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Unique glandular ex-vivo Th1 and Th17 receptor motifs in Sjögren's syndrome patients using single-cell analysis.

Actualités En Médecine Interne - dim, 22/04/2018 - 14:18
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Unique glandular ex-vivo Th1 and Th17 receptor motifs in Sjögren's syndrome patients using single-cell analysis.

Clin Immunol. 2018 Apr 18;:

Authors: Voigt A, Bohn K, Sukumaran S, Stewart CM, Bhattacharya I, Nguyen CQ

Abstract
Primary Sjögren's syndrome (pSS) is an autoimmune disease in which the underlying cause has yet to be elucidated. The main objective of this study was to determine the T cell receptor (TCR) repertoires of individual infiltrating T helper (Th)-1 and 17 cells of pSS patients using single-cell analysis. Single-cell analysis of ex-vivo infiltrating T cells demonstrated that pSS patients had higher frequencies of activated Th17 cells. Single-cell TCR sequencing revealed that TCRβ variable (TRBV)3-1/joint (J)1-2 (CLFLSMSACVW) and TRBV20-1/J1-1 (SVGSTAIPP*T) were expressed by activated Th1 and Th17 cells in both cohorts. Uniquely, TCRα variable (TRAV)8-2/J5 (VVSDTVLETAGE) was expressed by Th1 cells present only in patients and complementarity-determining region (CDR)3α-specific motif (LSTD*E) present in both Th1/Th17 cells. The study demonstrates that both activated Th1 and Th17 cells of pSS patients showed restricted clonal diversities of which two CDR3 motifs were present in controls and patients, with another two motifs unique to pSS.

PMID: 29679709 [PubMed - as supplied by publisher]

Proangiogenic and Profibrotic Markers in Pulmonary Sarcoidosis.

Actualités En Médecine Interne - dim, 22/04/2018 - 14:18
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Proangiogenic and Profibrotic Markers in Pulmonary Sarcoidosis.

Adv Exp Med Biol. 2018 Apr 21;:

Authors: Tuleta I, Biener L, Pizarro C, Nickenig G, Skowasch D

Abstract
The aim of our study was to determine the blood levels of vascular endothelial growth factor (VEGF), transforming growth factor (TGF)-β1, fibroblast growth factor (FGF)-2, and platelet-derived growth factor (PDGF)-AB in different stages of pulmonary sarcoidosis. There were 92 patients in sarcoidosis stages I + II, III, and IV enrolled into the study. All the patients underwent lung diffusing capacity and blood sampling. We found that VEGF levels differed significantly between the stage groups with the peak VEGF concentrations in stage III. TGF-β1 levels were similar in stages I + II and III, and tended to be lower in stage IV. The analysis of the subgroups showed increased VEGF and FGF-2, and reduced TGF-β1 concentration in stages I + II patients with relevantly reduced lung diffusing capacity or increased sarcoidosis activity compared to patients with normal lung diffusing capacity or inactive sarcoidosis. A tendency towards increased VEGF, PDGF-AB and TGF-β1 levels was observed in the analogical subgroup analysis within the stage III. We conclude that proangiogenic VEGF, and profibrotic FGF-2 and PDGF-AB may contribute to the progression of sarcoidosis, whereas TGF-β1, with its dual anti-inflammatory and profibrotic actions, may play a dichotomous protective or deleterious role. Reduced diffusing capacity and active sarcoidosis are associated with an unfavorable constellation of the markers studied, which predicts a progressive disease course.

PMID: 29679363 [PubMed - as supplied by publisher]

Autoantibodies and scleroderma phenotype define subgroups at high-risk and low-risk for cancer.

Actualités En Médecine Interne - dim, 22/04/2018 - 14:18
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Autoantibodies and scleroderma phenotype define subgroups at high-risk and low-risk for cancer.

