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Total 12-Lead QRS Voltage in Patients Having Orthotopic Heart Transplantation for Heart Failure Caused by Adriamycin-Induced Cardiomyopathy.

Actualités En Médecine Interne - jeu, 17/01/2019 - 15:50

Total 12-Lead QRS Voltage in Patients Having Orthotopic Heart Transplantation for Heart Failure Caused by Adriamycin-Induced Cardiomyopathy.

Cardiology. 2019 Jan 16;141(3):172-175

Authors: Roberts WC, Haque S, Hall SA

Abstract
OBJECTIVE: Although several studies have described the effects of adriamycin on the heart, electrocardiographic total 12-lead QRS voltage (distance in millimeters from the peak of the R wave to the nadir of either the Q or S wave, whichever was deeper, with 10 mm [1 mV] being standard) both before and after orthotopic heart transplantation (OHT) has not been reported. This study describes the total 12-lead QRS voltage in 8 patients studied at Baylor University Medical Center at Dallas, from 1994 to June 2018, who underwent OHT for severe heart failure caused by anthracycline-induced cardiomyopathy.
METHOD: Prior to OHT, the total 12-lead non-paced QRS voltages ranged from 86 to 189 mm (mean 125 ± 56) and for paced QRS voltages from 82 to 113 mm (mean 97 ± 15). The total 12-lead QRS voltages post-OHT ranged from 100 to 190 mm (mean 130 ± 30). Total 12-lead QRS voltages were lower in patients with a pacemaker than without.
RESULTS/CONCLUSION: These low voltages are like those found in patients with carcinoid syndrome, severe cardiac adiposity, cardiac amyloidosis, and cardiac sarcoidosis.

PMID: 30650419 [PubMed - as supplied by publisher]

Established coronary artery disease in systemic sclerosis compared to type 2 diabetic female patients: a cross-sectional study.

Actualités En Médecine Interne - jeu, 17/01/2019 - 15:50

Established coronary artery disease in systemic sclerosis compared to type 2 diabetic female patients: a cross-sectional study.

Clin Rheumatol. 2019 Jan 16;:

Authors: Colaci M, Giuggioli D, Spinella A, Vacchi C, Lumetti F, Mattioli AV, Coppi F, Aiello V, Perticone M, Malatino L, Ferri C

Abstract
INTRODUCTION: Systemic sclerosis (SSc) is an autoimmune disease characterized by endothelial dysfunction, which is also associated with other disorders, such as atherosclerosis. The direct role of SSc in facilitating cardiovascular events should be clarified. We compared the prevalence of established coronary artery disease (CAD) between SSc and type 2 diabetes, a well-known phenotype associated with high cardiovascular risk.
METHODS: In this cross-sectional study, we evaluated a cohort of 290 unselected female SSc patients, in comparison with 265 aged-matched female type 2 diabetics. "Established CAD" was defined as previous myocardial infarction, unstable angina or ischemia documented by ECG and troponin elevation, necessity/previous treatment with coronary angioplasty or stenting. Age subgroups < 45 (Q1), 45-54 (Q2), 55-64 (Q3), 65-74 (Q4), and ≥ 75 (Q5) years were considered for SSc and diabetes.
RESULTS: CAD prevalence resulted lower in SSc patients than in diabetics (10% (95%CI 6.9-14.1) versus 19.2% (95%CI 14.9-24.4); p = 0.0023). In Q2 patients, CAD never occurred in SSc (95%CI 0-8.4), but in 9.4% of diabetics (95%CI 3.7-20.7, p = 0.0567); in Q3 subjects, CAD was reported in 5.6% (95%CI 1.8-13.8) of SSc, but in 20% (95%CI 12.4-30.5) of diabetics (p = 0.0127). Instead, for Q4 and particularly Q5 patients, CAD prevalence was comparable in SSc and diabetes.
CONCLUSIONS: The prevalence of established CAD in SSc was lower compared with diabetics. However, in older SSc patients the prevalence of CAD was similar to that observed in diabetics.

