Search for: rheumatoid arthritis methotrexate autoimmune disease biomarker gene expression GWAS HLA genes non-HLA genes
ID | PMID | Title | PublicationDate | abstract |
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32760888 | The improvement in aerobic capacity, disease activity, and function in patients with rheum | 2020 Jun | OBJECTIVES: This study aims to evaluate the effect of cardiac rehabilitation (CR) on disease activity, functional status, fitness, and modified cardiovascular disease (CVD) risk factors in patients with rheumatoid arthritis (RA) with a moderate disease activity. PATIENTS AND METHODS: This single-center, controlled study included a total of 60 female RA patients (mean age 57.5 years; range, 50 to 64 years) with moderate disease activity according to the Disease Activity Score-28 (DAS28) between January 2014 and June 2015. The patients were divided into two equal groups as those receiving CR program (n=30) and those receiving home exercise program (HEP; n=30). The patients were evaluated at baseline, at Weeks 10 and 24 using exercise tolerance test (i.e., The Metabolic Equivalent of Task [MET] and maximal oxygen uptake [VO2 max]), Six-Minute Walk Test (6MWT), DAS28, Health Assessment Questionnaire (HAQ), modified Systematic Coronary Risk Evaluation (mSCORE), Short Form Health Survey-36 (SF-36), and Beck Depression Inventory (BDI). RESULTS: There was a significant improvement in the VO2 max (p<0.001), MET (p<0.001), DAS28 (p<0.001), HAQ (p<0.001), BDI (p=0.005), SF-36 physical function (p=0.039), pain (p<0.001) and vitality subscale scores (p=0.008), and 6MWT (p<0.001), after the initial and repetitive exercise programs in the CR group compared to the HEP group. At the end of Week 24, full compliance with HEP was higher in the patients with CR group, compared to the HEP group (p<0.001). There was no significant effect of supervised exercise program on the mSCORE, although systolic blood pressure (p=0.033) and resting heart rate (p=0.049) were significantly improved in the CR group versus HEP group. CONCLUSION: Based on our study results, supervised exercise program cannot reduce CVD risk as assessed by the mSCORE, although it improves physical fitness, disease activity, and functional outcomes which may reduce traditional CVD risk factors in patients with RA. | |
30679916 | Comparative analysis of US real-world dosing patterns and direct infusion-related costs fo | 2019 | PURPOSE: The objectives of this study were to evaluate and compare treatment patterns and infusion-related health care resource expenditures for rheumatoid arthritis (RA) patients initiating golimumab for intravenous use (GLM-IV) and infliximab (IFX) therapy and to assess cost implications from the commercial perspective. METHODS: Adult RA patients with a new episode of GLM-IV or IFX treatment between Janu-ary 1, 2014 and March 31, 2016 were identified from MarketScan databases and evaluated for maintenance infusion intervals and related costs of treatment. IFX and GLM-IV patients were matched 1:1 on index medication treatment duration, gender, payer type, prior biologic use, and post-index methotrexate use. Paid amounts for drugs and associated administration costs were applied to treatment group dosing patterns. RESULTS: Final matched treatment groups included 547 GLM-IV and 547 IFX patients (mean age = 55-56 years). Mean (SD) follow-up was 609 (161) days for GLM-IV and 613 (163) days for IFX. Treatment duration was 396 (240) days for GLM-IV and 397 (239) days for IFX. Overall, 80% of GLM-IV and 39% of IFX maintenance infusions were given approximately every 8 weeks; and 6% of GLM-IV and 53% of IFX maintenance infusions occurred more frequently than every 8 weeks (P<0.001). When weighting of the maintenance infusion interval was applied, the mean number of induction plus maintenance infusions during the first year of treatment was estimated at 7.03 for GLM-IV and 9.48 for IFX. From the commercial perspective, drug plus administration costs per infusion were $5,846 for GLM-IV and $5,444 for IFX with total annual cost of therapy for GLM-IV patients costing $10,507 less than that for IFX patients in the first year and $6,774 less than that for IFX patients in subsequent years. CONCLUSION: Annual GLM-IV drug plus administration costs for commercial health plans were significantly less than IFX in RA patients due to differences in real-world dosing and administration. | |