Ann Rheum Dis. 2018 Apr 20;:

Authors: Igusa T, Hummers LK, Visvanathan K, Richardson C, Wigley FM, Casciola-Rosen L, Rosen A, Shah AA

Abstract
OBJECTIVES: Recent studies demonstrate autoantibodies are powerful tools to interrogate molecular events linking cancer and the development of autoimmunity in scleroderma. Investigating cancer risk in these biologically relevant subsets may provide an opportunity to develop personalised cancer screening guidelines. In this study, we examined cancer risk in distinct serologic and phenotypic scleroderma subsets and compared estimates with the general population.
METHODS: Patients in the Johns Hopkins Scleroderma Center observational cohort were studied. Overall and site-specific cancer incidence was calculated in distinct autoantibody and scleroderma phenotypic subsets, and compared with the Surveillance, Epidemiology and End Results registry, a representative sample of the US population.
RESULTS: 2383 patients with scleroderma contributing 37 686 person-years were studied. 205 patients (8.6%) had a diagnosis of cancer. Within 3 years of scleroderma onset, cancer risk was increased in patients with RNA polymerase III autoantibodies (antipol; standardised incidence ratio (SIR) 2.84, 95% CI 1.89 to 4.10) and those lacking centromere, topoisomerase-1 and pol antibodies (SIR 1.83, 95% CI 1.10 to 2.86). Among antipol-positive patients, cancer-specific risk may vary by scleroderma subtype; those with diffuse scleroderma had an increased breast cancer risk, whereas those with limited scleroderma had high lung cancer risk. In contrast, patients with anticentromere antibodies had a lower risk of cancer during follow-up (SIR 0.59, 95% CI 0.44 to 0.76).
CONCLUSIONS: Autoantibody specificity and disease subtype are biologically meaningful filters that may inform cancer risk stratification in patients with scleroderma. Future research testing the value of targeted cancer screening strategies in patients with scleroderma is needed.

PMID: 29678941 [PubMed - as supplied by publisher]

Diagnostic Value of Vitamin D Status and Bone Turnover Markers in Rheumatoid Arthritis Complicated by Osteoporosis.

Actualités En Médecine Interne - dim, 22/04/2018 - 14:18
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Diagnostic Value of Vitamin D Status and Bone Turnover Markers in Rheumatoid Arthritis Complicated by Osteoporosis.

Ann Clin Lab Sci. 2018 Mar;48(2):197-204

Authors: Tan LM, Long TT, Guan XL, Wu SF, Zheng W, Fu HY, Wang QH, Meng YM, Wu Y, Zeng TT, Tian YJ, Yu JL, Chen JJ, Li H, Cao LP

Abstract
OBJECTIVE: To research the diagnostic performance of clinical potential bone turnover indexes in rheumatoid arthritis (RA) complicated with osteoporosis (OP).
METHODS: This study involved 87 RA patients, 48 with OP, and 39 without OP, and 204 non-RA control patients, including those with systemic lupus erythematosus, ankylosing spondylitis, primary Sjogren's syndrome, systemic sclerosis, and healthy patients. The levels of 25-hydroxyvitamin D [25(OH)D], β-crosslaps (β-CROSSL), parathyroid hormone (PTH) were measured by electrochemiluminescence (ECLIA), and the level of bone alkaline phosphatase (BALP) was measured by lectin affinity method.
RESULTS: The serum concentration of 25(OH)D in the RA with OP group was significantly lower than the control group (P<0.01), while the levels of β-CROSSL, BALP in the RA with OP group considerably exceeded those found in the control group (P<0.01). The levels of β-CROSSL and PTH were significantly higher in RA patients with OP than without OP (P<0.01), while the level of 25(OH)D was statistically lower than without OP (P<0.01). An unconditional logistical regression analysis proved an association with low 25(OH)D and elevated β-CROSSL in RA with OP, with 25(OH)D demonstrating greatest diagnostic potential according to the ROC curve.
CONCLUSION: The significantly reduced levels of 25(OH)D and excessive β-CROSSL may indicate a high risk of the secondary osteoporosis in RA patients.

PMID: 29678847 [PubMed - in process]

[Dermatologic toxicities of immune checkpoint inhibitors].

Actualités En Médecine Interne - dim, 22/04/2018 - 14:18
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[Dermatologic toxicities of immune checkpoint inhibitors].