PMID: 30649681 [PubMed - as supplied by publisher]

Fusobacterium necrophorum septic pelvic thrombophlebitis after intrauterine device insertion.

Actualités En Médecine Interne - jeu, 17/01/2019 - 15:50

Fusobacterium necrophorum septic pelvic thrombophlebitis after intrauterine device insertion.

Int J Gynaecol Obstet. 2019 Jan 16;:

Authors: Yamamoto S, Okamoto K, Okugawa S, Moriya K

Abstract
On physical examination, the patient was febrile, hypotensive, and tachycardic. After admission, her IUD was removed. Subsequently, three sets of blood cultures grew Fusobacterium necrophorum. Contrast-enhanced computed tomography (CT) showed a thrombus in the right internal iliac artery, multiple hypodense liver lesions with ring enhancement, and multiple pulmonary emboli (Fig. 1). Diagnoses of septic thrombophlebitis, liver abscesses, and pulmonary septic emboli were made. Magnetic resonance imaging (MRI) of the pelvis with intravenous contrast revealed an enhancement of the venous plexus on the right side of the uterus, suggestive of the origin of the infection. This article is protected by copyright. All rights reserved.

PMID: 30648745 [PubMed - as supplied by publisher]

Serum and urinary calcium level in Latvian patients with sarcoidosis.

Actualités En Médecine Interne - jeu, 17/01/2019 - 15:50
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Serum and urinary calcium level in Latvian patients with sarcoidosis.

Reumatologia. 2018;56(6):377-381

Authors: Ruža I, Lucāne Z

Abstract
Objectives: Sarcoidosis is a multisystem granulomatous disease of unknown etiology that in 90% of cases affects the lungs. Calcium metabolism testing can be useful in diagnostics. The aim of the study was to assess the correlation between calcium metabolism and sarcoidosis form of manifestation/demographic indicators.
Material and methods: In a retrospective study medical records of all patients (n = 699) who had been hospitalized with suspected sarcoidosis in a specialized clinic of Riga Eastern Clinical University Hospital during the period from January 1st, 2013 until December 31st, 2014 were analyzed. Further analysis included only patients with histologically and/or clinically confirmed sarcoidosis (n = 281).
Results: Patients' average age at the time of diagnosis was 39 ±13 years. Elevated serum calcium was observed in 9.9% of cases. A statistically significant correlation was found between serum calcium and age (p < 0.01). There was an association between serum calcium and gender (p < 0.05) - levels were higher in men (2.43 mmol/l) than in women (2.40 mmol/l). Elevated calcium in 24-hour urine was observed in 22.7% of patients. The mean value was 232.3 mg/24 h, levels were higher in men (258.7 mg/24 h) than in women (202.3 mg/24 h), and the association with gender was statistically significant (p < 0.01).
Conclusions: We can conclude that in Latvia sarcoidosis affects mostly young and middle-aged people. Both serum calcium and calcium in 24-hour urine are important parameters for sarcoidosis diagnostics. Hypercalcemia was found in 9.9% of patients, hypercalciuria in 22.7% of patients, and both were statistically significantly higher in men, regardless of age.

PMID: 30647484 [PubMed]

Subgroups of Sjögren's syndrome patients categorised by serological profiles: clinical and immunological characteristics.

Actualités En Médecine Interne - jeu, 17/01/2019 - 15:50
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Subgroups of Sjögren's syndrome patients categorised by serological profiles: clinical and immunological characteristics.