31644955 | The gut-eye-lacrimal gland-microbiome axis in Sjögren Syndrome. | 2020 Apr | The bacterial communities that collectively inhabit our body are called the microbiome. Virtually all body surface harbors bacteria. Recent advances in next-generation sequencing that have provided insight into the diversity, composition of bacterial communities, and their interaction are discussed in this review, as well as the current knowledge of how the microbiome promotes ocular health. The ocular surface is a site of low bacterial load. Sjögren Syndrome is an autoimmune disease that affects the exocrine glands, causing dry mouth and dry eye. Systemic antibiotic treatment and germ-free mice have demonstrated that commensal bacteria have a protective role for the ocular surface and lacrimal gland. The existence of a gut-eye-lacrimal gland axis-microbiome is discussed. | |
31376261 | Animal models of Sjögren's syndrome: an update. | 2019 May | Sjögren's syndrome (SS) is a chronic autoimmune disease characterised by lymphocytic infiltration in exocrine glands with secretory dysfunction. Although both environmental triggers and genetic predisposition have been recognised as important factors in the initiation and development of SS, the pathogenesis of SS is complex and still largely unclear. Animal models have served as useful tools for studying SS pathogenesis with several advantages. A number of animal models recapitulating key characteristics of primary SS patients including secretory dysfunction, glandular inflammation and presence of autoantibodies were developed in the past years. The studies based on the animal models of SS have provided significant insight in SS pathogenesis and therapeutic intervention. This review summarises current animal models with primary SS-like symptoms including spontaneous models, genetically modified models, induced models and humanised models, and discusses their contribution to the understanding of SS aetiology and therapies. | |
31539922 | Spontaneous Model of Sjögren's Syndrome in NOD Mice. | 2019 Sep | The non-obese diabetic (NOD) mouse model is the most widely described and validated method for investigating human primary Sjögren's syndrome (SS) and represents a useful model for translational studies. However, the systemic disease manifestation in NOD mice is sensitive to the housing environment, as stress modulates the immune system, so it is essential to confirm that readouts are robust, reproducible, and sensitive to known clinical treatments. This protocol describes the establishment of the spontaneous NOD model of SS and underscores the necessity of model validation to ensure that the housing environment is compatible. © 2019 by John Wiley & Sons, Inc. | |
31509198 | Parent Pain Cognitions and Treatment Adherence in Juvenile Idiopathic Arthritis. | 2019 Oct 1 | OBJECTIVE: Given the high levels of pain and low rates of treatment adherence in children with juvenile idiopathic arthritis (JIA) and their families, this study sought to examine the relationship between parent pain cognitions (i.e., pain catastrophizing, fear of pain) and treatment adherence, and how barriers to treatment (e.g., forgetting treatments, children resisting injections) may be implicated in this relationship. METHODS: Parents of children under 18 years of age who have been diagnosed with JIA were recruited to complete an online survey. In total, 221 parents (93% mothers) of children aged 2-17 years (M = 11.10, SD = 4.25) took part, completing questions regarding their pain cognitions, perceived barriers to treatment, and their child's arthritis treatment adherence ability. RESULTS: Hierarchical regressions demonstrated that both pain cognitions (i.e., pain catastrophizing and fear of pain) were related to a decrease in parent-reported treatment adherence, however, pain catastrophizing was no longer significant when fear of pain was added to the model. The presence of treatment barriers partially mediated the relationship between fear of pain and treatment adherence, above and beyond the alternate model proposed. CONCLUSION: These results suggest that parent pain catastrophizing and fears of pain are related to a greater difficulty following treatment plans, possibly in part because of barriers parents experience that preclude adherence. Given these findings, the identification and management of parent pain cognitions is critical to improving treatment adherence and outcomes for children with JIA and their families. | |
31862360 | Synergistic effect of all-trans-retinal and triptolide encapsulated in an inflammation-tar | 2020 Mar 10 | Targeted delivery of nano-encapsulated anti-inflammatory agent represents a promising while challenging strategy in the treatment of rheumatoid arthritis (RA). Pro-inflammatory macrophages play a major role in the pathogenesis of RA. In this study, we investigated the effect of a macrophage-targeted pH-sensitive nanoparticle on collagen-induced arthritis (CIA) in mice. To target macrophage, all-trans-retinal was conjugated into dextran backbone through pH-sensitive hydrazone bond, then grafted with galactose (GDR). This nanoparticle was used for the encapsulation of triptolide (TPT), a potent anti-inflammatory compound isolated from Chinese herb. As expected, GDR nanoparticles preferentially accumulated in the inflammatory tissues. Treatment with GDR-TPT nanoparticles resulted in a marked decrease in the infiltration of CD3(+) T cells and F4/80(+) macrophages and reduction of the expression of TNF-α, IL-6 and IL-1β in the inflamed lesions of CIA mice. Furthermore, Th1 and Th17 responses were also inhibited. Importantly, anti-arthritic effect of TPT was markedly enhanced while its toxic effect was attenuated by encapsulating with GDR. GDR by itself also had moderate effect in the inhibition of arthritis, due to its intrinsic anti-inflammatory property. Therefore, our results clearly show that GDR-TPT nanoparticle may represent a promising drug delivery system for the treatment of RA. | |
