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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30547812 | Off-label use of tocilizumab to treat non-juvenile idiopathic arthritis in pediatric rheum | 2018 Dec 14 | Tocilizumab, an anti-interleukin-6 (IL-6) agent, is indicated as a treatment for several autoimmune or inflammatory diseases, including rheumatoid arthritis and juvenile idiopathic arthritis (JIA). IL-6 plays roles in both immune system dysregulation and inflammation, and thus efforts to extend the utility of tocilizumab in patients with autoinflammatory conditions are ongoing. Here, we survey the literature on the off-label use of tocilizumab in patients with juvenile-onset rheumatic diseases including juvenile systemic lupus erythematosus (SLE), juvenile dermatomyositis (DM), vasculitis, juvenile scleroderma, and other autoinflammatory diseases. There is no real evidence that tocilizumab is useful for patients with SLE and juvenile DM, but several cases of childhood Takayasu arteritis have experienced promising outcomes. In juvenile-onset scleroderma, for which no therapy that can halt disease progression is available, tocilizumab may stop progression and the associated functional impairment. Tocilizumab prevents systemic inflammation in patients with Kawasaki's disease, but may develop coronary aneurysms. Tocilizumab has been used to treat several pediatric autoinflammatory diseases, including JIA-associated uveitis and Castleman's disease. Further work in larger populations is necessary to confirm the effects of tocilizumab in patients with pediatric rheumatic diseases. | |
30385646 | Expanding the phenotype of COPA syndrome: a kindred with typical and atypical features. | 2019 Nov | BACKGROUND: Copa syndrome is a rare autosomal dominant disorder with abnormal intracellular vesicle trafficking. The objective of this work is to expand the knowledge about this disorder by delineating phenotypic features of an unreported COPA family. METHODS AND RESULTS: A heterozygous missense variant (c.698 G>A, p.Arg233His) in COPA was identified in four members of a three-generation kindred with lung, autoimmune and malignant disease of unknown aetiology. Ages of onset were 56, 26, 16 and 1 year, with earlier age of onset in successive generations. Presenting symptoms were cough and dyspnoea. Findings included small lung cysts, follicular bronchiolitis, interstitial lung disease, neuroendocrine cell hyperplasia, rheumatoid arthritis, avascular necrosis and select abnormal autoimmune serologies. Neither alveolar haemorrhage nor glomerular disease were present. Features not previously associated with Copa syndrome included neuromyelitis optica, pulmonary carcinoid tumour, clear cell renal carcinoma, renal cysts, hepatic cysts, nephrolithiasis, pyelonephritis and meningitis. Longitudinal evaluations demonstrated slow progression of lung disease and extrapulmonary cysts. CONCLUSIONS: Worsening severity with successive generations may be observed in Copa syndrome. Extrapulmonary cysts, malignancies, autoimmune neurological disorders and infections are clinical features that may be associated with Copa syndrome. Further studies are indicated to fully define the phenotypic spectrum of this disorder. | |
29720243 | Fcγ receptor-mediated influx of S100A8/A9-producing neutrophils as inducer of bone erosio | 2018 May 2 | BACKGROUND: Osteoclast-mediated bone erosion is a central feature of rheumatoid arthritis (RA). Immune complexes, present in a large percentage of patients, bind to Fcγ receptors (FcγRs), thereby modulating the activity of immune cells. In this study, we investigated the contribution of FcγRs, and FcγRIV in particular, during antigen-induced arthritis (AIA). METHODS: AIA was induced in knee joints of wild-type (WT), FcγRI,II,III(-/-), and FcγRI,II,III,IV(-/-) mice. Bone destruction, numbers of tartrate-resistant acid phosphatase-positive (TRAP(+)) osteoclasts, and inflammation were evaluated using histology; expression of the macrophage marker F4/80, neutrophil marker NIMPR14, and alarmin S100A8 was evaluated using immunohistochemistry. The percentage of osteoclast precursors in the bone marrow was determined using flow cytometry. In vitro osteoclastogenesis was evaluated with TRAP staining, and gene expression was