Ann Dermatol Venereol. 2018 Apr 17;:

Authors: Sibaud V, Boulinguez S, Pagès C, Riffaud L, Lamant L, Chira C, Boyrie S, Vigarios E, Tournier E, Meyer N

Abstract
The development of immune checkpoint inhibitors (monoclonal antibodies targeting PD-1/PD-L1 or CTLA-4) represents a significant advance in the treatment of multiple cancers. Given their particular mechanism of action, which involves triggering CD4+/CD8+ T-cell activation and proliferation, they are associated with a specific safety profile. Their adverse events are primarily immune-related, and can affect practically all organs. In this context, dermatological toxicity is the most common, though it mostly remains mild to moderate and does not require discontinuation of treatment. More than a third of patients are faced with cutaneous adverse events, usually in the form of a maculopapular rash, pruritus or vitiligo (only in patients treated for melanoma). Much more specific dermatologic disorders, however, may occur such as lichenoid reactions, induced psoriasis, sarcoidosis, auto-immune diseases (bullous pemphigoid, dermatomyositis, alopecia areata), acne-like rash, xerostomia, etc. Rigorous dermatological evaluation is thus mandatory in the case of atypical, persistent/recurrent or severe lesions. In this article, we review the incidence and spectrum of dermatologic adverse events reported with immune checkpoint inhibitors. Finally, a management algorithm is proposed.

PMID: 29678394 [PubMed - as supplied by publisher]

Efficacy of tocilizumab in Takayasu arteritis: Multicenter retrospective study of 46 patients.

Actualités En Médecine Interne - dim, 22/04/2018 - 14:18
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Efficacy of tocilizumab in Takayasu arteritis: Multicenter retrospective study of 46 patients.

J Autoimmun. 2018 Apr 17;:

Authors: Mekinian A, Resche-Rigon M, Comarmond C, Soriano A, Constans J, Alric L, Jego P, Busato F, Cabon M, Dhote R, Estibaliz L, Pault IK, Landron C, Lavigne C, Lioger B, Michaud M, Ruivard M, Sacre K, Gottenberg JE, Gaches F, Goulenok T, Salvarani C, Cacoub P, Fain O, Saadoun D, French Takayasu network

Abstract
OBJECTIVES: To assess the efficacy of tocilizumab in patients with Takayasu arteritis (TA).
METHODS: We conducted a retrospective multicenter study in 46 TA patients treated with tocilizumab. We analyzed factors associated with response to tocilizumab (assessed using NIH score).
RESULTS: Forty-six patients with TA were included, with a median age of 43 years [29-54], and 35 (76%) females. We observed a decrease in the median NIH scale (from 3 [2-3] at baseline to 0 [0-1] and 0 at 3 and 6 months, respectively; p < 0.0001). The daily prednisone dose also decreased from 15 mg [8-19] at baseline to 4 mg [5-21] and 5 mg [4.5-9] at 3 and 6 months, respectively (p < 0.0001) under tocilizumab. The overall tocilizumab failure free survival was 81% [CI 95%; 0.7-0.95], 72% [CI 95%; 0.55-0.95] and 48% [CI 95%; 0.2-0.1] at 12, 24 and 48 months, respectively. The presence of constitutional symptoms (HR 5.6 [CI 95%; 1.08-29], p = 0.041), and C-reactive protein level (HR 1.16 [CI 95%; 1.01-1.31], P = 0.003) at the time of tocilizumab initiation were significantly associated with tocilizumab event-free survival. The event-free survival was significantly better under tocilizumab therapy in comparison to DMARDs (p = 0.02).
CONCLUSION: This large multicenter study shows that tocilizumab is efficient and may reduce the incidence of relapses in TA.

PMID: 29678346 [PubMed - as supplied by publisher]

Tocilizumab for the treatment of giant cell arteritis.

Actualités En Médecine Interne - sam, 21/04/2018 - 12:37
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Tocilizumab for the treatment of giant cell arteritis.

