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ID PMID Title PublicationDate abstract
32185341 Venous thromboembolism events among RA patients. 2019 Mar BACKGROUND: Rheumatoid arthritis (RA) is associated with an increased risk for venous thromboembolism. However, so far, relatively few and small size-based studies have been conducted. We aimed to investigate the link between RA and venous thromboembolism utilizing a large sample of subjects originating from a large data base. MATERIALS AND METHODS: The study was performed utilizing the medical database of Clalit Health Services, the largest healthcare provider in Israel. We enrolled all patients with RA and age- and gender-matched controls. Chi-square and t-tests were used for univariate analysis and a logistic regression model was used for a multivariate analysis. RA patients were compared to controls regarding the proportion of venous thromboembolic events (defined as deep vein thrombosis, pulmonary embolism or both). Multivariate logistic regression was employed to assess factors associated with thromboembolic events. RESULTS: The study included 11,782 patients with RA and 57,973 age- and gender-matched controls. RA patients had a higher rate of venous thromboembolism events compared with controls (6.92% vs. 3.18%, respectively, p<0.001). RA and mean C-reactive protein levels were found to be independently associated with the proportion of thromboembolic events (OR 2.27 for RA and 1.07 for each 1 mg/dL increment of mean C-reactive protein, respectively). CONCLUSION: RA and C-reactive protein levels are independently associated with venous thromboembolic events. Physicians should be aware of such findings and have a lower threshold for suspecting detecting such events in patients with RA, mainly those with mean high levels of C-reactive protein.
31884036 The effect of methanol extract from Saussurea involucrata in the lipopolysaccharide-stimul 2020 Apr 6 ETHNOPHARMACOLOGICAL RELEVANCE: Saussureae Involucratae Herba (SIH), known as "snow lotus" in Uyghur and/or Chinese medicines, is generated from the dried aerial part of Saussurea involucrata (Kar. et Kir.) Sch.-Bip. (Asteraceae). The major pharmaceutical value of SIH has been recorded in China Pharmacopoeia, i.e. to balance the immune system, and thus SIH is commonly used for rheumatoid arthritis treatment. Nevertheless, the detailed mechanism of SIH in immune function is still unresolved. AIM OF THE STUDY: Here, we employed macrophage RAW 264.7 cell as a model to demonstrate the signaling pathways, triggered by SIH, in regulating the LPS-induced inflammation. METHODS: The application of SIH methanolic extract suppressed the expression of cytokines, a hallmark of chronic inflammation, in lipopolysaccharide (LPS)-stimulated cultures. RESULTS: The anti-inflammatory functions of SIH were shown to be triggered via NF-κB/PI3K/MAPK signaling pathways by revealing the specific biomarkers, i.e. translocation activities of NF-κB and phosphorylations of Erk1/2, JNK and Akt. CONCLUSION: The aforementioned results showed the underlying action mechanism of SIH in chronic inflammation mitigation, and which might shed light on clinical applications of SIH in traditional Chinese and/or Uyghur medicines.
31720796 Digastric olecranon osteotomy a new approach to the elbow: retrospective study of 24 Coonr 2020 Apr BACKGROUND: Digastric olecranon osteotomy approach allows an excellent articular exposure and preserves principal vascular supply and the continuity of the extensor apparatus. The aim of this study was to assess the early clinical and radiological outcome after total elbow arthroplasty implanted from a digastric olecranon osteotomy approach. METHODS: We retrospectively enrolled 22 patients (two men and twenty women) treated with 24 Coonrad-Morrey(®) total elbow arthroplasty implanted from a digastric olecranon osteotomy approach in Island of France (Paris, Argenteuil and Saint-Denis). The mean age was 80 years (50-96). We treated 20 fractures; according to AO classification, seven patients suffered from a C1 fracture, seven from a C3 and C2, two from malunions, and four from rheumatoid arthritis. The mean time of follow-up was 30 months (6-132). Clinical outcomes were assessed with the Mayo elbow performance score. We evaluated triceps strength and radiographic healing. RESULTS: At the latest follow-up, the average flexion arc was 23° (5°-50°) to 112° (95°-130°). The Mayo elbow performance score averaged 92 points (75-100). The mean strength of the triceps in extension and flexion was, respectively, 1.9 and 4.7 kgs. All elbows were stable. A single immediate post-operative wound infection was reported and did not require any surgical revision. Radiological consolidation of the olecranon osteotomy was assessed in sixteen patients between 8 and 16 weeks. Heterotopic ossifications were noted in one elbow. One patient had an elbow dislocation by fracture of the axe's component. CONCLUSION: The early clinical and radiological outcomes are promising and support the use of digastric olecranon osteotomy for the implantation of total elbow arthroplasty. LEVEL OF EVIDENCE: Treatment study, level IV.