Reumatologia. 2018;56(6):346-353

Authors: Kontny E, Lewandowska-Poluch A, Chmielińska M, Olesińska M

Abstract
Objectives: Sjögren's syndrome (SS) is an autoimmune disease characterised by heterogeneous clinical presentation and presence of various autoantibodies - anti-SSA/Ro of diagnostic value, less specific anti-SSB/La and others. We searched for biomarker(s) and potential therapeutic target(s) of SS subsets that vary in their autoantibody profile.
Material and methods: Eighty-one patients with SS (70 female and 11 male) and 38 healthy volunteers (28 female and 10 male) were included in the study. Patients were categorised according to absence (group 1) or presence of anti-SSA/Ro antibody which occurred either alone (group 2) or together with anti-SSB/La (group 3). Clinical evaluation was performed, and presence of autoantibodies and concentrations of cytokines relevant to SS pathogenesis, i.e. a proliferation inducing ligand (APRIL), B-lymphocyte activating factor (BAFF), interleukin (IL) 4, IL-10, interferon α (IFN-α) and thymic stromal lymphopoietin (TSLP), in sera were determined.
Results: Frequency of autoantibodies other than anti-SSA/Ro and anti-SSB/La, the number of autoantibody specificities and anti-nuclear antibody titres were higher in group 2 and/or 3 than in group 1 of SS patients. Moreover, SS patients of groups 2 and 3 developed disease symptoms at younger age, and more often had positive Schirmer's test and skin lesions. In addition, serum concentrations of APRIL, but not other tested cytokines, were significantly higher in the patients of both groups 2 and 3 than those of group 1 and healthy volunteers.
Conclusions: Sjögren's syndrome patients with signs of B-cell epitope spreading are characterised by early disease onset, more frequent xerophthalmia and skin involvement, and up-regulated serum APRIL level. We suggest that therapeutic neutralisation of APRIL may be beneficial for these patients.

PMID: 30647480 [PubMed]

Report from the Hand Osteoarthritis Working Group at OMERACT 2018: Update on Core Instrument Set Development.

Actualités En Médecine Interne - jeu, 17/01/2019 - 15:50
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Report from the Hand Osteoarthritis Working Group at OMERACT 2018: Update on Core Instrument Set Development.

J Rheumatol. 2019 Jan 15;:

Authors: Wittoek R, Kroon FPB, Kundakci B, Abhishek A, Haugen IK, Berenbaum F, Conaghan PG, Ishimori ML, Smeets W, van der Heijde D, Kloppenburg M

Abstract
OBJECTIVE: To evaluate hand osteoarthritis tools for core instrument set development.
METHODS: For OMERACT 2018, a systematic literature review and advances in instrument validation were presented.
RESULTS: Visual analog and numerical rating scales were considered valuable for pain and patient's global assessment, despite heterogeneous phrasing and missing psychometric evidence for some aspects. The Modified Intermittent and Constant Osteoarthritis Pain scale was lacking evidence. The Michigan Hand Outcomes Questionnaire had advantages above other pain/function questionnaires. The Hand Mobility in Scleroderma scale was valid, although responsiveness was questioned. Potential joint activity instruments were evaluated.
CONCLUSION: The development of the core instrument set is progressing, and a research agenda was also developed.

PMID: 30647176 [PubMed - as supplied by publisher]

Efficacy of Methotrexate in Real-world Management of Giant Cell Arteritis: A Case-control Study.

Actualités En Médecine Interne - jeu, 17/01/2019 - 15:50
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Efficacy of Methotrexate in Real-world Management of Giant Cell Arteritis: A Case-control Study.

J Rheumatol. 2019 Jan 15;:

Authors: Koster MJ, Yeruva K, Crowson CS, Muratore F, Labarca C, Warrington KJ

Abstract
OBJECTIVE: To determine the effect of methotrexate (MTX) on relapse risk and glucocorticoid (GC) use in a large single-institution cohort of patients with giant cell arteritis (GCA).
METHODS: Patients diagnosed with GCA from 1998 to 2013 with confirmed evidence of temporal artery biopsy and/or radiographic evidence of large vessel vasculitis were identified. Each patient with GCA treated with adjunct MTX (case) was matched to a similar patient with GCA treated only with GC (control). GC requirements and relapse events before and after MTX initiation (or corresponding index date) were compared using rate ratios (RR).
RESULTS: Eighty-three cases and 83 controls were identified and compared. No significant differences in age, demographics, laboratory variables, baseline disease characteristics, or mean initial prednisone doses were observed. Median [interquartile range (IQR)] time from GCA diagnosis to MTX initiation in cases was 39 (13-80) weeks and the median (IQR) starting dose was 13.5 (10-15) mg/week. RR comparing relapse rates before and after MTX initiation/index date were significantly reduced in both cases (RR 0.32, 95% CI 0.24-0.41) and controls (RR 0.60, 95% CI 0.43-0.86). The decrease in relapse rate was significantly greater in patients taking MTX than in those taking GC alone (p = 0.004). Rates of GC discontinuation did not differ between groups.
CONCLUSION: In this large single-institution cohort, the addition of MTX to GC decreased the rate of subsequent relapse by nearly 2-fold compared to patients taking GC alone. MTX may be considered as adjunct therapy in patients with GCA to decrease the risk of further relapse events.