Expert Rev Clin Immunol. 2018 Apr 20;:

Authors: Schirmer M, Muratore F, Salvarani C

Abstract
INTRODUCTION: Giant cell arteritis (GCA) is the most frequent type of vasculitis, occurring in people older than 50 years. So far, treatment has been limited to corticosteroids and methotrexate only. Areas covered: Interleukin-6 (IL-6) plays a role in the pathophysiology of GCA. This review covers recent advances in the treatment of GCA with tocilizumab (TCZ), which specifically binds to both soluble and membrane-bound IL-6R and inhibits IL-6R-mediated signaling. Expert commentary: Two randomized controlled trials recently showed the efficacy of the IL-6 receptors inhibitor monoclonal antibody TCZ for the induction and maintenance of remission in patients with new-onset and relapsing GCA. Furthermore, addition of TCZ to prednisone led to a reduction in the cumulative prednisone doses required to control GCA. The profile of adverse events was balanced across treatment groups and no safety concerns were raised during the trial.

PMID: 29676602 [PubMed - as supplied by publisher]

Leonine facies presenting as scleromyxedema.

Actualités En Médecine Interne - sam, 21/04/2018 - 12:37
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Leonine facies presenting as scleromyxedema.

Arthritis Rheumatol. 2018 Apr 20;:

Authors: Park AY, Lowe L, Khanna D

Abstract
A 61-year-old man previously in good health was referred to Scleroderma Clinic for a 2-year history of slowly progressive cutaneous eruption involving his dorsal hands, extremities, and central face. On physical examination, the patient had nodular, erythematous indurated lesions of his forehead and erythematous papular lesions on his nose with coalescence of firm erythematous papulonodules, resulting in a leonine facies. On the dorsal hands, arms, and legs were numerous, shiny, firm, closely set, 1-2 mm slightly translucent papules with background erythema (face and hand, shown in Panel A). This article is protected by copyright. All rights reserved.

PMID: 29676525 [PubMed - as supplied by publisher]

Diagnostic positron emission tomography-computed tomography in clinically elusive giant cell arteritis.

Actualités En Médecine Interne - sam, 21/04/2018 - 12:37
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Diagnostic positron emission tomography-computed tomography in clinically elusive giant cell arteritis.

Indian J Ophthalmol. 2018 May;66(5):693-694

Authors: Mohamed R, Djama D, Ayoub T

PMID: 29676318 [PubMed - in process]

Prognosis and Monitoring of Giant Cell Arteritis and Associated Complications.

Actualités En Médecine Interne - sam, 21/04/2018 - 12:37
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Prognosis and Monitoring of Giant Cell Arteritis and Associated Complications.

Expert Rev Clin Immunol. 2018 Apr 20;:

Authors: Kermani TA, Warrington KJ

Abstract
INTRODUCTION: Giant cell arteritis (GCA) is the most common systemic vasculitis in people over the age of 50 years. Prospective imaging studies in GCA highlight the systemic nature of this vasculitis. Areas covered: This review summarizes literature using PubMed on complications of GCA and its treatment. Emphasis was placed on articles published within the past 5 years. Disease associated complications including vision loss from arteritic anterior ischemic optic neuropathy, large-artery stenoses and ischemia, and, aortic aneurysms and dissections. Glucocorticoids are effective but have serious adverse effects. Furthermore, relapses are frequent and treatment- or disease-associated damage may accrue. Tocilizumab is the first treatment that showed efficacy in a large randomized prospective trial as a glucocorticoid sparing agent for GCA. Patients with GCA are also at increased risk for multiple cardiovascular diseases and venous thromboembolism. Monitoring for large-vessel involvement, particularly late manifestations like aortic aneurysms is important. Expert commentary: Advances in, and the incorporation of, imaging in GCA have led to better recognition and diagnosis of patients with large-vessel involvement. Prompt treatment with glucocorticoids is essential in preventing the occurrence or progression of vision loss. Therapeutics that allow sustained remission and reduce vessel damage in patients with GCA will play a crucial role.

PMID: 29676201 [PubMed - as supplied by publisher]

Interobserver Reliability of Histopathologic Features for Distinguishing Between Cutaneous Polyarteritis Nodosa and Superficial Thrombophlebitis.

Actualités En Médecine Interne - sam, 21/04/2018 - 12:37
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Interobserver Reliability of Histopathologic Features for Distinguishing Between Cutaneous Polyarteritis Nodosa and Superficial Thrombophlebitis.