31489783 Risk of cutaneous herpes zoster in patients with spondyloarthritis treated with convention 2020 Feb OBJECTIVES: To investigate the risk of cutaneous herpes zoster (HZ) in spondyloarthritis (SpA) compared with that in rheumatoid arthritis (RA), and in disease-modifying antirheumatic drugs (DMARDs) used in SpA. METHOD: A total of 727 patients with an expert diagnosis of SpA were identified retrospectively from four rheumatology centers in Hong Kong. Electronic medical records from 1995 to 2018 were reviewed for incidence of cutaneous HZ and demographic data including age, sex, comorbidities, smoking and drinking status. DMARDs used included sulphasalazine, methotrexate, leflunomide, steroids, etanercept, infliximab, adalimumab, golimumab, secukinumab and ustekinumab. Cox regression models were used to evaluate hazard ratios (HRs) of different DMARDs in patients with SpA. Propensity score was used for matching and comparison with 857 patients with RA. RESULTS: There were 23 cases of cutaneous HZ in patients with SpA and 59 cases in patients with RA. Among patients with SpA, 7 cases of cutaneous HZ may be attributed to sulfasalazine treatment, 7 to methotrexate, 2 to leflunomide, 2 to infliximab, 1 to etanercept, 2 to adalimumab, and 1 to secukinumab. Risks of cutaneous HZ were the same in SpA (stratified HR 0.97; 95% CI 0.58; 1.61; P = .89) and RA. Methotrexate (adjusted HR 3.47; 95% CI 1.25; 9.63; P = .02) and infliximab (adjusted HR 10.67; 95% CI 1.37; 82.88; P = .02) were found to be associated with HZ after adjustments for traditional risk factors. CONCLUSION: Risk of cutaneous HZ in SpA was not lower than in RA. Methotrexate and infliximab were associated with cutaneous HZ in SpA.
31679995 Contraception and preconception counseling in women with autoimmune disease. 2020 Apr Appropriate contraception and preconception counseling are critical for women of reproductive age with systemic autoimmune diseases (AIDs) because clinical diagnosis, rheumatology medications, and disease activity may impact the safety or efficacy of certain contraceptives as well as the risk of adverse pregnancy outcomes. The presence of antiphospholipid (aPL) antibodies (anticardiolipin, anti-β2 glycoprotein I, and lupus anticoagulant) is the most important determinant of contraception choice, as women with these antibodies should not receive estrogen-containing contraceptives because of the increased risk of thrombosis. Prepregnancy counseling generally includes the assessment of preexisting disease-related organ damage, current disease activity, aPL antibodies, anti-Ro/SS-A and anti-La/SS-B antibodies, and medication safety in pregnancy. Quiescent AID for six months on pregnancy-compatible medications optimizes maternal and fetal/neonatal outcomes for most patients.
31376896 Connective Tissue Disease-Associated Interstitial Lung Disease: Evaluation and Management. 2019 Sep Interstitial lung disease is common among patients with connective tissue disease and is an important contributor to morbidity and mortality. Infection and drug toxicity must always be excluded as the cause of radiographic findings. Immunosuppression remains a mainstay of therapy despite few controlled trials supporting its use. When a decision regarding therapy initiation is made, considerations include an assessment of disease severity as well as a determination of the rate of progression. Because patients may have extrathoracic disease activity, a multidisciplinary approach is crucial and should include supportive and nonpharmacologic management strategies.