PMID: 30647171 [PubMed - as supplied by publisher]

Increased Incidence of Giant Cell Arteritis in Urban Areas?

Actualités En Médecine Interne - jeu, 17/01/2019 - 15:50
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Increased Incidence of Giant Cell Arteritis in Urban Areas?

J Rheumatol. 2019 Jan 15;:

Authors: Brekke LK, Fevang BT, Myklebust G

Abstract
Giant cell arteritis (GCA) is the most common systemic vasculitis in adults. The pathogenesis and the etiology of the disease are not fully understood, and environmental factors, which may influence the incidence and prevalence, are poorly investigated. Only a few small studies have previously addressed the potential influence of rural or urban residence on the occurrence of GCA1,2,3.

PMID: 30647168 [PubMed - as supplied by publisher]

Evaluation of Corneal Parameters with Dual Scheimpflug Imaging in Patients with Systemic Sclerosis.

Actualités En Médecine Interne - jeu, 17/01/2019 - 15:50
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Evaluation of Corneal Parameters with Dual Scheimpflug Imaging in Patients with Systemic Sclerosis.

Curr Eye Res. 2018 04;43(4):451-454

Authors: Gomes BF, Santhiago MR, Kara-Junior N, Moraes HV

Abstract
PURPOSE: To evaluate the cornea of systemic sclerosis (SSc) patients with Dual Scheimpflug Imaging.
METHODS: Twenty consecutive SSc patients and 20 age and sex matched controls were enrolled in this cross-sectional study. Corneal measurements were acquired by dual Scheimpflug analyzer.
RESULTS: SSc patients had statistically significant steeper corneas than the control group. The mean anterior curvature-average (SimK) was 44.93 ± 1.64 D (mean ± standard deviation) in SSc and 43.61 ± 0.99D in control group, p = 0.01. Posterior curvature was also steeper in SSc patients compared to controls (p = 0.02). There was no statistically significant difference regarding central average pachymetry (p = 0.07), thinnest pachymetry (p = 0.09).
CONCLUSIONS: Patients with SSc present with steeper corneas than controls.

PMID: 29336614 [PubMed - indexed for MEDLINE]

Disseminated toxoplasmosis with atypical symptoms which developed with exacerbation of systemic lupus erythematosus.

Vascularites - mer, 16/01/2019 - 20:50
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Disseminated toxoplasmosis with atypical symptoms which developed with exacerbation of systemic lupus erythematosus.

Lupus. 2019 Jan;28(1):133-136

Authors: Furuya H, Ikeda K, Iida K, Suzuki K, Furuta S, Tamachi T, Suzuki K, Miura G, Hiraguri M, Hase R, Hikosaka K, Norose K, Nakajima H

Abstract
Toxoplasma is a common parasite worldwide that mainly affects the brain, lungs and eyes. Although toxoplasmic encephalitis is a lethal disease without treatment, past case reports show most patients with systemic lupus erythematosus who developed toxoplasmic encephalitis were misdiagnosed and treated as neuropsychiatric systemic lupus erythematosus, which led to unfavorable outcomes. We herein describe a case of disseminated toxoplasmosis affecting all the above organs with atypical symptoms, which developed with exacerbation of systemic lupus erythematosus. She had initially manifested with retinochoroiditis without vitritis, mild cognitive impairment and an isolated lung mass. These are completely different from the classic symptoms of toxoplasmosis that have been reported in patients with HIV infection and/or those after hematopoietic transplantation. Our case, together with previously reported cases, suggests the manifestation of toxoplasmosis that develops in systemic lupus erythematosus patients can be different from that seen in conventional cases and varies between individual patients. Our case highlights both the difficulty in and the importance of diagnosing toxoplasmosis in patients with systemic lupus erythematosus and provides helpful information to identify this rare, devastating, yet treatable disease.