Histopathology. 2018 Apr 19;:

Authors: Hutachuda P, Hanamornroongruang S, Pattanaprichakul P, Chanyachailert P, Sitthinamsuwan P

Abstract
AIMS: Interobserver reliability of histopathologic features in differentiation between cutaneous polyarteritis nodosa (cPAN) and superficial thrombophlebitis (ST) by assessment of inter-rater agreement of five histologic features were investigated.
METHODS AND RESULTS: All sections of cPAN and ST were independently evaluated by three experienced pathologists and one resident of pathology. The studied histopathologic features included elastic fiber distribution in vascular wall, pattern of smooth muscle arrangement, internal elastic lamina pattern, fibrinoid necrosis, and luminal thrombosis. Agreement analysis was performed using kappa coefficient. Sensitivity, specificity, positive predictive value (PPV), positive likelihood ratio (PLR) and 95% confidence interval (95%CI) of the useful histopathologic features were analyzed. Of all 62 biopsies, 28 were cPAN and 34 were ST. Reproducibility between four observers was substantial agreement (к = 0.73). Elastic fiber distribution in vascular wall (к = 0.68), fibrinoid necrosis (к = 0.63), internal elastic lamina pattern (к = 0.51), and pattern of smooth muscle arrangement (к = 0.46) showed high specificity and PPV for differentiating between cPAN and ST. Pattern of smooth muscle arrangement, internal elastic lamina pattern, elastic fiber distribution in vascular wall may be obscured when extensive inflammation and necrosis occurs.
CONCLUSIONS: These aforementioned histopathologic features are useful in differentiation between cPAN and ST. Verhoeff-van Gieson (VVG) elastic stain is an important histochemical study for differentiating between cPAN and ST, particularly in cases with extensive inflammation and necrosis. This article is protected by copyright. All rights reserved.

PMID: 29675878 [PubMed - as supplied by publisher]

Multidetector CT of iatrogenic and self-inflicted vascular lesions and infections at the groin.

Actualités En Médecine Interne - sam, 21/04/2018 - 12:37
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Multidetector CT of iatrogenic and self-inflicted vascular lesions and infections at the groin.

Insights Imaging. 2018 Apr 19;:

Authors: Tonolini M, Ierardi AM, Carrafiello G, Laganà D

Abstract
The number and complexity of endovascular procedures performed via either arterial or venous access are steadily increasing. Albeit associated with higher morbidity compared to the radial approach, the traditional common femoral artery remains the preferred access site in a variety of cardiac, aortic, oncologic and peripheral vascular procedures. Both transarterial and venous cannulation (for electrophysiology, intravenous laser ablation and central catheterisation) at the groin may result in potentially severe vascular access site complications (VASC). Furthermore, vascular and soft-tissue groin infections may develop after untreated VASC or secondarily to non-sterile injections for recreational drug use. VASC and groin infections require rapid diagnosis and appropriate treatment to avoid further, potentially devastating harm. Whereas in the past colour Doppler ultrasound was generally used, in recent years cardiologists, vascular surgeons and interventional radiologists increasingly rely on pelvic and femoral CT angiography. Despite drawbacks of ionising radiation and the need for intravenous contrast, multidetector CT rapidly and consistently provides a panoramic, comprehensive visualisation, which is crucial for correct choice between conservative, endovascular and surgical management. This paper aims to provide radiologists with an increased familiarity with iatrogenic and self-inflicted VASC and infections at the groin by presenting examples of haematomas, active bleeding, pseudoaneurysms, arterial occlusion, arterio-venous fistula, endovenous heat-induced thrombosis, septic thrombophlebitis, soft-tissue infections at the groin, and late sequelae of venous injuries.
TEACHING POINTS: • Complications may develop after femoral arterial or venous access for interventional procedures. • Arterial injuries include bleeding, pseudoaneurysm, occlusion, arteriovenous fistula, dissection. • Endovenous heat-induced thrombosis is a specific form of iatrogenic venous complication. • Iatrogenic infections include groin cellulitis, abscesses and septic thrombophlebitis. • CT angiography reliably triages vascular access site complications and groin infections.