32122171 | Factors Associated With Reoperation After Silicone Metacarpophalangeal Joint Arthroplasty | 2020 Nov | Background: Silicone metacarpophalangeal (MCP) joint arthroplasty has a high revision rate. It has been suggested that the preoperative degree of ulnar drift and radial wrist deviation influences the durability of MCP silicone arthroplasties. The goal of this study was to evaluate what factors are associated with reoperation after silicone MCP arthroplasty. Materials and Methods: We retrospectively evaluated all adult patients who underwent MCP silicone arthroplasty between 2002 and 2016 at one institutional system for inflammatory arthritis. After manual chart review, we included 73 patients who underwent 252 arthroplasties. Fingers treated included 66 index, 67 long, 60 ring, and 59 small fingers. Results: The overall reoperation rate was 9.1% (N = 23). Indications for reoperation were implant breakage (n = 11), instability (n = 4), soft tissue complications (n = 4), infections (n = 3), and stiffness (n = 1). There was a trend that patients who underwent single-digit arthroplasty had higher rates of revision (19% vs 3.5%, P = .067). Radiographic follow-up demonstrated joint incongruency in 50% of cases, bone erosion in 58% of cases, and implant breakage in 19% of cases. There was a trend toward higher rates of revision in patients without preoperative MCP joint subluxation (19% vs 6.7%, P = .065) The 1-, 5-, and 10-year implant survival rates were 96%, 92%, and 70%, respectively. Revision surgery occurred at <14 months in 15 patients (65%) and after 5 years in 8 (35%) patients. Conclusions: Revision surgery after silicone MCP arthroplasty appears to be bimodal. Patients with greater hand function preoperatively may be at higher risk of revision surgery. | |
30074417 | Combined gemfibrozil (peroxisome proliferator-activated receptor alpha agonist) with reduc | 2019 Jul | Objectives: The study aimed to evaluate the efficacy of combined gemfibrozil with prednisolone in the management of adjuvant-induced arthritis (AIA) rat model. Methods: Seventy two adult male Wistar albino rats were divided equally into 1-control group, three diseased groups: 2- Adjuvant induced arthritis (AIA), 3- high fat diet (HF), and 4- combined AIA-HF, and treated groups: 5- gemfibrozil 30 mg/kg treated AIA group (AIA-G) and the combined AIA-HF treated groups: 6- prednisolone equivalent to human 10 mg (AIA-HF-P10), 7- prednisolone equivalent to human 5 mg (AIA-HF-P5) 8- gemfibrozil (HF-AIA-G) and 9- combined treatment (HF-AIA-G-P5) Results: HF diet represents a precipitating factor for knee arthritis. Gemfibrozil improved the inflammatory findings in both AIA and AIA-HF groups. Combined administration of gemfibrozil with reduced steroid dose gave a similar therapeutic effect to the full steroid dose. Fortunately, we reported more reduction in the nuclear factor kappa-B (NF-κB) and high mobility group box 1 (HMGB1) in the HF-AIA-G-P5 compared with the HF-AIA-P10 group. The improvement was proved by the histological findings. Conclusion: Combined reduced prednisolone dose with gemfibrozil potentiates its anti-inflammatory activity. This could be a target in the management of rheumatoid arthritis. | |
31562547 | [Physical activity, exercise and nutrition in rheumatism : Adjuvant treatment options for | 2019 Nov | BACKGROUND AND OBJECTIVES: The positive effects of physical activity, physical training and an adaptation of diet on health have been scientifically proven for many diseases. Focusing on inflammatory rheumatic diseases and their potential comorbidities, positive effects are assumed from these two adjuvant treatment opportunities, which are examined in more detail in this review. MATERIAL AND METHODS: Based on a literature search, randomized controlled trials (RCTs), non-RCT studies, reviews and recommendations from professional societies were included. RESULTS: Physical activity and training show positive effects on the disease itself and also on its comorbidities with existing certainty. In addition, the exercise and training recommendations of the American College of Sports Medicine (ACSM) and the American Heart Association (AHA) provide recommendations, which were adapted by the European League Against Rheumatism (EULAR) to control intensity, duration and training extent. Nutritional medical approaches also provide preventive and rehabilitative beneficial possibilities. DISCUSSION: The increase of physical activity, regular physical training and the adaptation of diet should be a basic additive component of the treatment of inflammatory rheumatic diseases. In individual sub-aspects, the study situation is very heterogeneous and requires further research. | |