PMID: 30486727 [PubMed - indexed for MEDLINE]

Bacterial endocarditis manifesting as autoimmune pulmonary renal syndrome: ANCA-associated lung hemorrhage and pauci-immune crescentic glomerulonephritis
.

Vascularites - mer, 16/01/2019 - 20:50
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Bacterial endocarditis manifesting as autoimmune pulmonary renal syndrome: ANCA-associated lung hemorrhage and pauci-immune crescentic glomerulonephritis
.

Clin Nephrol. 2018 Dec;90(6):431-433

Authors: Mohandes S, Satoskar A, Hebert L, Ayoub I

Abstract
The etiology of pulmonary renal syndrome can be broadly divided into infectious and autoimmune (predominantly ANCA vasculitis). The importance of timely differentiating between them stems from the deleterious effects of their respective treatment if misdiagnosed. Serology and tissue evaluation by pathology are employed to aid in this, however, in rare cases, this can be difficult. We present a case of infectious endocarditis that presented with pulmonary renal syndrome but had positive ANCA serology and a pauci-immune glomerulonephritis picture on kidney biopsy that posed diagnostic difficulty. Factors most helpful in differentiating between the two conditions are highlighted as well as treatment options.
.

PMID: 30369400 [PubMed - indexed for MEDLINE]

ANCA-associated vasculitis in scleroderma: A renal perspective
.

Vascularites - mer, 16/01/2019 - 20:50
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ANCA-associated vasculitis in scleroderma: A renal perspective
.

Clin Nephrol. 2018 Dec;90(6):413-418

Authors: Kant S, Shah AA, Hummers LK, Wigley FM, Geetha D

Abstract
AIMS: Overlap syndrome of ANCA-associated vasculitis (AAV) and scleroderma (SSc) is rare with conflicting data on renal outcomes. We describe the clinical characteristics and treatment outcome of ANCA-associated glomerulonephritis (AAG) in SSc patients followed at a single center.
MATERIALS AND METHODS: We conducted a retrospective study of 3,570 patients in our SSc database to identify SSc patients who subsequently developed AAV with renal involvement. Patient demographics, serology, renal function, renal histology, and treatment outcomes were assessed.
RESULTS: Of the 3,570 patients, we identified 7 patients who developed acute glomerulonephritis, and all were ANCA positive. The mean age at SSc diagnosis was 47 years, 4 patients were female, and 6 had diffuse SSc. Anti-nuclear antibody (ANA) was positive in all. Mean time of onset of AAV from time of diagnosis of SSc was 6 years, and all were myeloperoxidase (MPO) positive. Patients presented with hematuria, proteinuria, with or without rise in serum creatinine, and all patients had biopsy-proven crescentic glomerulonephritis. One patient required dialysis at presentation. Five patients were treated with cyclophosphamide and steroids, and 2 were treated with rituximab and steroids. Of the 7 patients, 4 did not receive maintenance immunosuppression. Three patients died, and 1 of them experienced relapse with fulminant alveolar hemorrhage.
CONCLUSION: AAG in SSc is rare, with disease manifestation and course similar to that of AAV. This case series demonstrates that disease remission can be achieved with standard induction therapy. Vasculitis relapse can occur, and similar to idiopathic AAV, maintenance immunosuppression should be initiated to maintain remission.
.

PMID: 30106367 [PubMed - indexed for MEDLINE]

Serum-soluble TRAIL: a potential biomarker for disease activity in myositis patients.