31550708 High Risk of Sudden Sensorineural Hearing Loss in Several Autoimmune Diseases according to 2019 OBJECTIVE: We investigated the risk of sudden sensorineural hearing loss (SSNHL) in patients with autoimmune diseases compared with a control group in a population-based study using a National Health Insurance Service National Sample Cohort data from Korea. METHODS: We enrolled autoimmune-disease patients who were ≥20 years of age in 2006 into the autoimmune-disease group, and selected a control group with similar demographic characteristics. We tracked the 2 groups from 2006 to 2015 and compared the proportions of patients who developed SSNHL. RESULTS: Of 13,250 patients in the autoimmune-disease group, 145 experienced an SSNHL event (1.09%). Of the 66,250 in the control group, 484 experienced an SSNHL event (0.73%). The SSNHL risk was significantly higher in the autoimmune-disease group than in the control group. SSNHL incidence was significantly higher among patients with antiphospholipid syndrome (APS), multiple sclerosis (MS), rheumatoid arthritis (RA), and connective-tissue diseases including Sjögren syndrome and Behçet disease. In detailed stratified analyses using reinforced additional diagnostic codes, only RA patients had a significantly higher SSNHL incidence than the control group. CONCLUSION: The association of several autoimmune diseases with SSNHL was evaluated in this large-scale, population-based, big-data study. The risk of SSNHL was significantly higher in patients with APS, MS, RA, and connective-tissue diseases including Sjögren syndrome and Behçet disease than in patients without autoimmune diseases. SSNHL, in particular, was significantly associated with RA according to detailed analyses using reinforced additional diagnostic codes.
31912015 Rheumatological Findings in Patients with Breast Cancer. 2020 Jan OBJECTIVE: Breast Cancer (BC) is the most frequently diagnosed malignancy worldwide. Not only may BC be associated with rheumatic symptoms and diseases, but also the drugs used in the treatment of this disease, including aromatase inhibitors (AIs), may lead to musculoskeletal system symptoms. In this study, we aimed to investigate the spectrum of rheumatic symptoms and diseases developing in patients with BC having no previous diagnosis of any inflammatory rheumatic disease. MATERIALS AND METHODS: Patients with a history of BC referring to Rheumatology Outpatient Clinics with complaints of musculoskeletal system symptoms at two centers between 2008 and 2018 were screened retrospectively. Patients with a previous diagnosis of any inflammatory rheumatic diseases before the occurrence of BC were excluded. Demographic data, onset and duration of BC, as well as onset and duration of rheumatic symptoms/diseases were recorded. Relevant laboratory tests, including autoantibodies, available imaging findings and the treatments received were also registered. RESULTS: Mean age of 128 BC patients at the time of admission was found to be 54.76±8.21 years. Mean durations of disease for BC and rheumatic disorders were 85.705±15.507 and 60.84±19.20 months, respectively. Out of 128 BC patients, nearly one third (n: 41; 32.03%), developed an inflammatory rheumatic disease, and rheumatoid arthritis was the most frequent pathology. Nonspecific arthralgia and myalgia were more frequent in patients receiving AIs than those receiving tamoxifen, despite lack of significant difference (p=0.421, p=0.411). CONCLUSION: Given that nearly one third of the patients developed an inflammatory rheumatic disease, it should be remembered that locomotor symptoms in patients with BC may be caused not only by bone metastasis or paraneoplastic effects, but they may also suggest the presence of associated rheumatic diseases.