31872173 | Development and validation of a deep-learning model for scoring of radiographic finger joi | 2019 | OBJECTIVE: The purpose of this research was to develop a deep-learning model to assess radiographic finger joint destruction in RA. METHODS: The model comprises two steps: a joint-detection step and a joint-evaluation step. Among 216 radiographs of 108 patients with RA, 186 radiographs were assigned to the training/validation dataset and 30 to the test dataset. In the training/validation dataset, images of PIP joints, the IP joint of the thumb or MCP joints were manually clipped and scored for joint space narrowing (JSN) and bone erosion by clinicians, and then these images were augmented. As a result, 11Â 160 images were used to train and validate a deep convolutional neural network for joint evaluation. Three thousand seven hundred and twenty selected images were used to train machine learning for joint detection. These steps were combined as the assessment model for radiographic finger joint destruction. Performance of the model was examined using the test dataset, which was not included in the training/validation process, by comparing the scores assigned by the model and clinicians. RESULTS: The model detected PIP joints, the IP joint of the thumb and MCP joints with a sensitivity of 95.3% and assigned scores for JSN and erosion. Accuracy (percentage of exact agreement) reached 49.3-65.4% for JSN and 70.6-74.1% for erosion. The correlation coefficient between scores by the model and clinicians per image was 0.72-0.88 for JSN and 0.54-0.75 for erosion. CONCLUSION: Image processing with the trained convolutional neural network model is promising to assess radiographs in RA. | |
30941136 | Cell-Free DNA as a Biomarker in Autoimmune Rheumatic Diseases. | 2019 | Endogenous DNA is primarily found intracellularly in nuclei and mitochondria. However, extracellular, cell-free (cf) DNA, has been observed in several pathological conditions, including autoimmune diseases, prompting the interest of developing cfDNA as a potential biomarker. There is an upsurge in studies considering cfDNA to stratify patients, monitor the treatment response and predict disease progression, thus evaluating the prognostic potential of cfDNA for autoimmune diseases. Since the discovery of elevated cfDNA levels in lupus patients in the 1960s, cfDNA research in autoimmune diseases has mainly focused on the overall quantification of cfDNA and the association with disease activity. However, with recent technological advancements, including genomic and methylomic sequencing, qualitative changes in cfDNA are being explored in autoimmune diseases, similar to the ones used in molecular profiling of cfDNA in cancer patients. Further, the intracellular origin, e.g., if derived from mitochondrial or nuclear source, as well as the complexing with carrier molecules, including LL-37 and HMGB1, has emerged as important factors to consider when analyzing the quality and inflammatory potential of cfDNA. The clinical relevance of cfDNA in autoimmune rheumatic diseases is strengthened by mechanistic insights into the biological processes that result in an enhanced release of DNA into the circulation during autoimmune and inflammatory conditions. Prior work have established an important role of accelerated apoptosis and impaired clearance in leakage of nucleic acids into the extracellular environment. Findings from more recent studies, including our own investigations, have demonstrated that NETosis, a neutrophil cell death process, can result in a selective extrusion of inflammatory mitochondrial DNA; a process which is enhanced in patients with lupus and rheumatoid arthritis. In this review, we will summarize the evolution of cfDNA, both nuclear and mitochondrial DNA, as biomarkers for autoimmune rheumatic diseases and discuss limitations, challenges and implications to establish cfDNA as a biomarker for clinical use. This review will also highlight recent advancements in mechanistic studies demonstrating mitochondrial DNA as a central component of cfDNA in autoimmune rheumatic diseases. | |