assessed using real-time PCR. RESULTS: FcγRI,II,III,IV(-/-) mice showed decreased bone erosion compared with WT mice during AIA, whereas both the humoral and cellular immune responses against methylated bovine serum albumin were not impaired in FcγRI,II,III,IV(-/-) mice. The percentage of osteoclast precursors in the bone marrow of arthritic mice and their ability to differentiate into osteoclasts in vitro were comparable between FcγRI,II,III,IV(-/-) and WT mice. In line with these observations, numbers of TRAP(+) osteoclasts on the bone surface during AIA were comparable between the two groups. Inflammation, a process that strongly activates osteoclast activity, was reduced in FcγRI,II,III,IV(-/-) mice, and of note, mainly decreased numbers of neutrophils were present in the joint. In contrast to FcγRI,II,III,IV(-/-) mice, AIA induction in knee joints of FcγRI,II,III(-/-) mice resulted in increased bone erosion, inflammation, and numbers of neutrophils, suggesting a crucial role for FcγRIV in the joint pathology by the recruitment of neutrophils. Finally, significant correlations were found between bone erosion and the number of neutrophils present in the joint as well as between bone erosion and the number of S100A8-positive cells, with S100A8 being an alarmin strongly produced by neutrophils that stimulates osteoclast resorbing activity. CONCLUSIONS: FcγRs play a crucial role in the development of bone erosion during AIA by inducing inflammation. In particular, FcγRIV mediates bone erosion in AIA by inducing the influx of S100A8/A9-producing neutrophils into the arthritic joint. | |
28556555 | Examination of Hydroxychloroquine Use and Hemolytic Anemia in G6PDH-Deficient Patients. | 2018 Mar | OBJECTIVE: Some sources urge caution when prescribing hydroxychloroquine (HCQ) to patients with G6PDH deficiency, presumably due to a risk of hemolytic anemia. There are limited published data, however, to support this risk. Additionally, not all patients with G6PDH deficiency are at similar risk for hemolysis, and people with the African variant are at particularly low risk. Through a retrospective chart review, we aimed to quantify the frequency of G6PDH-deficient patients with hemolysis attributed to HCQ. METHODS: We identified Duke University Medical Center rheumatology patients with HCQ use and a measured G6PDH level. A retrospective chart review was performed, recording demographics, G6PDH levels, episodes of anemia, laboratory values consistent with hemolysis, and HCQ use. RESULTS: Of the 275 patients reviewed, 84% were female; 46% were African American and 48% were white. The leading diagnoses were systemic lupus erythematosus (32%), rheumatoid arthritis (29%), and inflammatory arthritis (14%). Only 4% of patients were G6PDH deficient (all African American). Two G6PDH-deficient patients had hemolysis during severe lupus flares that occurred while not taking HCQ. There were no reported episodes of hemolysis in more than 700 months of HCQ exposure among the 11 G6PDH-deficient patients. CONCLUSION: This is the largest study to date evaluating G6PDH deficiency with concurrent use of HCQ. Of 11 patients with G6PDH deficiency, 2 had episodes of hemolysis, but these did not occur during HCQ therapy. These data do not support routine measurement of G6PDH levels or withholding HCQ therapy among African American patients with G6PDH deficiency. | |
30829171 | [Pain and fatigue - a systematic review]. | 2018 Dec | Pain and fatigue - a systematic review Introduction: Chronic pain and fatigue are symptoms that often occur together and characterize the symptom picture of various diseases. Nevertheless, integrative explanatory approaches have so far received little attention in medical practice. METHODS: Based on a systematic literature search in the Embase, Medline, PsychInfo, and CENTRAL databases, we searched for high-quality intervention studies for the simultaneous treatment of pain and fatigue. RESULTS: From 1,496 total hits, 158 studies were included in the evaluation. The most commonly studied clinical pictures of the symptomcomplex were tumor catabolism, fibromyalgia, chronic fatigue, rheumatoid arthritis, and osteoarthritis.Current explanations of the symptom complex focus on the activation of proinflammatory microglia in centralized pain syndrome with multiple effects on neurotransmission, neuroendocrinology, neuroplasticity, and the autonomic nervous system. In this model, fatigue can be understood as an evolutionary, meaningful symptom protective of the organism. CONCLUSIONS: A deeper understanding of the relationship between centralized pain syndrome and fatigue allows for new explanatory and treatment approaches. | |