30975623 Discovery of 1,3-dihydro-2H-imidazo[4,5-c]quinolin-2-ones based novel, potent and PI3Kδ s 2019 Jun 1 PI3Kδ is implicated in various inflammatory and autoimmune diseases. For the effective treatment of chronic immunological disorders such as rheumatoid arthritis, it is essential to develop isoform selective PI3Kδ inhibitors. Structure guided optimization of an imidazo-quinolinones based pan-PI3K/m-TOR inhibitor (Dactolisib) led to the discovery of a potent and orally bioavailable PI3Kδ isoform selective inhibitor (10h), with an improved efficacy in the animal models.
30885844 Apical splenic nerve electrical stimulation discloses an anti-inflammatory pathway relying 2019 Aug The autonomic nervous system innervates all lymphoid tissues including the spleen therefore providing a link between the central nervous system and the immune system. The only known mechanism of neural inhibition of inflammation in the spleen relies on the production of norepinephrine by splenic catecholaminergic fibers which binds to β2-adrenergic receptors (β 2-ARs) of CD4(+) T cells. These CD4(+) T cells trigger the release of acetylcholine that inhibits the secretion of inflammatory cytokines by macrophages through α7 nicotinic acetylcholine receptor (α7nAchRs) signaling. While the vagal anti-inflammatory pathway has been extensively studied in rodents, it remains to be determined whether it coexists with other neural pathways. Here, we have found that three nerve branches project to the spleen in mice. While two of these nerves are associated with an artery and contain catecholaminergic fibers, the third is located at the apex of the spleen and contain both catecholaminergic and cholinergic fibers. We found that electrical stimulation of the apical nerve, but not the arterial nerves, inhibited inflammation independently of lymphocytes. In striking contrast to the anti-inflammatory pathway mechanism described so far, we also found that the inhibition of inflammation by apical nerve electrical stimulation relied on signaling by both β 2-ARs and α7nAchRs in myeloid cells, with these two signaling pathways acting in parallel. Most importantly, apical splenic nerve electrical stimulation mitigated clinical symptoms in a mouse model of rheumatoid arthritis further providing the proof-of-concept that such an approach could be beneficial in patients with Immune-mediated inflammatory diseases.
31275778 An Incidental Finding of Anemia: Rectal Adenocarcinoma in a Young Adult. 2019 Apr 27 Rectal adenocarcinoma is an uncommon finding in patients under the age of 40. However, epidemiological surveys have suggested that colorectal cancers are increasing in incidence among patients aged 20 to 39 years in the United States. Colorectal adenocarcinoma is often not considered in the differential diagnosis in this demographic because of age. Here, we present the case of an incidental finding of anemia during a preliminary evaluation of rheumatoid arthritis leading to the diagnosis of stage IV-B rectal adenocarcinoma in a 34-year-old male patient. A 34-year-old Caucasian male presented with the incidental finding of anemia during a preliminary evaluation for rheumatoid arthritis. The patient was asymptomatic with the exception of a three-month history of wrist and ankle joint pain. Past medical history was positive for only a three-year history of occasional spotty, painless rectal bleeding attributed to internal hemorrhoids. Physical exam findings were positive for mild extremity pallor and positive fecal occult blood test. Hematologic studies revealed a significant microcytic, hypochromic anemia with severe iron deficiency. Laboratory studies revealed no evidence of vitamin deficiency, hemolytic activity, hematuria, hypothyroidism, or clotting factor disorder. Erythrocyte sedimentation rate (ESR), rheumatoid factor, and cyclic citrullinated peptide 3 (CCP3) were elevated supporting the diagnosis of underlying rheumatoid arthritis. On further questioning, the patient revealed that he had been utilizing an average of 2000 mg of ibuprofen daily during the previous several months in an attempt to control his joint pain. The patient was evaluated for a potential upper gastrointestinal bleed by esophagogastroduodenoscopy (EGD), which found no evidence of active bleeding. As the patient continued to have decreasing hemoglobin levels, he was evaluated for a lower gastrointestinal source of bleeding by colonoscopy, which revealed an 8 cm circumferential mass at the anal verge. Pathological evaluation of biopsy samples revealed a moderately differentiated invasive adenocarcinoma. The patient had no family history of colorectal cancer or major associated risk factors, such as obesity, smoking history, heavy alcohol use, diabetes mellitus type 2, or a history of inflammatory bowel disease. Following discharge, positron emission tomography (PET) scan showed extensive metastatic disease to multiple regional lymph nodes as well as multiple suspicious hepatic lesions and bilateral pulmonary nodules. Due to the poor prognosis, recommended treatment consisted of folinic acid, 5-fluorouracil, oxaliplatin (FOLFOX-4) along with palliative radiation. The cause of the increase in the incidence rate of colorectal cancer in young adults remains unknown. Among this demographic, colorectal cancers appear to be more aggressive and present at later stages with more advanced disease. In young adults, the most common clinical sign at presentation is rectal bleeding. In young adults presenting with seemingly common gastrointestinal complaints, a high degree of suspicion for colorectal cancer may be warranted by clinicians.
