Rheumatology and immunology research最新文献

Eosinophilic granulomatosis with polyangiitis(EGPA) is a systemic vasculitis syndrome associated with eosinophilia, which most commonly involves the lung, skin, cardiovascular, gastrointestinal, renal, and peripheral nervous systems (PNS). We report a case of a 48-year-old man presented as obstructive jaundice caused by intra-abdominal mass, and he also had elevated peripheral eosinophils. The pathological features of the mass included vasculitis and eosinophils infiltration. At first he was diagnosed as EGPA and treated by glucocorticoid and cyclophosphamide. The patient did not get complete response after six months and then the repeat biopsy proved that he had non-Hodgkin's lymphoma.

Objectives: Systemic sclerosis (SSc) is a heterogeneous complex autoimmune connective tissue disease with variable presentation as a consequence of multisystem involvement. One of the key features of SSc is Raynaud's phenomenon along with vascular endothelial dysfunction that leads to digital ulcers (DUs). Raynaud's tends to be triggered by decreasing thermal gradient exposure, while stress and smoking also play a role. DUs arising as a consequence of severe Raynaud's and vasculopathy are a major cause of morbidity and disability in SSc. We set out to determine the relationship between smoking, Raynaud's phenomenon, DUs, and skin thickness in our Waikato Systemic Sclerosis cohort.

Methods: The Waikato Systemic Sclerosis (SSc) database was used to extract data. Variables collected included demographics, age of diagnosis, SSc subtypes, age at first non-Raynaud's phenomenon, medications used for treatment of Raynaud's phenomenon or ulcers, and maximal modified Rodnan skin score (mRSS). Raynaud's phenomenon and finger DUs (severity for each over the past week and since diagnosis) and a Scleroderma Health Assessment Questionnaire (SHAQ) visual analog 10 cm scale were collected. The lead rheumatologist completed a physician's assessment of Raynaud's and the disease severity questionnaire.

Results: Of the cohort of 143 patients, 100 patients were eligible to complete the questionnaires. Seventy-five patients returned completed questionnaires. Of these, the majority were female (88%), 52 (69.3%) had limited cutaneous systemic sclerosis (lcSSc), 17 (22.7%) had diffuse cutaneous systemic sclerosis (dcSSc), and 6 (8%) had an overlap syndrome. Thirty-six (48%) had a smoking history (in the time frame of collection of serial data). Mean ± standard deviation (SD) pack-years smoked were 17.11 ± 15.29 years. Thirty-five participants had a history of DUs, with a median of 4 DU (range 1-20). Of 17 patients with dcSSc, 12 (70.6%) had ulcers in comparison with 17 of 52 (32.7%) patients with lcSSc. There was a significant relationship between SSc subtype and the number with ulcers (X² = 10.1, P = 0.007). There was also a significant relationship between physician severity of Raynaud's and presence of ulcers (t = 6.1, P < 0.001), which was not evident between patients' severity of Raynaud's and presence of ulcers (t = 1.9, P = 0.06). On the SHAQ score, smokers had significantly worse Raynaud's phenomenon over the prior week (t = 3.08, P = 0.03) and were more likely to note DUs over the preceding week, although the latter was not statistically significant (t = 1.95, P = 0.055). There was no association between smoking and skin thickness as measured by mRSS (r = 0.23, P = 0.19).

Conclusion: Our study demonstrates that smokers have had worse Raynaud's phenomenon over the past week and they

Inflammatory bowel disease (IBD) is a chronic inflammatory disease primarily affecting the gastrointestinal (GI) tract and other organs. In this article, we provide a comprehensive review of IBD, particularly in the context of enteropathic arthritis and its therapeutic advances. Patients with IBD present with intestinal and extraintestinal manifestations (EIMs). Enteropathic arthritis or arthritis associated with IBD (Crohn's disease [CD] and ulcerative colitis [UC]) is the most common EIM and can involve both peripheral and axial joints with some overlaps. Furthermore, peripheral arthritis can be divided into two subcategories. Due to its varied inflammatory presentations and association with NOD2 mutations, CD can mimic other autoimmune and autoinflammatory diseases. Differential diagnosis should be extended to include another NOD2-associated disease, Yao syndrome. Therapy for IBD entails a myriad of medications and procedures, including various biologics targeting different pathways and Janus kinase (JAK) inhibitors. A better understanding of the therapeutic efficacy and mechanism of each drug aids in proper selection of more effective treatment for IBD and its associated inflammatory arthritis.

Systemic lupus erythematosus (SLE) is a chronic autoimmune and inflammatory disease with multiple organs and systems involved such as the kidney, lung, brain and the hematopoietic system. Although increased knowledge of the disease pathogenesis has improved treatment options, current immunosuppressive therapies have failed to prevent disease relapse in more than half of treated patients. Thus, the cell replacement therapy approach that aims to overcome adverse events of traditional treatment and improve recovery rate of refractory SLE is considered as an alternative treatment option. A large number of animal studies and clinical trials have shown stem cell therapy to be a promising therapeutic approach for the treatment of SLE. Since the first transplantation into human patients, several stem cell types have been applied in this field, including hematopoietic stem cells (HSCs) and mesenchymal stem cells (MSCs). In this review, we overview different cell sources of stem cells and applications of the stem cell therapy for treatment of SLE, as well as the comparison between HSCs transplantation (HSCT) and MSCs transplantation (MSCT).