27277133 | Diagnoses and Management of Drug Hypersensitivity and Anaphylaxis in Cancer and Chronic In | 2018 Jun | Due to the increase in utilization of chemotherapies and antibodies, drug hypersensitivity reactions have increased dramatically worldwide, preventing the use of first-line therapies and impacting patients' survival and quality of life. Some of the more frequently used medications in cancer include taxanes for ovarian, lung, breast, and prostate cancers. Monoclonal antibodies are used in the treatment of neoplastic, autoimmune, and inflammatory diseases, and their clinical applications are becoming broader. Monoclonal antibody targets include CD20, HER-2, EGFR, IL-6 receptor, TNF-α, CD30, VEGF-A, IgE, and more, and examples of immune-mediated and inflammatory diseases that respond to monoclonal antibodies include rheumatoid arthritis, Crohn's disease, ulcerative colitis, juvenile idiopathic arthritis, psoriasis and psoriatic arthritis, Wegener's granulomatosis, microscopic polyangiitis, ankylosing spondylitis, plaque psoriasis, and asthma. Neoplastic diseases include non-Hodgkin's lymphoma, chronic lymphocytic leukemia, and colorectal, breast, gastric, and lung cancer. The clinical presentation of drug hypersensitivity reactions ranges from mild cutaneous reactions to life-threatening symptoms including anaphylaxis. Rapid drug desensitization (RDD) has become a groundbreaking approach to the management of immediate drug hypersensitivity reactions IgE and non-IgE mediated. It is the only effective procedure that enables sensitized patients to receive the full treatment dose safely, thus representing an important advance in the patients' treatment and prognosis. The aim of this review is to provide an update on hypersensitivity reactions to commonly used monoclonal and taxanes, their clinical presentations, diagnosis, and the use of RDD for their management. | |
29161466 | Treating Early Undifferentiated Arthritis: A Systematic Review and Meta-Analysis of Direct | 2018 Sep | OBJECTIVE: We undertook a systematic review and meta-analysis of direct and indirect trial evidence to evaluate the efficacy of treatments for patients with undifferentiated arthritis (UA). METHODS: We searched 4 electronic databases from inception to January 2016, clinicaltrials.gov, and bibliographies of relevant articles. Two reviewers independently screened and evaluated the studies. The primary outcome was development of rheumatoid arthritis (RA). RESULTS: Nine studies were included. Interventions included methotrexate, abatacept, infliximab, intraarticular or intramuscular glucocorticoids, and radiation synovectomy. Treating patients resulted in lower rates of RA at 12 months compared to placebo or no treatment (odds ratio [OR] 0.49 [95% confidence interval (95% CI) 0.26, 0.90]). From direct meta-analysis, patients treated with methotrexate were less likely to develop RA at 12 months compared to patients treated without methotrexate (OR 0.13 [95% CI 0.03, 0.48]). This difference was no longer significant at 30 or 60 months. From indirect comparisons, most interventions showed decreased risk of developing RA compared to placebo at 12 months, reaching statistical significance for methotrexate (OR 0.16 [95% CI 0.08, 0.33]) and intramuscular methylprednisolone (OR 0.72 [95% CI 0.53, 0.99]). Most individual interventions included a limited number of studies. CONCLUSION: Treating patients with UA resulted in a statistically significant delay in the development of RA, with the largest effect observed for methotrexate. These findings suggest that there is a window of opportunity to treat patients with UA early, to delay subsequent progression to RA. | |