28455825 Taxol alleviates collagen-induced arthritis in mice by inhibiting the formation of microve 2019 Jan The objective of the present study is to evaluate the inhibitory effects of taxol (PTX) on angiogenesis in a collagen-induced arthritis (CIA) mouse model. Collagen II (C II) and complete Freund's adjuvant (CFA) were used in C57BL/6 (H-2b) mice to generate the CIA model. Random grouping was performed in the normal control group, CIA model group, PTX 1.5 mg/kg group, PTX 1.0 mg/kg group, and PTX 0.5 mg/kg group. Arthritis index scores, tissue pathology scores, and synovium microvessel density (MVD) analysis were performed. Immunohistochemistry and enzyme-linked immunosorbent assay were used to detect the expression of vascular endothelial growth factor (VEGF) and hypoxia-inducible factor-α (HIF-1α). The correlation between MVD and pathological scores and between MVD and the expression of VEGF as well as HIF-1α in the synovium were also evaluated. After PTX treatment, the three intervention group arthritis index scores were reduced when compared with the CIA group. The total histological scores in the three PTX treatment groups were lower than those in the CIA group. Similarly, PTX significantly alleviated the scores for synovitis, pannus formation, and bone destruction. Compared with the CIA group, the MVD of the three intervention groups decreased in a dose-dependent manner. The expression of VEGF and HIF-1α in synovial tissues and serum also significantly decreased after PTX treatment. Further analysis showed that MVD and pathological scores and MVD and expression of VEGF as well as HIF-1α in the synovium were positively correlated. PTX may alleviate CIA by suppressing angiogenesis, providing new insights into the treatment of rheumatoid arthritis (RA). VEGF and HIF-1α may be targets for PTX suppression of microvessel formation.
31471716 Incidence of cancer in a cohort of patients with primary Sjögren syndrome in Argentina. 2019 Oct Primary Sjögren syndrome (pSS) is usually a mild disease, but serious complications such as non-Hodgkin lymphoma-and hypothetically other malignancies-may develop. The aim of this study was to evaluate both overall and specific incidence of cancer in a cohort of patients with pSS compared to the expected incidence in general population of Argentina. Retrospective analytic study of pSS patients fulfilling American-European Consensus Group (AECG) criteria, followed from the time of their diagnosis until the end of the study, death, loss of follow- up, or being given a diagnosis of cancer. Cancer incidence for the general population was obtained from GLOBOCAN 2018, and demographic information was obtained from the national institute for statistics and census. Age- and sex-specific Standardized Incidence Ratio (SIR) were then calculated. One hundred fifty-seven patients, with a mean age of 57.8 years (SD 18.3), were included. Mean patient follow-up duration was 7.37 years (SD 4.2), contributing to a total of 1158 patient/years. Fifteen patients developed a malignancy during follow-up. Cancer incidence for pSS patients was compared with the general population's incidence through SIRs. Female patient's SIRs for overall cancer was 4.17 (95% CI 2.30-6.87), non-Hodgkin lymphoma 41.40 (95% CI 10.12-102.1), multiple myeloma 41.49 (95% CI 1.14-167.28), tongue cancer 44.4 (95% CI 1.23-177.31), uterus cancer 8.39 (95% CI 0.19-40.73), lung cancer 4.51 (95% CI 0.1-22.16), and breast cancer 3.76 (95% CI 1.04-9.45). An increased overall cancer risk, and particularly for non-Hodgkin lymphoma, multiple myeloma, breast cancer and tongue cancer was observed in female pSS patients compared to control group.
