between patient and control groups using Scaffold, and the proteins were further analysed using the Database for Annotation, Visualization and Integrated Discovery (DAVID), for gene ontology overrepresentation, and the Search Tool for the Retrieval of Interacting Genes/Proteins for protein-protein interaction network analysis. RESULTS: Upregulation of proteins involved in innate immunity (LCN2), cell signalling (CALM) and wound repair (GRN and CALML5) were detected in saliva in pSS. Saliva EVs also displayed biomarkers critical for activation of the innate immune system (SIRPA and LSP1) and adipocyte differentiation (APMAP). Tear analysis indicated overexpression of proteins involved in TNF-α signalling (CPNE1) and B cell survival (PRDX3). Moreover, neutrophil gelatinase-associated lipocalin was upregulated in saliva and tears in pSS. Consistently, DAVID analysis demonstrated pathways of the adaptive immune response in saliva, of cellular component assembly for saliva EVs, and of metabolism and protein folding in tears in pSS patients. CONCLUSIONS: LC-MS of saliva and tears from pSS patients, solely and in combination with size-exclusion chromatography allowed screening for possible novel biomarkers encompassing both salivary and lacrimal disease target organs. This approach could provide additional diagnostic accuracy in pSS, and could possibly also be applied for staging and monitoring the disease. | |
| 28552100 | Health anxiety and illness behaviour in children of mothers with severe health anxiety. | 2017 May | Excessive health anxiety, still designated as hypochondriasis in ICD-10, refers to worries and anxiety about harbouring serious illness. It is common in both primary and secondary health care with prevalence rates up to 9% and causes great suffering for the individual as well as high health care costs when untreated. Growing research suggests that health anxiety may originate in childhood, and studies have demonstrated that cognitive and behavioural features similar to those described for health anxiety in adults may be present. The development of health anxiety probably has a complex nature involving a number of interacting factors such as genetics and environmental factors. A few studies have highlighted a possible transmission of health anxiety symptoms from a parent to a child and found significant associations between child and parental self-reported health anxiety symptoms. Theoretical perspectives also assume an association between childhood experiences and family factors and a later development of health anxiety. This dissertation is based on a systematic review and a family case-control study and aims to answer the following questions: 1) What is the empirical evidence for the influence of childhood and family factors for the development of health anxiety? 2) Does exposure to severe maternal health anxiety contribute to health anxiety symptoms in their children or perhaps more broadly affect the children emotionally? 3) Do mothers with severe health anxiety express more health anxiety on behalf of their child, more maladaptive illness perceptions and behaviours compared to mothers with rheumatoid arthritis and healthy mothers? The first part, the systematic review, was performed in accordance with the PRISMA statement and focused on the current empirical evidence for childhood and family factors involved in the development of health anxiety. In total 25 papers were examined emanating from 23 studies. The results, based on this limited research, suggested potential relationships between the development of health anxiety and 1) the intergenerational transmission, i.e. from parent to child, of health anxiety symptoms, 2) early childhood experience involving illness and 3) the expression of an anxious attachment style. The second part, the family case-control study, adds to the limited knowledge of health anxiety symptoms in childhood with one paper presenting original data on health anxiety, related symptoms and illness behaviour in three groups of children exposed to different maternal health status. Another paper examines the phenomenon of maternal health anxiety by proxy in mothers with severe health anxiety. The data for these two papers stem from 150 families with a child in the age group 8-17 years. These were grouped into a case group of children of mothers with severe health anxiety and two control groups; children of mothers with rheumatoid arthritis and children of healthy mothers. The children completed a questionnaire battery including items on health anxiety and related constructs. The mothers and fathers filled in questionnaires regarding their own mental and physical health including health anxiety, and the mothers moreover filled in questionnaires regarding illness perceptions, illness worries and illness behaviour related to their children. The findings