PMID: 29675625 [PubMed - as supplied by publisher]

Utility and safety of endobronchial ultrasound-guided transbronchial needle aspiration in patients with mediastinal and hilar lymphadenopathy: Western region experience.

Actualités En Médecine Interne - sam, 21/04/2018 - 12:37
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Utility and safety of endobronchial ultrasound-guided transbronchial needle aspiration in patients with mediastinal and hilar lymphadenopathy: Western region experience.

Ann Thorac Med. 2018 Apr-Jun;13(2):92-100

Authors: Aljohaney AA

Abstract
AIMS: The aim of the study was to evaluate the clinical utility and safety of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) in patients with mediastinal and hilar lymphadenopathy and to explicitly describe the utility of this procedure in patient's outcome.
METHODS: A retrospective review and analysis was conducted on 52 patients with mediastinal or hilar lymphadenopathy who underwent EBUS-TBNA from June 2012 to June 2016. All the patients were evaluated by computed tomography (CT) chest with contrast before EBUS examination. Enlarged mediastinal or hilar lymph node was defined as >1 cm short axis on the enhanced CT.
RESULTS: Among the 52 patients studied, 57.7% were presented with mediastinal or hilar lymphadenopathy for diagnosis and 42.3% presented with suspected mediastinal malignancy. Paratracheal stations were the most common site for puncture in 33 lymph nodes (43%). The best diagnostic yield was obtained from subcarinal stations and the lowest yield from the hilar stations. Surgical biopsies confirmed lymphoma in six patients, tuberculosis (TB) in three, sarcoidosis in two and one had metastatic adenocarcinoma of unknown primary. The sensitivity, specificity, positive predictive value, and negative predictive value of EBUS-TBNA for diagnosis of mediastinal and hilar lymph node abnormalities were 78.6%, 100%, 100%, and 80%, respectively. The diagnostic yield of EBUS-TBNA in malignant and benign conditions was 79.0%.
CONCLUSIONS: EBUS-TBNA is a safe and efficacious procedure which can be performed using conscious sedation with high yields. It can be used for the staging of malignancies as well as for the diagnosis of inflammatory and infectious conditions such as sarcoidosis and TB.

PMID: 29675060 [PubMed]

Risk factors and disease mechanisms in myositis.

Actualités En Médecine Interne - sam, 21/04/2018 - 12:37
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Risk factors and disease mechanisms in myositis.

Nat Rev Rheumatol. 2018 Apr 20;14(5):255-268

Authors: Miller FW, Lamb JA, Schmidt J, Nagaraju K

Abstract
Autoimmune diseases develop as a result of chronic inflammation owing to interactions between genes and the environment. However, the mechanisms by which autoimmune diseases evolve remain poorly understood. Newly discovered risk factors and pathogenic processes in the various idiopathic inflammatory myopathy (IIM) phenotypes (known collectively as myositis) have illuminated innovative approaches for understanding these diseases. The HLA 8.1 ancestral haplotype is a key risk factor for major IIM phenotypes in some populations, and several genetic variants associated with other autoimmune diseases have been identified as IIM risk factors. Environmental risk factors are less well studied than genetic factors but might include viruses, bacteria, ultraviolet radiation, smoking, occupational and perinatal exposures and a growing list of drugs (including biologic agents) and dietary supplements. Disease mechanisms vary by phenotype, with evidence of shared innate and adaptive immune and metabolic pathways in some phenotypes but unique pathways in others. The heterogeneity and rarity of the IIMs make advancements in diagnosis and treatment cumbersome. Novel approaches, better-defined phenotypes, and international, multidisciplinary consensus have contributed to progress, and it is hoped that these methods will eventually enable therapeutic intervention before the onset or major progression of disease. In the future, preemptive strategies for IIM management might be possible.

PMID: 29674613 [PubMed - in process]

Autoantibodies in myositis.

Actualités En Médecine Interne - sam, 21/04/2018 - 12:37
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Autoantibodies in myositis.