31309081 Chronic obstructive pulmonary disease and rheumatic diseases: A systematic review on a neg 2019 Jan BACKGROUND: Although, both chronic obstructive pulmonary disease (COPD) and rheumatic diseases (RDs) are common, and each has significant impact on patients' overall health/quality of life, their co-occurrence has received little attention, while 15% of COPD remains undiagnosed in RDs. OBJECTIVE: To update the information regarding the comorbid state of RD/COPD (prevalence, incidence), to examine whether patients with RD have increased risk of developing COPD and vice versa, and what implications this comorbidity has on patients' outcomes (mortality, hospitalizations, exacerbations). METHODS: We performed a systematic literature review regarding the comorbidity of an RD (rheumatoid arthritis (RA), ankylosing spondylitis (AS), psoriatic arthritis (PsA), systemic lupus erythematosus (SLE), primary Sjogren syndrome disease (pSS), and systemic sclerosis (SSc)) with COPD. From 2803 reports retrieved, 33 articles were further screened. Finally, 27 articles were included. RESULTS: Robust evidence supports that COPD develops up to 68% more frequently in patients with RA, as compared to the general population. Similarly, COPD is increased in every other RD that was studied. Further, self-referred arthritis is more common in COPD patients versus non-COPD controls and a predictor of worst self-rated health status. Patients with inflammatory arthritis/COPD have increased mortality (threefold in RA-COPD, irrespectively of which is first diagnosed), hospitalizations, and emergency visits. CONCLUSION: COPD is more common in patients with RA, AS, PsA, SLE, pSS, and SSc; yet, the association, vice versa, warrants further investigation. Nevertheless, COPD/RDs coexistence has significant prognostic value for worst outcomes; therefore, awareness is required to track early identification, especially in primary care.
30578887 Venlafaxine and carvedilol ameliorate testicular impairment and disrupted spermatogenesis 2019 Feb 1 Testicular impairment has been commonly described in long-standing rheumatoid arthritis (RA) patients. Since depression and cardiovascular disorders are the most disturbing co-morbidities of RA, investigating the efficacy of the anti-depressant venlafaxine or the beta-blocker carvedilol in RA-associated testicular dysfunction may add to their clinical utility for RA patients. Previously, both agents have demonstrated significant in vivo anti-oxidant and anti-inflammatory actions. In the current study, venlafaxine (50 mg/kg/day) and carvedilol (10 mg/kg/day) were orally administered to adjuvant arthritic rats for 20 days. Interestingly, venlafaxine and carvedilol effectively suppressed paw edema and mitigated the testicular histopathological aberrations and the disrupted spermatogenesis. Both drugs enhanced testicular steroidogenesis through upregulation of 3β-HSD, 17β-HSD and StAR gene expression with concomitant augmentation of serum testosterone. They also blunted the inflammatory burden via attenuation of myeloperoxidase, TNF-α and the protein expression of NF-κBp65 along with elevation of IL-10. They attenuated testicular oxidative perturbations via lowering lipid peroxides and nitric oxide and boosting glutathione levels. With regard to apoptosis, the two agents lowered the protein expression of caspase-3, cleaved caspase-3, cleaved PARP, Bax and p53, promoting germ cell survival. They also modulated the AMPK/ERK signaling via lowering of p-AMPK and upregulation of p-ERK1/2 along with PI3K/AKT/mTOR transduction by enhancing the PI3Kp110α, p-AKT and p-mTOR protein expression. Together, the present work demonstrates the beneficial effects of venlafaxine and carvedilol in RA testicular dysfunction and impaired spermatogenesis via modulation of AMPK/ERK and PI3K/AKT/mTOR signaling and intervention with the testicular oxidative stress, inflammation and apoptosis.