suggest that severe maternal health anxiety only weakly affects children's own report of health anxiety symptoms and hence may not be a strong risk factor for the development and clinical presentation of excessive health anxiety symptoms early in life, i.e. in children aged 8-17 years. However, mothers with severe health anxiety perceived their children as having more emotional and physical symptoms compared to mothers with RA and healthy mothers and accordingly more often took their child to see a doctor compared to mothers with rheumatoid arthritis. They reported a more negative illness perception and more health anxiety on behalf of their child, i.e. health anxiety by proxy, as well as more dissatisfaction with their medical consultation in general practice regarding their child compared to mothers with rheumatoid arthritis and healthy mothers. Thus, although we in the first study did not find that the children of mothers with severe health anxiety themselves reported more physical symptoms compared to children in the control groups, the findings of the second study raise the possibility that the upbringing by a parent with negative illness perceptions and health anxiety in the long run could learn the child that minor bodily changes (i.e. feeling unwell) are unusual and need extra attention. Targeting health anxiety by proxy in the treatment of mothers who suffer from severe health anxiety may therefore be important to prevent not only iatrogenic harm to the child but also the exposure of the child to a maladaptive illness behaviour, which potentially could be a risk factor for the child to develop this behaviour itself when growing up. | |
| 28879569 | [Virus-associated arthritis]. | 2017 Oct | Epidemiological studies suggest a viral etiology in approximately 1% of patients presenting with acute arthritis. The arthritogenic effect of viral infections may be related to viral invasion of synovial cells, the cellular and humoral immune response to viral antigens or by induction of autoimmunity. Viral arthritis can mimic rheumatoid arthritis by presenting as a symmetrical polyarticular disease often accompanied by a rash and influenza-like symptoms. Serological testing for pathogen-specific IgM and IgG antibodies is frequently performed for establishing a viral etiology of arthritis. Virus isolation from the joints or detection of viral nucleic acids in the synovium or synovial fluid is only rarely successful and does not always provide proof of a viral origin of arthritis. While viral arthritis in most cases is self-limiting, protracted disease can occur. | |
| 27987339 | Evaluation of ovarian reserve using anti-müllerian hormone and antral follicle count in S | 2017 Feb | AIM: The aim of this study was to determine ovarian reserve status using anti-müllerian hormone (AMH) level and antral follicle count (AFC) in patients with Sjögren's syndrome (SS). METHODS: Twenty-four women with SS diagnosed according to the classification criteria proposed by the American-European Consensus Group and 25 healthy women as controls were enrolled in this study. Ovarian reserve was assessed on clinical findings, AFC, and serum AMH and reproductive hormone levels. RESULTS: Compared with the healthy controls, in the SS patients, the duration of menstrual cycle was significantly shorter (P = 0.043); serum AMH (P = 0.001) and AFC (P = 0.001) were significantly lower, and serum luteinizing hormone (LH) was significantly higher (P = 0.019). The right (P = 0.555) and left ovarian (P = 0.386) volumes were also lower but this did not reach statistical significance. Serum follicle-stimulating hormone (P = 0.327), estradiol (P = 0.241), and prolactin (P = 0.55) were similar between the two groups. CONCLUSIONS: Ovarian reserve may be reduced in SS patients. For the assessment of ovarian reserve, serum AMH and ovarian AFC with serum LH may be useful. Further studies with long-term follow-up are required to determine the course of ovarian reserve abnormalities and best possible biomarkers of reduced ovarian reserve in SS patients. | |
| 27682894 | Interstitial lung disease in primary Sjögren's syndrome. | 2017 Jan | Interstitial lung disease (ILD) has been reported in 3 to 11% of patients with primary Sjögren's syndrome (pSS). The aims of this retrospective multicenter study were to: 1) analyze characteristics and outcome of ILD in pSS; and 2) evaluate predictive factors associated with ILD onset and deterioration. Twenty-one of 263 patients with pSS (8%) developed ILD. ILD onset preceded pSS diagnosis (n=5), was concurrently identified in association with pSS (n=6) and developed after pSS onset (n=9). Presenting ILD manifestations were: acute/subacute (n=11) onset of ILD, symptomatic progressive onset of ILD (n=5), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT-scan (n=5). ILD therapy included: steroids (n=21), cyclophosphamide (n=1), azathioprine (n=4) and rituximab (n=1). The course of ILD was as follows: improvement (15.8%), stabilization (47.4%) or deterioration (36.8%). Predictive parameters of ILD onset were: older age (p=0.044), Raynaud's phenomenon (p=0.001) and esophageal involvement (p=0.001). Factors associated with ILD deterioration were: older age (p=0.038) and esophageal involvement (p=0.038). Thus, this study underscores the poor outcome of ILD during pSS; thus, systematic screening of pulmonary involvement is required in pSS patients, resulting in both diagnosis and management at early stage of ILD. We also suggest that patients presenting predictive factors of ILD deterioration may need a closer follow-up and a more aggressive therapy. | |
| 28288719 | Marginal zone lymphoma: Associated autoimmunity and auto-immune disorders. | 2017 Mar | Large epidemiological studies have shown a consistent increased risk for developing lymphoma in the setting of autoimmune disorders (AID). It is known that this link appears to be stronger for some AID and certain non-Hodgkin lymphoma subtypes e.g. Sjögren's syndrome and extra-nodal marginal zone lymphoma of the salivary gland, and thyroid MALT lymphoma in a background of Hashimoto's thyroiditis. B and T-cell hyperactivity due to chronic antigenic stimulation and the consequent presence of acquired lymphoid tissue seems to play a key role in the pathogenesis of AI-related lymphomas. Advanced age at diagnosis, prolonged disease course and disease severity are thought to increase the risk of lymphoma development in AID patients. There is increasing evidence that AI-related lymphomas constitute a different spectrum of entities indicating a different pathobiology with specific clinical features and treatment implications. This chapter will provide a general overview on the epidemiological aspects of the NHL-AID association focussing on marginal zone lymphomas - one of the NHL subtypes mostly implicated in the synchronous/metachronous association with AID. We will review the possible biological mechanisms involved and the risk factors in each autoimmune condition related to this lymphoma. | |
| 28282148 | Alterations in the Salivary Proteome and N-Glycome of Sjögren's Syndrome Patients. | 2017 Apr 7 | We used isobaric mass tagging (iTRAQ) and lectin affinity capture mass spectrometry (MS)-based workflows for global analyses of parotid saliva (PS) and whole saliva (WS) samples obtained from patients diagnosed with primary Sjögren's Syndrome (pSS) who were enrolled in the Sjögren's International Collaborative Clinical Alliance (SICCA) as compared with two control groups. The iTRAQ analyses revealed up- and down-regulation of numerous proteins that could be involved in the disease process (e.g., histones) or attempts to mitigate the ensuing damage (e.g., bactericidal/permeability increasing fold containing family (BPIF) members). An immunoblot approach applied to independent sample sets confirmed the pSS associated up-regulation of β2-microglobulin (in PS) and down-regulation of carbonic anhydrase VI (in WS) and BPIFB2 (in PS). Beyond the proteome, we profiled the N-glycosites of pSS and control samples. They were enriched for glycopeptides using lectins Aleuria aurantia and wheat germ agglutinin, which recognize fucose and sialic acid/N-acetyl glucosamine, respectively. MS analyses showed that pSS is associated with increased N-glycosylation of numerous salivary glycoproteins in PS and WS. The observed alterations of the salivary proteome and N-glycome could be used as pSS biomarkers enabling easier and earlier detection of this syndrome while lending potential new insights into the disease process. | |
| 27098775 | Pain, xerostomia, and younger age are major determinants of fatigue in Korean patients wit | 2017 Jan | OBJECTIVES: Fatigue is a common clinical manifestation in patients with primary Sjögren's syndrome (pSS). The aims of this study were to investigate the association between fatigue severity and other clinical characteristics in pSS patients and to determine the factors contributing to fatigue. METHOD: We analysed 257 participants from the Korean Initiative of pSS (KISS), a prospective pSS cohort. Fatigue was assessed according to the fatigue domain of the European League Against Rheumatism (EULAR) Sjögren's Syndrome Patient-Reported Index (ESSPRI). Health-related quality of life (HRQoL) was evaluated using the EuroQol-5 dimensions (EQ-5D) questionnaire. Multiple linear regression analysis was used to estimate the effect of each variable on fatigue severity. RESULTS: The median total ESSPRI score was 5 [interquartile range (IQR) 4-6]. Thirty-four per cent of patients reported a fatigue score > 5. Younger and premenopausal patients presented with more fatigue (p = 0.013 and p < 0.001, respectively). Higher Xerostomia Inventory (XI) scale (p < 0.001) and Ocular Surface Dryness Index (OSDI) (p < 0.001) scores were observed in patients with a fatigue score > 5. Pain, xerostomia, and age were determined to be significantly associated with fatigue severity after adjusting for depression/anxiety, OSDI score, and the presence of fibromyalgia using a multivariate general linear model. The ESSPRI fatigue score was correlated with the EQ-5D by time trade-off (TTO) values and visual analogue scale (VAS) scores. CONCLUSIONS: In Korean patients with pSS, younger age, xerostomia, and pain were correlated significantly with fatigue, and fatigue was associated with HRQoL. | |