Actualités En Médecine Interne - mer, 16/01/2019 - 14:11

Serum-soluble TRAIL: a potential biomarker for disease activity in myositis patients.

Clin Rheumatol. 2019 Jan 15;:

Authors: Zhou H, Wang Y, Bi K, Qi H, Song S, Zhou M, Chen L, Wang G, Duan T

Abstract
OBJECTIVES: Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) is a member of the TNF super-family, which is involved in the regulation of immune response and pathogenesis of autoimmune diseases, including polymyositis (PM) and dermatomyositis (DM). In this study, we examined the level and origin of serum-soluble TRAIL (sTRAIL) in patients with PM and DM and analyzed its association with disease activity and clinical features.
METHOD: 11 PM patients, 33 DM patients, and 20 healthy controls were enrolled in this study. Clinical features were recorded when admitted, and disease activity was evaluated by myositis disease activity assessment visual analogue scale (MYOACT). TRAIL expression in muscle tissues was detected by immunohistochemistry. Serum sTRAIL levels were measured by enzyme-linked immunosorbent assay. The expression of membrane TRAIL (mTRAIL) and its receptors, including DR4 and DR5, on circulating T cells was analyzed by flow cytometry.
RESULTS: TRAIL was expressed in infiltrated inflammatory cells in muscle tissues from patients. The serum sTRAIL level was markedly increased in patients and was positively correlated with the disease activity. Serum sTRAIL was decreased after therapy in patients and was specifically higher in patients with dysphagia, but lower in patients with autoantibody Jo-1 positive. The frequency of mTRAIL and its receptors on circulating T cells from patients were significantly elevated than that from healthy controls.
CONCLUSIONS: The serum sTRAIL could be a biomarker for evaluating the disease activity of PM and DM, and targeting the generation of TRAIL in T cells might be a potential approach in the treatment of PM and DM.

PMID: 30645753 [PubMed - as supplied by publisher]

Novel Biomarkers for the Precisive Diagnosis and Activity Classification of Takayasu Arteritis.

Actualités En Médecine Interne - mer, 16/01/2019 - 14:11

Novel Biomarkers for the Precisive Diagnosis and Activity Classification of Takayasu Arteritis.

Circ Genom Precis Med. 2019 Jan;12(1):e002080

Authors: Cui X, Qin F, Song L, Wang T, Geng B, Zhang W, Jin L, Wang W, Li S, Tian X, Zhang H, Cai J

Abstract
BACKGROUND: Establishing the diagnosis and determining disease activity of Takayasu arteritis (TA) remains challenging. Novel biomarkers might help to solve this problem.
METHODS: In the screening phase, by using large-scale protein arrays detecting samples from 90 subjects (TA active, 29; TA inactive 31; and controls, 30). In the validation phase, by using enzyme-linked immunosorbent assay (ELISA), potential biomarkers for TA diagnosis, and activity classification were measured in independent cohorts, respectively.
RESULTS: In the screening phase, 18 cytokines significantly differentially enriched between TA patients and controls and another 15 cytokines significantly differentially enriched between TA patient in active and inactive status were identified (adjusted P<0.05). In the validation phase, TIMP (tissue inhibitor of metalloproteinases)-1 was identified as a specific biomarker for TA diagnosis that a cutoff value of 221.86 μg/L could provide a specificity of 89.58% and a positive predictive value of 0.92. Meanwhile, we found it unreliable to use a single biomarker for TA activity classification. Considering this, we further built a logistic regression model based on multiple cytokines, including CA (cancer antigen) 125, FLRG (follistatin-related protein), IGFBP (insulin-like growth factor-binding protein)-2, CA15-3, GROa (growth-regulated alpha protein), LYVE (lymphatic vessel endothelial hyaluronic acid receptor)-1, ULBP (UL16-binding protein)-2, and CD (cluster of differentiation) 99, with an area under the curve reaching 0.909 for discriminating TA activity status.
CONCLUSIONS: This study suggested TIMP-1 as a specific biomarker for TA diagnosis with a cutoff value of 221.86 μg/L. Furthermore, we provided a logistic regression model based on 8 biomarkers for the precisive activity classification of TA with an area under the curve of 0.909.