30719247 | The Effect of Orally Administered Probiotics on the Behavioral, Cellular, and Molecular As | 2018 Sep | INTRODUCTION: Rheumatoid Arthritis (RA) is a chronic autoimmune disease, which is accompanied with pain, hyperalgesia, and edema. Overproduction of pro-inflammatory cytokines and activation of intracellular signaling pathways sustain the RA symptoms considerably. There is a strong correlation between the expression of cytokines and opioid receptors in the arthritis process. Studies have shown that probiotics via different pathways such as reducing the levels of pro-inflammatory cytokines can alleviate inflammatory symptoms. Therefore, based on the crucial role of cellular and humoral immunity in induction of RA symptoms and potency of probiotics in modulation of immune responses, the purpose of this study was to investigate the effect of orally administered probiotics on the behavioral, cellular and molecular aspects of adjuvant-induced arthritis in male Wistar rats. METHODS: Complete Freund's Adjuvant (CFA)-induced arthritis was caused by single subcutaneous injection of CFA into the rat's hind paw on day 0. Different doses of probiotics (1/250, 1/500 and 1/1000 [10(9) CFU/g]) were administered daily (gavage) after CFA injection. Hyperalgesia, edema, serum IL-1β levels, μ-Opioid Receptor (MOR) expression, and p38MAPK (Mitogen-Activated Protein Kinase) activities were assessed on days 0, 7, 14 and 21 of the study. RESULTS: The results of this study indicated the efficacy of probiotics in reducing hyperalgesia, edema, serum levels of Interleukin-1β, and p38MAPK pathway activity during different phases of arthritis as well as increasing the expression of MORs during chronic phase of CFA-induced arthritis. CONCLUSION: It seems that probiotics can effectively reduce inflammatory symptoms by inhibiting the intracellular signaling pathway and cytokine production. | |
30589451 | Aberrant IL-35 levels in patients with primary Sjogren's syndrome. | 2018 Nov | OBJECTIVE: IL-35 is a newly discovered immunoregulatory cytokine that possesses the ability to inhibit CD4Â +Â effector T cells and alleviate autoimmune diseases. The objective of this study was to investigate IL-35 levels in patients with primary Sjogren's syndrome (pSS) and explore the roles of IL-35 in the pathogenesis of pSS. METHODS: Thirty-four hospitalized patients with pSS were recruited, and 34 volunteers were enrolled as healthy controls. An ELISA was adopted to measure plasma IL-35 levels. The levels of P35 and EBI3 mRNAs in peripheral blood mononuclear cells (PBMCs) were determined using real-time quantitative PCR. The percentage of CD4Â +Â EBI3Â +Â T cells and CD19Â +Â EBI3Â +Â B cells was analysed using flow cytometry. Correlations between IL-35 levels, P35 and EBI3 mRNAs, numbers of CD4Â +Â EBI3Â +Â T cells, CD19Â +Â EBI3Â +Â B cells and clinical parameters were analysed. RESULTS: Significantly lower plasma IL-35 levels, P35 and EBI3 mRNA levels, and percentages of CD4Â +Â EBI3Â +Â T cells but increased percentages of CD19Â +Â EBI3Â +Â B cells were observed in patients with pSS than in healthy controls. IL-35 levels, EBI3 mRNA expression and the percentage of CD4Â +Â EBI3Â +Â T cells exhibited negative correlations with the ESSDAI score, whereas levels of the IL-35 protein and EBI3 mRNA were negatively correlated with the ESR. Patients who were positive for anti-SSB antibodies presented with lower IL-35 levels and percentages of CD4Â +Â EBI3Â +Â T cells. CONCLUSIONS: Based on these results, a decrease in the IL-35 levels may play an important role in the pathogenesis of pSS. IL-35 may act as a potential therapeutic agent against inflammation in patients with pSS. | |
28664835 | Prevalence and significance of anti-saccharomyces cerevisiae antibodies in primary Sjögre | 2018 May | OBJECTIVES: Saccharomyces cerevisiae is a common yeast used in the food industry. IgG and IgA antibodies against the phosphopeptidomannan of the S. cerevisiae cell wall (ASCA) are a well known marker of disease severity in Crohn's disease. Moreover, a number of studies assessed ASCA in several systemic and organ-specific autoimmune diseases postulating molecular mimicry as a possible link between ASCA and autoimmunity. However, since they have never been tested in primary Sjögren's syndrome (pSS), the purpose of this study was to investigate these antibodies in a large cohort of pSS patients, compared to healthy donors (HD), and their significance as potentially helpful biomarker in a clinical