| 28353596 | Adult-onset hypophosphatemic osteomalacia associated with Sjogren syndrome: Clinical case | 2017 Mar | RATIONALE: Hypophosphatemic osteomalacia (HO) is a metabolic bone disease, exhibiting different etiologies such as genetic mutation, tumor induction, dysimmunity, or renal disease. Sjogren's syndrome (SS) is a connective tissue disorder commonly involving exocrine glands; however kidney involvement is also encountered, leading to abnormal phosphorus metabolism, even HO. PATIENT CONCERNS: A 47-year-old female patient presented progressively worsening pain in the chest wall, back and bilateral lower extremities as well as muscle weakness was referred to our department. DIAGNOSES, INTERVENTIONS AND OUTCOMES: Due to the laboratory test results, radiographic findings and pathologic results, she was diagnosed with adult-onset HO associated with SS. She was then treated with alkalinization, steroids, neutral phosphate, calcium supplements together with activated vitamin D. So far, she recovered uneventfully with relieved pain and increased serum phosphorus level. LESSONS: HO may be secondary to renal tubular acidosis of SS patients, and it might be a diagnostic challenge when the kidney involvement in SS is latent and precede the typical sicca symptoms. | |
| 28745007 | Better arthritis care: What training do community-based health professionals need to impro | 2018 Mar | OBJECTIVE: The aim of the present study was to identify the competencies that non-specialist community-based nurses and allied health professionals (AHPs) need to enable them to assess, care for and manage arthritis appropriately. METHODS: A Delphi survey with an expert panel of 43 rheumatology specialists and expert patients was used to identify the competencies needed by community-based nurses and AHPs to enable them to improve their care of people with arthritis. The process was informed by feedback from focus groups with arthritis patients, community-based nurses and AHPs. RESULTS: The core competencies in arthritis care needed by non-specialist community-based nurses and AHPs were identified. The key goals identified were to increase the understanding of arthritis and its impact on patients' lives, and to increase the ability to help patients to self-manage their condition and access support. Competencies included an understanding of the pathology underlying inflammatory and non-inflammatory arthritis, the ability to distinguish between the two and the ability to recognize early warning signs, with an emphasis on osteoarthritis (OA), rheumatoid arthritis, gout and septic arthritis. Essential competencies included the ability to engage in shared decision making, goal setting and signposting, to provide patients with education and information and to make appropriate referrals. CONCLUSIONS: Health professionals working in the community commonly encounter arthritis as a presenting problem or as a co-morbidity. The quality of care provided to people with inflammatory arthritis and OA in the community is currently variable. The present study identified the core competencies that all community-based nurses and AHPs should have in relation to OA and inflammatory arthritis. | |
| 28255960 | JAK-STAT Signaling as a Target for Inflammatory and Autoimmune Diseases: Current and Futur | 2017 Apr | The Janus kinase/signal transduction and activator of transcription (JAK-STAT) signaling pathway is implicated in the pathogenesis of inflammatory and autoimmune diseases including rheumatoid arthritis, psoriasis, and inflammatory bowel disease. Many cytokines involved in the pathogenesis of autoimmune and inflammatory diseases use JAKs and STATs to transduce intracellular signals. Mutations in JAK and STAT genes cause a number of immunodeficiency syndromes, and polymorphisms in these genes are associated with autoimmune diseases. The success of small-molecule JAK inhibitors (Jakinibs) in the treatment of rheumatologic disease demonstrates that intracellular signaling pathways can be targeted therapeutically to treat autoimmunity. Tofacitinib, the first rheumatologic Jakinib, is US Food and Drug Administration (FDA) approved for rheumatoid arthritis and is currently under investigation for other autoimmune diseases. Many other Jakinibs are in preclinical development or in various phases of clinical trials. This review describes the JAK-STAT pathway, outlines its role in autoimmunity, and explains the rationale/pre-clinical evidence for targeting JAK-STAT signaling. The safety and clinical efficacy of the Jakinibs are reviewed, starting with the FDA-approved Jakinib tofacitinib, and continuing on to next-generation Jakinibs. Recent and ongoing studies are emphasized, with a focus on emerging indications for JAK inhibition and novel mechanisms of JAK-STAT signaling blockade. | |