31635367 | Active Cellular and Subcellular Targeting of Nanoparticles for Drug Delivery. | 2019 Oct 18 | Nanoparticle (NP)-mediated drug delivery (NMDD) for active targeting of diseases is a primary goal of nanomedicine. NPs have much to offer in overcoming the limitations of traditional drug delivery approaches, including off-target drug toxicity and the need for the administration of repetitive doses. In the last decade, one of the main foci in NMDD has been the realization of NP-mediated drug formulations for active targeted delivery to diseased tissues, with an emphasis on cellular and subcellular targeting. Advances on this front have included the intricate design of targeted NP-drug constructs to navigate through biological barriers, overcome multidrug resistance (MDR), decrease side effects, and improve overall drug efficacy. In this review, we survey advancements in NP-mediated drug targeting over the last five years, highlighting how various NP-drug constructs have been designed to achieve active targeted delivery and improved therapeutic outcomes for critical diseases including cancer, rheumatoid arthritis, and Alzheimer's disease. We conclude with a survey of the current clinical trial landscape for active targeted NP-drug delivery and how we envision this field will progress in the near future. | |
31581724 | Using the Immunophenotype to Predict Response to Biologic Drugs in Rheumatoid Arthritis. | 2019 Oct 2 | Tumour necrosis factor (TNF)-α is a key mediator of inflammation in rheumatoid arthritis, and its discovery led to the development of highly successful anti-TNF therapy. Subsequently, other biologic drugs targeting immune pathways, namely interleukin-6 blockade, B cell depletion, and T cell co-stimulation blockade, have been developed. Not all patients respond to a biologic drug, leading to a knowledge gap between biologic therapies available and the confident prediction of response. So far, genetic studies have failed to uncover clinically informative biomarkers to predict response. Given that the targets of biologics are immune pathways, immunological study has become all the more pertinent. Furthermore, advances in single-cell technology have enabled the characterization of many leucocyte subsets. Studying the blood immunophenotype may therefore, define biomarker profiles relevant to each individual patient's disease and treatment outcome. This review summarises our current understanding of how immune biomarkers might be able to predict treatment response to biologic drugs. | |
31893271 | What are the risk factors of poor medication adherence in the target-to-treat era? | 2019 Jun | OBJECTIVES: This study aims to identify adherence rate and risk factors of poor adherence in patients with tightly controlled rheumatoid arthritis (RA) based on the treat-to-target (TTT) strategy. PATIENTS AND METHODS: In this cross-sectional, observational study, a total of 103 patients (22 males, 81 females; mean age 58.6±9.5 years; range, 35 to 76 years) with tightly controlled RA between November 2016 and May 2017 were included. The patients were evaluated in terms of sociodemographic features, smoking and alcohol drinking status, body mass index (BMI), Disease Activity Score 28 (DAS28), and clinical and medication data. They filled out a series of standardized questionnaires including the Morisky 8-item Medication Adherence Scale (MMAS-8), Beck Depression Inventory (BDI), Mini-Mental State Examination (MMSE), and Health Assessment Questionnaire-Disability Index (HAQ-DI). Multiple multivariate linear regression analysis was used to identify variables which were possibly associated with the MMAS-8. RESULTS: Of the patients, 53 (51.5%) were non-adherent and 50 (48.5%) were adherent to medication. The DAS28-erythrocyte sedimentation rate, mean DAS28, HAQ, BDI scores, and the number of visits were higher and the MMSE scores were lower in non-adherent patients than adherent patients. In the linear multivariate analysis, significant associations were found between the MMAS-8 and MMSE, BDI, DAS28, and mean DAS28 scores. CONCLUSION: Our study results show that the medication adherence rate is significantly higher compared to previous studies and high disease activity, depression, and cognitive dysfunction significantly affect medication adherence in this patient population. | |
31213775 | 15-Deoxy-Δ(12,14)-prostaglandin J(2) as a potential regulator of bone metabolism via PPAR | 2019 | Bone metabolism is a complex physiological process that primarily involves osteoblast-mediated bone formation and osteoclast-mediated bone resorption, both of which are regulated by a variety of biological factors. There is increasing evidence that peroxisome proliferator-activated receptor γ (PPARγ) is a member of the nuclear receptor superfamily and plays an important role in lipid metabolism and bone metabolism. Through the PPARγ-dependent pathway, 15-deoxy-Δ(12,14)-prostaglandin J(2) (15d-PGJ(2)) promotes the formation of marrow adipocytes and inhibits the formation of osteoblasts, resulting in bone loss and increasing the risk of fracture and osteoporosis. Recent studies have found that through the PPARγ-independent pathway, 15d-PGJ(2) plays a regulatory role in bone metastasis of breast cancer, which can inhibit osteoclastogenesis and reduce bone destruction. The purpose of our review is to summarize the recent progress in elucidating the mechanisms and effects of 15d-PGJ(2) in bone metabolism, which can serve as a novel therapeutic target for bone tumors, osteoporosis, rheumatoid arthritis (RA), and other bone diseases. | |