setting. METHODS: ASCA IgG+IgA were assessed with ASCA screen dot for Blue Diver instrument (Alphadia sa/nv, Belgium). The comparison between the aminoacid sequence of mannan of S. cerevisiae and well characterised auto-antigens peculiar to pSS (52kD and 60kD Ro/SSA, La/SSB) was performed with the Basic Local Alignment Search Tool (BLAST). RESULTS: The prevalence of ASCA in our pSS cohort was 4.8%. We also reported that the ASCA target protein has a high similarity with Ro60/SSA protein further supporting the molecular mimicry hypothesis. Finally, we observed that ASCA positivity is associated with pSS specific clinical and serological features. ASCA+ pSS patients displayed a triple combination of circulating anti-Ro52/SSA, anti-Ro60/SSA and anti-La/SSB antibodies, associated with low complement and cutaneous involvement. CONCLUSIONS: Our data suggest a possible pathogenic/prognostic significance of ASCA in pSS. | |
28551822 | Glucocorticoid therapy causes contradictory changes of serum Wnt signaling-related molecul | 2018 Aug | The objective of this study was to investigate the clinical significance of the Wnt/β-catenin signaling pathway in glucocorticoid-induced osteoporosis. A total of 91 patients with systemic autoimmune diseases who received initial glucocorticoid therapy with prednisolone (30-60 mg daily) were prospectively enrolled. We measured serum levels of N-terminal peptide of type I procollagen (P1NP), bone alkaline phosphatase (BAP), tartrate-resistant acid phosphatase isoform 5b (TRACP-5b), N-telopeptide cross-linked type I collagen (NTX), sclerostin, Dickkopf-1 (Dkk-1), and Wnt3a before starting glucocorticoid therapy and every week for 4 weeks after its initiation. The effects of dexamethasone on expression of mRNA and protein of sclerostin and Dkk-1 by cultured normal human osteoblasts (NHOst) were evaluated by RT-PCR and ELISA, respectively. Serum levels of sclerostin and Dkk-1 increased significantly by 1 week of glucocorticoid therapy and then decreased from the second week onward. Serum Wnt3a tended to decrease and serum P1NP showed a significant decrease. However, TRACP-5b was significantly elevated from the first week of treatment onwards. In vitro study, dexamethasone increased Dkk-1 mRNA expression in cultured NHOst, but sclerostin mRNA was not detected. Dexamethasone also increased Dkk-1 protein production by osteoblasts, whereas sclerostin protein was not detected. Bone formation might be impaired at least in the first week of the initiation of glucocorticoid therapy by increase of the serum Wnt signaling inhibitors; however, their reductions in the subsequent weeks were contradictory to the maintained suppression of the bone formation markers after glucocorticoid therapy for patients with systemic autoimmune diseases. | |
29434816 | Effectiveness of lentivirus-mediated RNA interference targeting mouse tumor necrosis facto | 2018 Feb | The aim of the present study was to identify the effectiveness of lentivirus-mediated RNA interference (RNAi) targeting mouse tumor necrosis factor-α (TNF-α). RNAi lentivirus was used in vitro to transfect RAW264.7 cells, and the expression of TNF-α, interleukin (IL)-1β and IL-6 mRNAs and TNF-α protein in RAW264.7 cells was measured by reverse transcription quantitative polymerase chain reaction (RT-qPCR) and enzyme-linked immunosorbent assay, respectively. In vivo, mice with collagen-induced arthritis (CIA) were injected intravenously with RNAi lentivirus, and CIA arthritis scores and the serum levels of TNF-α were detected. Additionally, joint tissues were subjected to pathological examination. In the cells, the expression level of TNF-α mRNA in the RNAi lentivirus group was 0.29±0.02, which was significantly lower than that of the lentivirus negative control (0.93±0.01; t=25.4, P<0.001). In the mice, the serum TNF-α level in the RNAi lentivirus group was 249.25±11.22 ng/ml, which was significantly lower than that of the negative control group (381.86±6.28 ng/ml; P<0.05). However, no difference in IL-1α and IL-6 mRNA levels was identified among the groups (t=1.00, P=0.37; t=1.22, P=0.29). The CIA arthritis score in the RNAi lentivirus group was significantly reduced compared with those in the control and negative control groups (P<0.05). Furthermore, the arthritis scores in the RNAi lentivirus and positive control groups continued to decrease for ≥2 weeks, and the serum TNF-α levels in the RNAi lentivirus and positive control groups were 31.58±2.18 and 35.21±2.25 pg/ml, which were significantly lower than those in the negative control group (46.62±3.02 pg/ml; P<0.05). Thus, targeting of the TNF-α gene in mice via lentivirus-mediated RNAi in vitro and in vivo achieved TNF-α gene downregulation, which indicates that lentivirus-mediated RNA interference may be an effective form of gene therapy against rheumatoid arthritis. | |