| 28799079 | Protective effects of paeonol on inflammatory response in IL-1β-induced human fibroblast- | 2017 Aug 10 | Various investigations have demonstrated that human fibroblast-like synoviocytes rheumatoid arthritis (HFLS-RA) take part in the chronic inflammatory responses and RA progression. Inhibition of synovium activation and inflammatory processes may represent a therapeutic target to alleviate RA. Paeonol, a major natural product, has many biological and pharmacological activities. However, its protective effects against RA considering HFLS-RA have not been explored. In this study, anti-inflammatory effects of paeonol were detected in interleukin-1β (IL-1β)-treated HFLS-RA. Our results demonstrated that paeonol had no effect on cell survival and IL-1β-induced proliferation in HFLS-RA. Pretreatment with paeonol significantly suppressed the production of pro-inflammatory TNF-α, IL-6 and IL-1β, and the expressions of matrix metalloproteinase-1/-3 in vitro and in vivo. Mice treated with paeonol (10 mg/kg) remarkablely attenuated arthritic symptoms based on clinical arthritis scores and histopathology in collagen-induced arthritis mice. Furthermore, the TLR4 expression and NF-κB p65 activation were inhibited by paeonol in vitro and in vivo. Our findings illustrated that paeonol had significantly suppressed inflammation effects in synovial tissues and RA progression. The potential mechanism might be based on the attenuation TLR4-NF-κB activation. These collective results indicated that paeonol might be a promising therapeutic agent for alleviating RA progress through inhibiting inflammations and NF-κB signalling pathway. | |
| 28770639 | The preventive effects of nanopowdered red ginseng on collagen-induced arthritic mice. | 2018 May | This study was carried out to investigate the efficiency of red ginseng nanopowder in preventing collagen-induced arthritis (CIA) in mice. The mice were divided into five groups: normal group (no immunisation), control (CIA), powdered red ginseng (PRG), nanopowdered red ginseng (NRG) and methotrexate (MTX). Administering MTX, PRG and NRG to arthritic mice significantly decreased spleen indexes, clinical and histological scores compared to control group. Serum analysis of NRG and MTX groups showed a reduction in the cytokines such as the levels of tumour necrosis factor alpha (TNF-α), interleukin 6 (IL-6) and interleukin 1β (IL-1β) in comparison to PRG group. The levels of immunoglobulin M (IgM) and immunoglobulin G(1) (IgG(1)) in the NRG group were significantly lower than those of the PRG group. In summary, the present study indicated that NRG can be effective in preventing type II collagen-induced rheumatoid arthritis in mice. | |
| 28405158 | Patient experience with intravenous biologic therapies for ankylosing spondylitis, Crohn's | 2017 | OBJECTIVE: The objective of this study was to describe patient experience with intravenous (IV) biologics for ankylosing spondylitis, Crohn's disease, psoriatic arthritis, psoriasis, rheumatoid arthritis, or ulcerative colitis. METHODS: Semi-structured telephone interviews were conducted in 405 patients with these autoimmune diseases who were receiving an IV biologic to treat their disease. RESULTS: On a 7-point scale (1= not at all satisfied; 7= very satisfied), mean satisfaction with IV medication was rated 6.1; 77% of patients rated satisfaction as 6 or 7. The most frequently perceived benefits of IV therapy were related to supervision provided by health care professionals. Most patients (82%, n=332) preferred their IV medication to subcutaneous injection. The three most common reasons for preferring IV were not wanting to self-inject (43%), less frequent dosing (34%), and preference for administration by a health care professional (24%). African-American/black patients had a stronger preference for IV administration than Caucasian/white patients (97% vs 80%, P<0.05) and a greater dislike of needles/self-injection (71% vs 40%, P<0.05). Hospital outpatient departments were not rated as well as physician in-office infusion. Only half (49%) of the patients reported that both they and their physician equally influenced the choice to switch from subcutaneous to IV therapy, and only 30% were given a choice of infusion center. CONCLUSION: Users of IV biologics are highly satisfied with their medications and perceive the opportunity for health care provider interaction at their infusion facilities as an advantage of their regimen. These findings support continued need for IV therapeutic options and shared decision-making between patients and physicians while selecting biologic treatments. | |