PMID: 30645172 [PubMed - in process]

Thromboinflammation: Challenges of Therapeutically Targeting Coagulation and other Host Defence Mechanisms.

Actualités En Médecine Interne - mer, 16/01/2019 - 14:11
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Thromboinflammation: Challenges of Therapeutically Targeting Coagulation and other Host Defence Mechanisms.

Blood. 2019 Jan 14;:

Authors: Jackson SP, Darbousset R, Schoenwaelder SM

Abstract
Thrombosis with associated inflammation (thromboinflammation) occurs commonly in a broad range of human disorders. It is well recognized clinically in the context of superficial thrombophlebitis (thrombosis and inflammation of superficial veins), however it is more dangerous when it develops in the microvasculature of injured tissues and organs. Microvascular thrombosis with associated inflammation is well recognized in the context of sepsis and ischemia-reperfusion injury, however it also occurs in organ transplant rejection, major trauma, severe burns, the antiphospholipid syndrome, preeclampsia, sickle cell disease and biomaterial-induced thromboinflammation. Central to thromboinflammation is the loss of the normal antithrombotic and anti-inflammatory functions of endothelial cells, leading to dysregulation of coagulation, complement, platelet activation and leukocyte recruitment in the microvasculature. α-thrombin plays a critical role in co-ordinating thrombotic and inflammatory responses and has long been considered an attractive therapeutic target to reduce thromboinflammatory complications. This review focuses on the role of basic aspects of coagulation and α-thrombin in promoting thromboinflammatory responses, and discusses insights gained from clinical trials on the effects of various inhibitors of coagulation on thromboinflammatory disorders. Studies in sepsis patients have been particularly informative, in that despite using anticoagulant approaches with different pharmacological profiles, which act at distinct points in the coagulation cascade, bleeding complications continue to undermine clinical benefit. Future advances may require the development of therapeutics with primary anti-inflammatory and cytoprotective properties, that have less impact on hemostasis. This may be possible with the growing recognition that components of blood coagulation and platelets have prothrombotic and proinflammatory functions independent of their hemostatic effects.

PMID: 30642917 [PubMed - as supplied by publisher]

Autoimmune myelofibrosis: a rare haematological involvement in systemic lupus erythematosus.

Actualités En Médecine Interne - mer, 16/01/2019 - 14:11
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Autoimmune myelofibrosis: a rare haematological involvement in systemic lupus erythematosus.

BMJ Case Rep. 2019 Jan 14;12(1):

Authors: Belfeki N, Shankarasivam G, Declerck D, Diamantis S

Abstract
Autoimmune myelofibrosis is a distinct clinicopathological entity that occurs with autoimmune disorders. We report the case of a 44-year-old woman admitted with pancytopenia and clinical features of systemic lupus erythematosus, Sjogren's syndrome and antiphospholipid antibodies syndrome. Bone marrow biopsy showed decreased global cells and an increase of reticulin fibres on argentic coloration, consistent with myelofibrosis. The JAK2 V617, Myeloproliferative leukemia (MPL) and calreticulin mutations were negative. The patient's condition improved after treatment with hydroxychloroquine, vitamin K antagonists and prednisone.

PMID: 30642862 [PubMed - in process]

Type I Interferon Signaling Is Required for Dacryoadenitis in the Nonobese Diabetic Mouse Model of Sjögren Syndrome.

Actualités En Médecine Interne - mer, 16/01/2019 - 14:11
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Type I Interferon Signaling Is Required for Dacryoadenitis in the Nonobese Diabetic Mouse Model of Sjögren Syndrome.