Objectives: Fibromyalgia symptoms have a significant impact on the quality of life and respond poorly to medications. It has been hypothesized that the use of low-energy pulsed electromagnetic field (PEMF) induces neuroprotective effects that may interfere with pain perception. We explored the efficacy of PEMF in patients affected by fibromyalgia.

Methods: Twenty-one females (median age 59 years, interquartile range [IQR] 16.5) affected by fibromyalgia were randomized to receive pulsed electromagnetic field-triple energy pain treatment (PEMF-TEPT) or placebo at T0 and at 4 weeks and 8 weeks. Fibromyalgia impact questionnaire (FIQ), widespread pain index (WPI), visual analog score (VAS) pain, symptom severity (SS) scale, and short form 36 (SF-36) health survey questionnaire have been evaluated.

Results: Patients in the PEMF-TEPT group had a significantly higher reduction of WPI compared to placebo (mean difference -12.90 ± standard deviation [SD] 5.32 vs. -1.91 ± 4.55, difference in difference [DD] of -10.99; P < 0.001), of SS score (-4.10 ± 4.85 vs. -2.00 ± 2.32; DD = -2.1; P < 0.05), of VAS pain (-48 ± 30.75 vs. -16.82 ± 23.69; DD = -31.18; P < 0.01). They also reported a higher improvement of FIQ and SF-36, albeit not reaching statistical significance.

Conclusion: In our pilot controlled study, PEMF-TEPT appeared to be safe and improved fibromyalgia symptoms.

As a newly emerged infectious disease, the coronavirus disease 2019 (COVID-19) has caused millions of deaths, resulting in a global health challenge. Currently, several vaccines have been approved with significant benefits against disease transmission. However, effective therapies are still needed for the clinical management of infected COVID-19 patients. Available evidence has indicated elevated levels of proinflammatory cytokines, including interleukin-6 (IL-6), in COVID-19 patients, with cytokine storm involving excessive cytokine release being observed in some severe cases. Several clinical studies have shown the promising effects of IL-6-blocking strategy in treating severe COVID-19 patients, but some observational studies have reported that IL-6-blocking therapy has no effects in preventing disease progression or death among COVID-19 patients. Herein, we review recent findings on the immunopathogenesis of COVID-19, with specific emphasis on the proinflammatory function of IL-6 and discuss the therapeutic potential of IL-6-blocking therapy for the treatment of COVID-19 patients, especially those with rheumatic diseases.

Objective: To investigate the efficacy of conventional rehabilitation alone and conventional rehabilitation combined with aerobic training on muscle strength and function, health condition, and quality of life for patients with stable idiopathic inflammatory myopathy (IIM).

Methods: This is a historical retrospective cohort study, in which the medical records of patients with IIM, who received the combination of conventional rehabilitative therapy and aerobic training (combined training group [CTG]), from February 2015 to December 2017 were reviewed. Patients with IIM who received conventional therapy alone were matched based on their age, gender, and disease activity as the control group (CG). Scores obtained on manual muscle testing of eight designated muscles (MMT8) was the primary outcome measure, and scores on the myositis Functional Index-2 (FI-2), Health Assessment Questionnaire (HAQ), and 36-item Short Form Medical Outcomes Study Questionnaire (SF-36) at 12 weeks during training were the secondary outcomes.

Results: Fifty-six patients (28 in the CTG and 28 in the CG) were included in this analysis. Patients in both groups had improved MMT8, FI-2, HAQ, and SF-36 scores after 12 weeks of physical therapy. The CTG had a significantly higher score on the MMT8 and HAQ than the CG in the 12th week. The FI-2 scores were significantly higher in the CTG for the four items (P < 0.05) of hip flexion, step test, heel lift, and toe lift. SF-36 scores of the CTG were also higher than those of the CG for the five items (P < 0.05) of physical functioning, general health, vitality, social functioning, and mental health.

Conclusions: Physical exercise training including conventional rehabilitation and aerobic training improved muscle function, health condition, and quality of life. Conventional rehabilitative training combined with aerobic training achieved better improvement compared with conventional rehabilitation training alone.

Anti-DNA autoantibodies are pathogenic in systemic lupus erythematosus (SLE). Cell-free chromatin associated long DNA fragments are antigens for anti-DNA antibodies. In health state, released by cell death and actively secreted by live cells, these cell-free DNA are cleared by deoxyribonucleases (DNASES). In SLE, cell-free DNA are accumulated. The defective clearance of long fragments of cell-free DNA in SLE is largely attributed to impaired deoxyribonuclease 1 like 3 (DNASE1L3). DNASE1L3 null mutation results in monogenic SLE. The SLE risk single-nucleotide polymorphism (rs35677470) encodes R260C variant DNASE1L3, which is defective in secretion, leading to reduced levels of DNASE1L3. In addition, neutralizing autoantibodies to DNASE1L3 are produced in SLE to inhibit its enzymatic activity.