30701740 | Exudative-constrictive tuberculous pericarditis in combination with arthritis in cardiolog | 2018 Sep 20 | AIM: The goal is to present the possibilities of diagnosis verification, the features of the clinical picture of tuberculous pericarditis in the therapeutic clinic and the results of its treatment. MATERIALS AND METHODS: The paper presents clinical observation and a general analysis of 10 cases of tuberculous pericarditis in patients aged 31-79 (mean age 58.0 ± 15.1 years), 6 women and 4 men. Diagnostic puncture pericardium was performed on two patients, pleural puncture - on three Thoracoscopic biopsy of hilar lymph nodes and lung (n=1), pleura (n=1), supraclavicular lymph node biopsy (n=1). Dyskin test was carried out, as well as sputum examination, multispiral computed tomography, oncological search. RESULTS: A 31-year-old patient with a massive effusion in the pericardial cavity, pleural lesion, arthritis of the left knee joint, whose results of the pericardial effusion and sputum were not diagnosed, tuberculosis was detected only with thoracoscopic biopsy of the lung and intrathoracic lymph nodes; the treatment via prednisolone and subtotal pericardectomy was performed. Among 10 patients with MSCT of the lung, changes were noted in general, but in only one case they were highly specific. Diaskin test is positive in 70%. In the study of punctata, bronchoalveolar flushing, Koch bacteria were not detected; at sputum in microscopy and biological sample BC was detected in two patients. The lymphocytic character of effusion in the pericardium / pleura is noted in 4 out of 5 cases. At a biopsy of lymphonoduses and a lung at 2 patients the picture of a granulomatous inflammation with a caseous necrosis. Pericarditis was predominantly large (from 2 cm and more) effusion, signs of constriction were noted in 50% of patients. CONCLUSION: Tuberculosis is one of the frequent causes of pericarditis in the Moscow therapeutic clinic. The most lymphocytic effusion with fibrin and the development of constriction. The negative results of all laboratory tests for tuberculosis do not exclude a diagnosis, It is necessary to use invasive morphological diagnostics, including thoracoscopic biopsy. | |
30542231 | Rotational Shortening of Collateral Ligament in TKR With Severe Deformity. | 2018 Dec | Instability of the knee joint after total knee replacement (TKR) is one of the most important reasons for revision TKR. Inadequate release or tightening of the collateral ligaments in the knee joint may cause instability and early failure. This study presents a case series study of a new technique for ligament balancing wherein the collateral ligament is detached from its origin and rotated (twisted) around its longitudinal axis to tighten the ligament before the origin is reattached to its original position. The surgical technique for collateral ligament tightening during TKR was performed on 6 patients with a deformed knee caused by osteoarthritis and rheumatoid arthritis. The range of motion, knee society score, and laxity of the patients' knee joint, after 7 months to 13 years of follow-up, were evaluated. The technique was successful, achieving good range of motion and satisfactory stability of the joint. Further evaluation in a larger number of cases and a comparative analysis with different techniques would further support the usefulness of this rotational ligamentoplasty technique. | |
30375403 | Author Correction: Uncovering pharmacological mechanisms of Wu-tou decoction acting on rhe | 2018 Oct 30 | A correction has been published and is appended to both the HTML and PDF versions of this paper. The error has not been fixed in the paper. | |