Nat Rev Rheumatol. 2018 Apr 20;14(5):290-302

Authors: McHugh NJ, Tansley SL

Abstract
The discovery of novel autoantigen systems related to idiopathic inflammatory myopathies (collectively referred to as myositis) in adults and children has had major implications for the diagnosis and management of this group of diseases across a wide range of medical specialties. Traditionally, autoantibodies found in patients with myositis are described as being myositis-specific autoantibodies (MSAs) or myositis-associated autoantibodies (MAAs), depending on their prevalence in other, related conditions. However, certain MSAs are more closely associated with extramuscular manifestations, such as skin and lung disease, than with myositis itself. It is very rare for more than one MSA to coexist in the same individual, underpinning the potential to use MSAs to precisely define genetic and disease endotypes. Each MSA is associated with a distinctive pattern of disease or phenotype, which has implications for diagnosis and a more personalized approach to therapy. Knowledge of the function and localization of the autoantigenic targets for MSAs has provided key insights into the potential immunopathogenic mechanisms of myositis. In particular, evidence suggests that the alteration of expression of a myositis-related autoantigen by certain environmental influences or oncogenesis could be a pivotal event linking autoantibody generation to the development of disease.

PMID: 29674612 [PubMed - in process]

Tricky case of Takayasu arteritis in a young child presenting with heart failure and femoral pulses.

Actualités En Médecine Interne - sam, 21/04/2018 - 12:37
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Tricky case of Takayasu arteritis in a young child presenting with heart failure and femoral pulses.

Arch Dis Child. 2018 Apr 19;:

Authors: Fabi M, Brighenti M, Donti A, Lanari M

PMID: 29674517 [PubMed - as supplied by publisher]

TBK1: A key regulator and potential treatment target for interferon positive Sjögren's syndrome, systemic lupus erythematosus and systemic sclerosis.

Actualités En Médecine Interne - sam, 21/04/2018 - 12:37
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TBK1: A key regulator and potential treatment target for interferon positive Sjögren's syndrome, systemic lupus erythematosus and systemic sclerosis.

J Autoimmun. 2018 Apr 16;:

Authors: Bodewes ILA, Huijser E, van Helden-Meeuwsen CG, Tas L, Huizinga R, Dalm VASH, van Hagen PM, Groot N, Kamphuis S, van Daele PLA, Versnel MA

Abstract
OBJECTIVE: Upregulation of type I interferons (IFN-I) is a hallmark of systemic autoimmune diseases like primary Sjögren's syndrome (pSS), systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). Expression of IFN-I is induced by three different receptor families: Toll-like receptors (TLRs), RIG-like receptors (RLRs) and DNA-sensing receptors (DSRs). TANK-binding kinase (TBK1) is an important signaling hub downstream of RLRs and DSRs. TBK1 activates IRF3 and IRF7, leading to IFN-I production and subsequent induction of interferon stimulated genes (ISGs). The objective of this study was to explore the potential of BX795, an inhibitor of TBK1, to downregulate IFN-I activation in pSS, SLE and SSc.
METHODS: TBK1, IRF3, IRF7 and STAT1 were determined by RT-PCR in PAXgene samples and phosphorylated-TBK1 (pTBK1) was analyzed by flowcytometry in plasmacytoid dendritic cells (pDCs) from IFN-I positive (IFNpos) patients. Peripheral blood mononuclear cells (PBMCs) of pSS, SLE and SSc patients and TLR7 stimulated PBMCs of healthy controls (HCs) were cultured with the TBK1 inhibitor BX795, followed by analysis of ISGs.
RESULTS: Increased gene expression of TBK1, IRF3, IRF7 and STAT1 in whole blood and pTBK1 in pDCs was observed in IFNpos pSS, SLE and SSc patients compared to HCs. Upon treatment with BX795, PBMCs from IFNpos pSS, SLE, SSc and TLR7-stimulated HCs downregulated the expression of the ISGs MxA, IFI44, IFI44L, IFIT1 and IFIT3.
CONCLUSIONS: TBK1 inhibition reduced expression of ISGs in PBMCs from IFNpos patients with systemic autoimmune diseases indicating TBK1 as a potential treatment target.

PMID: 29673738 [PubMed - as supplied by publisher]

Transient monocular blindness: Vascular causes and differential diagnoses.

Actualités En Médecine Interne - sam, 21/04/2018 - 12:37
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Transient monocular blindness: Vascular causes and differential diagnoses.