31349012 Variants at potential loci associated with Sjogren's syndrome in Koreans: A genetic associ 2019 Oct Sjogren's syndrome (SS), a chronic autoimmune disease, typically causes or involves inflammation in the salivary and lacrimal glands. Although recent genetic association studies have contributed to the discovery of SS susceptible genes, few studies have reported on the Korean population. Here, we did a genetic association study of SS in Korean patients using whole-exome sequencing data of 15 patients and 100 healthy controls. In addition to confirming previously described SS susceptibility loci MSH5 (p = 1.67 × 10-5) and RELN (p = 4.91 × 10-6), we also validated PRAMEF13 (p = 2.28 × 10-5), TARBP1 (p = 1.87 × 10-5), UGT2B28 (p = 1.33 × 10-5), TRBV5-6 (p = 2.27 × 10-5) and NAPB (p = 3.73 × 10-5) as novel susceptibility loci for SS. Furthermore, we identified UGT2B28, TARBP1 and PRAMEF13 as associated with human immune function. These findings may provide useful insight into to the pathways and pathogenesis contributing to SS susceptibility in the Korean population.
30887439 A Patient with Sjogren's Syndrome and Subsequent Diagnosis of Inclusion Body Myositis and 2019 Jun We discuss a challenging case of a 58-year-old Vietnamese-American woman who presented to her new primary care provider with an 8-year history of slowly progressive dysphagia, hoarseness, muscle weakness with associated frequent falls, and weight loss. She eventually reported dry eyes and dry mouth, and she was diagnosed with Sjogren's syndrome. Subsequently, she was additionally diagnosed with inclusion body myositis and gastric light-chain (AL) amyloidosis. Although inclusion body myositis has been previously associated with Sjogren's syndrome, inclusion body myositis is rare in non-Caucasians, and the trio of Sjogren's syndrome, inclusion body myositis, and AL amyloidosis has not been previously reported. Sjogren's syndrome is a systemic autoimmune condition characterized by ocular and oral dryness. It is one of the most common rheumatologic disorders in the USA and worldwide. Early diagnosis of Sjogren's is particularly important given the frequency and variety of associated autoimmune diseases and extraglandular manifestations. Furthermore, although inclusion body myositis has a low prevalence, it is the most common inflammatory myopathy in older adults and is unfortunately associated with long delays in diagnosis, so knowledge of this disorder is also crucial for practicing internists.
30767871 Adult-onset Still's disease-associated interstitial lung disease represents severe phenoty 2019 Nov OBJECTIVES: Adult-onset Still's disease (AOSD) is an inflammatory disorder characterised by sustained fevers, arthritis, and skin involvement. Interstitial lung disease (ILD) is a rare manifestation, and its clinical characteristics have yet to be determined. METHODS: We sought to examine the clinical characteristics of AOSD-associated ILD. We retrospectively investigated 78 patients diagnosed as AOSD. ILD was diagnosed based on chest high-resolution computed tomography (HRCT). Clinical characteristics were compared between patients with and without ILD. Relapse was defined as sustained fevers, re-emergence of arthritis, and skin involvement after remission. We further investigated the pathological features of ILD on available samples. RESULTS: Patients with ILD, found in 9 of 78 (11.5 %), had older age of onset (mean age 62.6) than those without ILD (mean age 38.8) (p<0.01). The 3-year survival rates were comparable between patients with ILD (92.5%) and those without ILD (88.9%) (p=0.23). Patients with ILD had a higher cumulative rate of haemophagocytic syndrome (HPS) and relapse than those without (p<0.0001 and p=0.009, respectively). Chest HRCT showed marked thickening of the interlobular septa, the bronchovascular bundles, or the visceral pleura in all cases. There was no honeycomb or volume loss. Pulmonary pathological findings revealed marked thickening of the visceral pleura and the interlobular septa. CONCLUSIONS: Patients with ILD might have higher risks for HPS and relapse. Careful observation and appropriate therapeutic intervention might be needed.