30344909 | Synthesis and Biological Evaluation of an Indazole-Based Selective Protein Arginine Deimin | 2018 Oct 11 | Protein arginine deiminase 4 (PAD4) is a calcium-dependent enzyme that catalyzes the conversion of arginine to citrulline within target proteins. Dysregulation of PAD4 has been implicated in a number of human diseases, including rheumatoid arthritis and other inflammatory diseases as well as cancer. In this study, we report on the design, synthesis, and evaluation of a new class of haloacetamidine-based compounds as potential PAD4 inhibitors. Specifically, we describe the identification of 4,5,6-trichloroindazole 24 as a highly potent PAD4 inhibitor that displays >10-fold selectivity for PAD4 over PAD3 and >50-fold over PAD1 and PAD2. The efficacy of this compound in cells was determined by measuring the inhibition of PAD4-mediated H4 citrullination in HL-60 granulocytes. | |
30057739 | Management of T-cell large granular lymphocyte leukemia and concurrent retroperitoneal lip | 2018 Jul | T-cell large granular leukemia (T-LGL) is a rare lymphoproliferative disorder characterized by the clonal expansion of cytotoxic T lymphocytes. We present a unique case of T-LGL and concurrent retroperitoneal sarcoma occurring in a patient with long-standing rheumatoid arthritis. Pathology revealed a high-grade dedifferentiated liposarcoma. The diagnosis of T-LGL with a synchronous retroperitoneal sarcoma is a case that highlights the surgical management of these two rare conditions. | |
30051737 | Biologic and small-molecule medications in the management of pyoderma gangrenosum. | 2019 May | Pyoderma gangrenosum (PG) is an uncommon inflammatory skin disorder characterized by neutrophil dysfunction. There are currently no FDA-approved drugs for the treatment of this disease, and treatment has typically relied on traditional immunosuppressive medications such as prednisone or cyclosporine. The efficacy of biologics in the treatment of other pro-inflammatory conditions such as psoriasis, rheumatoid arthritis, and inflammatory bowel disease is well-documented in the literature. Therefore, the use of biologic medications for the treatment of rarer inflammatory skin conditions, such as PG, is a compelling topic for investigation. Biologic and small-molecule therapies allow physicians to target specific pro-inflammatory mediators that underlie PG pathogenesis. This review provides an update on the use of biologic and small-molecule medications for the treatment of PG and summarizes the latest data on the clinical efficacy and pharmacology of these treatments. | |
30568149 | A Rare Presentation of Hypermagnesemia Associated with Acute Kidney Injury due to Hypercal | 2019 Apr 15 | A 79-year-old Japanese woman presented with consciousness disturbance, hypercalcemia, and hypermagnesemia. She had rheumatoid arthritis and osteoporosis. Three months before admission, she was treated with oral calcitriol for osteoporosis. Two months before admission, she was treated with magnesium oxide for constipation. One month before admission, she underwent articular femoral bone replacement. Two weeks postoperatively, consciousness disturbance and elevated serum calcium levels were observed, and she was transferred to our hospital. On admission, her laboratory data showed elevated serum magnesium and creatinine levels. This is a rare case of coexistent hypercalcemia and hypermagnesemia associated with consciousness disturbance and acute kidney injury. | |
29992828 | Emotion regulation contributes to the well-being of patients with autoimmune diseases thro | 2020 Nov | This prospective study aimed to examine whether illness-related negative emotions mediate the relationship of cognitive reappraisal and expressive suppression to the well-being of 99 patients with rheumatoid arthritis or multiple sclerosis. After adjusting for disease and patient-related parameters, only cognitive reappraisal was associated with physical and psychological well-being through emotions. Expressive suppression was associated with psychological well-being only for patients reporting less use of cognitive reappraisal. These results underscore the need for prospective studies that will investigate the long-term impact of emotion regulation on adaptation to chronic illness and the conditions under which this impact takes place. |