| 29282397 | Swan Neck Deformity Mimicking Claw Hand Caused by Arthritis in Leprosy. | 2017 Sep | Swan neck deformity is a hyperextension of the proximal interphalangeal (PIP) joints and flexion of the distal interphalangeal (DIP) joints. Claw hand is a hyperextension of the metacarpal joints and flexion of the PIP joints, accompanied by reduced motor strength. A 23-year-old female, who was released from leprosy treatment, presented with a bend of the second to fifth fingers of both hands. There was hyperextension of the PIP joints and flexion of the DIP joints from the second to fifth fingers of both hands, thickening of the ulnar nerves, and hypoesthesia without motor impairment of the fourth and fifth fingers of both hands. Radiograph examination revealed cupping of the base of the proximal phalangeal joints of the second, third, and fifth fingers of the left hand and of the second and fifth fingers of the right hand. Additionally, narrowing of the metacarpophalangeal joints of the second, third, and fifth fingers of the right hand and sclerosis of the second and fifth fingers of the right hand were also observed. Claw hand is frequently reported in leprosy, while swan neck deformity is frequently reported in rheumatoid arthritis. To our knowledge, this is the first reported case with swan neck deformity caused by arthritis in leprosy. | |
| 28474288 | Could Lymphocyte Profiling be Useful to Diagnose Systemic Autoimmune Diseases? | 2017 Oct | Considering the implications of B, T, and natural killer (NK) cells in the pathophysiology of systemic autoimmune diseases, the assessment of their distribution in the blood could be helpful for physicians in the complex process of determining a precise diagnosis. In primary Sjögren's syndrome, transitional and active naive B cells are increased and memory B cells are decreased compared to healthy controls and other systemic diseases. However, their utility to improve the accuracy of classification criteria has not been proven. In early untreated rheumatoid arthritis, proportions of regulatory T cells are constantly reduced, but other patterns are difficult to determine given the heterogeneity of published studies. In systemic lupus erythematosus, the lack of studies using large cohorts of patients and the diversity of the possible pathological mechanisms involved are also important impediments. Nevertheless, transitional B cell and plasma cell proportions are increased in most of the studies, the CD4/CD8 ratio is decreased, and the number of NK cells is reduced. Despite the low number of studies, anomalies of lymphocyte subset distribution was also described in ANCA-associated vasculitis, systemic scleroderma, and myositis. For now, flow cytometric analysis of lymphocyte subsets has focused mainly on specific subpopulations and is more useful for basic and translational research than for diagnostics in clinical practice. However, new modern methods such as mass cytometry and bioinformatics analyses may offer the possibility to simultaneously account for the relative proportions of multiple lymphocyte subsets and define a global profile in homogeneous groups of patients. The years to come will certainly incorporate such global lymphocyte profiling in reclassification of systemic autoimmune diseases. | |
| 29196420 | Fibrillary Glomerulonephritis in Primary Sjogren's Syndrome: A Rare Cause of Renal Failure | 2017 Dec | Renal involvement in primary Sjogren's syndrome (pSS) varies in severity and prevalence. Although previously felt to be uncommon, kidneys can be involved in 25% to 30% of pSS patients. Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease that can occur in association with another autoimmune condition or malignancy. The diagnosis relies on renal biopsy findings of haphazardly arranged fibrils in all glomerular compartments and distinction from other forms of fibrillary glomerulopathies such as renal amyloidosis and immunotactoid glomerulopathy. FGN responds poorly to immunosuppressive therapy and has a poor prognosis. Here, we describe a case of FGN in a patient with asymptomatic pSS. We describe the diagnostic work-up, clinical course, treatment utilized, and 1-year follow-up. There is one other case in the literature of FGN in a patient with pSS. The rarity of this association and distinction of FGN from other forms of renal involvement in pSS is important as it impacts therapy and prognosis. The case highlights electron microscopy findings in FGN and poor prognosis. |