31607022 [Primary Sjogren's syndrome associated pulmonary arterial hypertension: 20 new cases]. 2019 Oct 8 Objective: To describe baseline characteristics of patients with primary Sjogren's syndrome (pSS) with right heart catheterization (RHC)-confirmed pulmonary arterial hypertension (PAH). Methods: This retrospective study included consecutive patients hospitalized with pSS-PAH from Jan.2013 to June.2019 in Tianjin Medical University General Hospital. All patients fulfilled the 2002 revised criteria for pSS proposed by the American-European Consensus Group. PAH was defined according to RHC-based European Society of Cardiology/European Respiratory Society guidelines. Associated variables were described and analyzed. Predictive factors for achieving treatment goals were explored. Sub-groups(based on Anti-SSB antibody positive/negative) were analyzed. Results: Twenty patients with RHC-confirmed pSS-PAH were included (median age at onset, 54.5 years; median PAH duration, 12 months).PAH was the initial manifestation of pSS in 5 patients(26.3%), and shortness of breath was the most common symptom (17/20,85%). Mean pulmonary arterialpressure (mPAP) was 48(38, 55.75)mmHg, pulmonary vascularresistance (PVR) was 11.23 (6.17,14.69)IU, and mean cardiac index was 2.59(2.04,2.98)L·min(-1)·m(-2) in this group. In addition, right ventricular diameter≤40 mm[P=0.009, HR=5.92, 95%CI(1.55, 22.59)] was predictive factor for achieving the treatment goal. Furthermore, compared with Anti-SSB antibody negative patients, patients with Anti-SSB antibody positive had a higher PVR(IU) [14.1(11.23, 22.63)vs 6.4(6.15, 12.36),P=0.025], a higher mPAP(mmHg)[5(48.5,59)vs 39(32.5,50.75),P=0.013], and a lower cardiac index (L·min(-1)·m(-2)) [2.1(1.6,2.5)vs 2.9(2.56,3.41),P=0.002]. Conclusions: pSS-PAH patients are mainly middle-aged women with the most common symptom shortness of breath. And PAH can be the first manifestation of pSS. More attention should be paid to patients with Anti-La/SSB antibody positive and right ventricular diameter ≤40 mm because of higher mPAP, PVR, worse cardiac function and prognosis.
30917421 [Marrow megakaryocyte count as a response predictor of severe thrombocytopenia in patients 2019 Apr 1 Objective: To explore the predictive factors for determining the therapeutic response and prognosis of severe thrombocytopenia (TP) in patients with primary Sjögren syndrome(pSS). Method: Patients with pSS and severe TP (platelet count ≤ 50×10(9)/L) admitted between 2010 to 2016 at Peking Union Medical College Hospital were classified according to their therapeutic response and analyzed retrospectively. The response parameters and clinical data including bone marrow aspiration results and laboratory findings were collected. Result: Thirty patients were finally analyzed, including twenty with appreciable bone marrow aspiration results. Fourteen and 7 patients achieved a complete response (CR) and a partial response (PR) respectively, other 9 patients with no response (NR). The megakaryocyte counts in bone marrow (BM-MK) counts per slide in each group were 13.0 (9.2,23.5) in CR patients, 7.0 (7.0,20.0) in PR patients, and 5.0 (1.0,6.0) in NR patients. BM-MK counts in patients with clinical response (CR+PR) were significantly higher than those with NR (P=0.006). A receiver-operation characteristic analysis revealed a cutoff value of BM-MK counts at 6.5 per slide stratifying patients by different responses with a sensitivity of 13/14, a specificity of 6/7, and area under the curve of 0.879. Univariate analysis indicated a better prognosis as BM-MK counts>6.5 per slide. Conclusion: BM-MK count could be a predictive factor of response in patients with pSS and severe TP. Patients with BM-MK counts≤6.5 per slide represent worse platelet improvement..