Int J Mol Sci. 2018 Oct 20;19(10):

Authors: Chaly Y, Barr JY, Sullivan DA, Thomas HE, Brodnicki TC, Lieberman SM

Abstract
Nonobese diabetic (NOD) mice spontaneously develop lacrimal and salivary gland autoimmunity similar to human Sjögren syndrome. In both humans and NOD mice, the early immune response that drives T-cell infiltration into lacrimal and salivary glands is poorly understood. In NOD mice, lacrimal gland autoimmunity spontaneously occurs only in males with testosterone playing a role in promoting lacrimal gland inflammation, while female lacrimal glands are protected by regulatory T cells (Tregs). The mechanisms of this male-specific lacrimal gland autoimmunity are not known. Here, we studied the effects of Treg depletion in hormone-manipulated NOD mice and lacrimal gland gene expression to determine early signals required for lacrimal gland inflammation. While Treg-depletion was not sufficient to drive dacryoadenitis in castrated male NOD mice, chemokines (Cxcl9, Ccl19) and other potentially disease-relevant genes (Epsti1, Ubd) were upregulated in male lacrimal glands. Expression of Cxcl9 and Ccl19, in particular, remained significantly upregulated in the lacrimal glands of lymphocyte-deficient NOD-severe combined immunodeficiency (SCID) mice and their expression was modulated by type I interferon signaling. Notably, Ifnar1-deficient NOD mice did not develop dacryoadenitis. Together these data identify disease-relevant genes upregulated in the context of male-specific dacryoadenitis and demonstrate a requisite role for type I interferon signaling in lacrimal gland autoimmunity in NOD mice.

PMID: 30347820 [PubMed - indexed for MEDLINE]

((((((thrombophlebitis) OR takayasu) OR temporal arteritis) OR sarcoidosis) OR myositis) OR scleroderma) OR sjogren's disease; +23 new citations

Actualités En Médecine Interne - mar, 15/01/2019 - 15:34

23 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

((((((thrombophlebitis) OR takayasu) OR temporal arteritis) OR sarcoidosis) OR myositis) OR scleroderma) OR sjogren's disease

These pubmed results were generated on 2019/01/15

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Necrosis of the Fingers and Toes.

Vascularites - dim, 13/01/2019 - 18:22
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Necrosis of the Fingers and Toes.

N Engl J Med. 2018 Dec 27;379(26):2557

Authors: Taniguchi Y, Inotani S

PMID: 30586520 [PubMed - indexed for MEDLINE]

Phenotypic Characterization of Circulating CD4+ T Cells in ANCA-Associated Vasculitis.

Vascularites - dim, 13/01/2019 - 18:22
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Phenotypic Characterization of Circulating CD4+ T Cells in ANCA-Associated Vasculitis.

J Immunol Res. 2018;2018:6984563

Authors: Lilliebladh S, Johansson Å, Pettersson Å, Ohlsson S, Hellmark T

Abstract
T cell-mediated immune responses are thought to play an important role in the pathogenesis of anti-neutrophil cytoplasmic antibody- (ANCA-) associated vasculitides (AAV). CD4+ T cells can be divided into subsets depending on their expression of chemokine receptors. In this study, different CD4+ T cell populations in patients with AAV were analysed and compared to healthy blood donors as well as therapy controls. 18 patients with active AAV, 46 in remission, 21 healthy controls (HBD), and 15 therapy controls (TC) were enrolled. CD4+ T cells were divided into Th1, Th2, and Th17 cells and further subdivided into naïve, central memory, effector memory, and effector cells. Regulatory T cells were also analysed. Concentrations of cytokines and chemokines produced by the respective CD4+ T cell subset in plasma from 33 of the patients were measured by ELISA and compared to HBD. Clinical data were collected on all patients. CCL20 concentrations and percentages of Th17 cells (p = 0.019) were elevated in AAV patients compared to HBD. AAV patients had lower percentages of naïve CD4+ T cells (p = 0.0016) and a corresponding increase in proportion of effector memory CD4+ T cells when comparing to HBD (p = 0.027). Therapy controls showed similar results as AAV patients. In this study, we found that CD4+ T cell phenotype distribution is altered in AAV patients, in line with previously published work. However, no differences were found between AAV patients and TC, stressing the importance of treatment impact on this kind of studies.

PMID: 30510966 [PubMed - indexed for MEDLINE]

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