J Fr Ophtalmol. 2018 Apr 16;:

Authors: Bidot S, Biotti D

Abstract
Transient monocular blindness is an acute episode of ischemic origin in which one eye has profound visual loss, followed by full recovery within one hour. Transient monocular blindness most often occurs in the setting of retinal ischemia secondary to carotid embolism, but other mechanisms have been reported, including thrombosis (most often in the setting of giant cell arteritis), hemodynamic disorders (secondary to severe carotid stenosis), or vasospasm. Transient monocular blindness is considered a transient ischemic attack originating in the carotid arteries, and must be managed the same as transient ischemic attack involving the brain, in order to prevent a subsequent stroke.

PMID: 29673627 [PubMed - as supplied by publisher]

Comparing the importance of quality measurement themes in juvenile idiopathic inflammatory myositis between patients and families and healthcare professionals.

Actualités En Médecine Interne - sam, 21/04/2018 - 12:37
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Comparing the importance of quality measurement themes in juvenile idiopathic inflammatory myositis between patients and families and healthcare professionals.

Pediatr Rheumatol Online J. 2018 Apr 19;16(1):28

Authors: Tory HO, Carrasco R, Griffin T, Huber AM, Kahn P, Robinson AB, Zurakowski D, Kim S, CARRA Juvenile Dermatomyositis Quality Measures Workgroup

Abstract
BACKGROUND: A standardized set of quality measures for juvenile idiopathic inflammatory myopathies (JIIM) is not in use. Discordance has been shown between the importance ascribed to quality measures between patients and families and physicians. The objective of this study was to assess and compare the importance of various aspects of high quality care to patients with JIIM and their families with healthcare providers, to aid in future development of comprehensive quality measures.
METHODS: Surveys were developed by members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Juvenile Dermatomyositis Workgroup through a consensus process and administered to patients and families through the CureJM Foundation and to healthcare professionals through CARRA. The survey asked respondents to rate the importance of 19 items related to aspects of high quality care, using a Likert scale.
RESULTS: Patients and families gave generally higher scores for importance to most of the quality measurement themes compared with healthcare professionals, with ratings of 13 of the 19 measures reaching statistical significance (p < 0.05). Of particular importance, however, was consensus between the groups on the top five most important items: quality of life, timely diagnosis, access to rheumatology, normalization of functioning/strength, and ability for self care.
CONCLUSIONS: Despite overall differences in the rating of importance of quality indicators between patients and families and healthcare professionals, the groups agreed on the most important aspects of care. Recognizing areas of particular importance to patients and families, and overlapping in importance with providers, will promote the development of standardized quality measures with the greatest potential for improving care and outcomes for children with JIIM.

PMID: 29673367 [PubMed - in process]

Musculoskeletal Manifestations of Non-RA Connective Tissue Diseases: Scleroderma, Systemic Lupus Erythematosus, Still's Disease, Dermatomyositis/Polymyositis, Sjögren's Syndrome, and Mixed Connective Tissue Disease.

Actualités En Médecine Interne - ven, 20/04/2018 - 18:09
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Musculoskeletal Manifestations of Non-RA Connective Tissue Diseases: Scleroderma, Systemic Lupus Erythematosus, Still's Disease, Dermatomyositis/Polymyositis, Sjögren's Syndrome, and Mixed Connective Tissue Disease.

Semin Musculoskelet Radiol. 2018 Apr;22(2):166-179

Authors: Jacques T, Sudoł-Szopińska I, Larkman N, O'Connor P, Cotten A

Abstract
The most common systemic rheumatologic conditions are connective tissue diseases (including rheumatoid arthritis [RA]) followed by spondyloarthropathy. With the advent of biotherapies and imaging biomarkers, development in the imaging of RA and spondyloarthropathies has received substantial attention in the literature. This article details the various musculoskeletal imaging features of the other connective tissue diseases such as scleroderma and progressive systemic sclerosis, systemic lupus erythematosus, Still's disease, dermatomyositis and polymyositis, Sjögren's syndrome, and mixed connective tissue disease.

PMID: 29672805 [PubMed - in process]

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