30853311 | Immunological effects of vitamin D and their relations to autoimmunity. | 2019 Jun | Vitamin D deficiency is an established risk factor for many autoimmune diseases and the anti-inflammatory properties of vitamin D underscore its potential therapeutic value for these diseases. However, results of vitamin D3 supplementation clinical trials have been varied. To understand the clinical heterogeneity, we reviewed the pre-clinical data on vitamin D activity in four common autoimmune diseases: multiple sclerosis (MS), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and inflammatory bowel disease (IBD), in which patients are commonly maintained on oral vitamin D3 supplementation. In contrast, many pre-clinical studies utilize other methods of manipulation (i.e. genetic, injection). Given the many actions of vitamin D3 and data supporting a vitamin D-independent role of the Vitamin D receptor (VDR), a more detailed mechanistic understanding of vitamin D3 activity is needed to properly translate pre-clinical findings into the clinic. Therefore, we assessed studies based on route of vitamin D3 administration, and identified where discrepancies in results exist and where more research is needed to establish the benefit of vitamin D supplementation. | |
31853152 | Observational study of inflammatory arthritis treatment by etanercept originator switched | 2019 | OBJECTIVES: The aim of the study was to assess the safety and efficacy of switching an etanercept originator to an etanercept biosimilar in rheumatoid arthritis, juvenile idiopathic arthritis, psoriatic arthritis, and ankylosing spondylitis patients. MATERIAL AND METHODS: In 162 patients etanercept originator treatment had been replaced with the biosimilar (Group 1), and in six patients the biosimilar was initiated as the first biological agent (Group 2). The efficacy and safety of the treatment were monitored at 3-6 months. RESULTS: In the majority of patients in Group 1 (n = 138) the etanercept biosimilar was well tolerated, whereas in 24 patients a switch back to the originator was required. The loss of efficacy was confirmed in nine patients using clinical scoring system, and nine patients reported subjective loss of efficacy; 13 patients reported adverse events, most often headache (n = 3) and skin lesions (n = 3). In four patients injection site reactions were present. The adverse events (AE) and/or the loss of the biosimilar efficacy were more commonly observed in women, patients with rheumatoid arthritis (especially in those who did not receive methotrexate), and in patients with a previous history of any other biological treatment. In patients in Group 2 the therapy was effective and no adverse events were observed. CONCLUSIONS: The etanercept biosimilar seems to be effective and well-tolerated in the majority of patients. Nevertheless, in some cases, switching from the originator to the biosimilar was associated with AEs or loss of efficacy. | |
31662815 | Correction to: The Effect of Auricular Plaster Therapy on Insomnia of Patients with Rheuma | 2019 Oct 1 | [This corrects the article DOI: 10.1089/acu.2019.1346.]. | |
31249572 | Pulmonary Manifestations of Primary Sjögren's Syndrome: Underlying Immunological Mechanis | 2019 | Pulmonary involvement in primary Sjögren's syndrome (pSS) is an understudied entity with important clinical implications. Its prevalence has been reported in up to 20% of pSS patients. Pulmonary manifestations of pSS are diverse with involvement of airway and/or lung parenchyma. Histopathology of lung lesions suggests a predominance of submucosal mononuclear cell infiltration consisting predominantly of CD4+ T cells. Current understanding of the pathophysiology of lung disease in pSS suggests a similar process driving the pulmonary process as those in the salivary glands, with epithelial cells playing a critical role in the initiation, maintenance, and symptomatology of the disease. Clinical manifestations of lung involvement in pSS are as varied as the underlying pathology and can be vague and non-specific, thus delaying diagnosis. Management options depend on the underlying pathology but are generally limited due to lack of systematic randomized controlled trials. This review helps summarize our current understanding of lung involvement in pSS. |