Database : MEDLINE
Search on : mixed and connective and tissue and disease [Words]
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[PMID]: 29499223
[Au] Autor:Le Coz C; Trofa M; Syrett CM; Martin A; Jyonouchi H; Jyonouchi S; Anguera MC; Romberg N
[Ad] Address:Division of Immunology and Allergy, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
[Ti] Title:CD40LG duplication-associated autoimmune disease is silenced by non-random X-chromosome inactivation.
[So] Source:J Allergy Clin Immunol;, 2018 Feb 27.
[Is] ISSN:1097-6825
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:An inherited syndrome of autoimmunity associated with CD40LG duplication is silenced by non-random X-chromosome inactivation and is treatable with CD40L directed therapy.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180311
[Lr] Last revision date:180311
[St] Status:Publisher

  2 / 2951 MEDLINE  
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[PMID]: 29515069
[Au] Autor:Watanuki S; Kikuchi T; Toyama T; Abe R; Nakayama H; Karigane D; Shimizu T; Kikuchi J; Matsumoto K; Yasuoka H; Kataoka M; Okamoto S; Mori T
[Ad] Address:Division of Hematology, Department of Medicine, Keio University School of Medicine.
[Ti] Title:[Mixed connective tissue disease with pulmonary hypertension developing in a chronic myeloid leukemia patient on dasatinib treatment].
[So] Source:Rinsho Ketsueki;59(2):174-177, 2018.
[Is] ISSN:0485-1439
[Cp] Country of publication:Japan
[La] Language:jpn
[Ab] Abstract:A 37-year-old woman was diagnosed with chronic phase chronic myeloid leukemia. Nilotinib treatment was initiated; however, it had to be discontinued due to an allergic reaction one month later, and dasatinib treatment was provided. Although favorable response was obtained, she started complaining of shortness of breath 7 months after initiating dasatinib treatment. Chest X-ray and echocardiography indicated pulmonary congestion and hypertension. Further, she was diagnosed with mixed connective tissue disease (MCTD) based on Raynaud phenomenon, swollen fingers, sclerodactyly, pancytopenia, hypocomplementemia, and positive anti-U1-RNP antibody. Consequently, dasatinib treatment was discontinued, and she was administered prednisolone (1 mg/kg/day), which was effective and successfully tapered with concomitant administration of cyclophosphamide. This is the first case of MCTD that developed during dasatinib treatment. However, because the present case was a young woman, the development of MCTD could probably be attributed to autoimmune diatheses or it may be a coincidence. However, the possibility of patients receiving dasatinib treatment developing autoimmune diseases needs to be assessed.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:In-Data-Review
[do] DOI:10.11406/rinketsu.59.174

  3 / 2951 MEDLINE  
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[PMID]: 29447389
[Au] Autor:Bernstein EJ; Bathon JM; Lederer DJ
[Ad] Address:Department of Medicine, Columbia University Medical Center, New York, NY.
[Ti] Title:Survival of adults with systemic autoimmune rheumatic diseases and pulmonary arterial hypertension after lung transplantation.
[So] Source:Rheumatology (Oxford);, 2018 Feb 13.
[Is] ISSN:1462-0332
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Objectives: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in adults with systemic autoimmune rheumatic diseases (ARDs). The aim of this study was to determine whether adults with ARDs and PAH on right-sided heart catheterization (ARD-PAH) have increased mortality following lung transplantation compared with those with PAH not due to an ARD. Methods: We conducted a retrospective cohort study of 93 adults with ARD-PAH and 222 adults with PAH who underwent lung transplantation in the USA between 4 May 2005 and 9 March 2015 using data from the United Network for Organ Sharing. We examined associations between diagnosis and survival after lung transplantation using stratified Cox models adjusted for potential confounding recipient factors. Results: Among adults undergoing lung transplantation in the USA, we did not detect a difference in the multivariable-adjusted mortality rate between those with ARD-PAH and those with PAH [hazard ratio 0.75 (95% CI 0.47, 1.19)]. Conclusion: The presence of an ARD was not associated with increased mortality after lung transplantation in adults with PAH.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:Publisher
[do] DOI:10.1093/rheumatology/kex527

  4 / 2951 MEDLINE  
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[PMID]: 29507032
[Au] Autor:Gameiro RS; Reis AIA; Grilo AC; Noronha C
[Ad] Address:Medicina Interna, Hospital Beatriz Ângelo, Loures, Portugal.
[Ti] Title:Following leads: connecting dysphagia to mixed connective tissue disease.
[So] Source:BMJ Case Rep;2018, 2018 Mar 05.
[Is] ISSN:1757-790X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Mixed connective tissue disease (MCDT) is a rare condition characterised by the presence of high titres of anti-U1 ribonucleoprotein antibodies and selected clinical features of systemic lupus erythematosus, systemic sclerosis and polymyositis/dermatomyositis. Early symptoms are non-specific, including easy fatigability, myalgia, arthralgia and Raynaud's phenomenon. Some reports emphasised the favourable outcome and excellent response to glucocorticoids, but there are contradictory studies reporting worse prognosis. Also, a subset of patients evolve into a clinical picture more consistent with a major diffuse connective tissue disease. We present the case of a 50-year-old black woman whose inaugural presentation of MCDT was oropharyngeal dysphagia, symmetrical proximal muscle weakness, tongue atrophy and skin sclerosis. High-dose corticosteroids and methotrexate were given with little improvement, maintaining disabling dysphagia leading to a percutaneous endoscopic gastrostomy tube placement. She was then started on intravenous immunoglobulin with progressive remission of symptoms.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180306
[Lr] Last revision date:180306
[St] Status:In-Process

  5 / 2951 MEDLINE  
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[PMID]: 29353024
[Au] Autor:Gannon WD; Lederer DJ; Biscotti M; Javaid A; Patel NM; Brodie D; Bacchetta M; Baldwin MR
[Ad] Address:Division of Pulmonary, Allergy, and Critical Care Medicine, Columbia University College of Physicians and Surgeons/New York-Presbyterian Hospital, New York, NY.
[Ti] Title:Outcomes and Mortality Prediction Model of Critically Ill Adults With Acute Respiratory Failure and Interstitial Lung Disease.
[So] Source:Chest;, 2018 Jan 17.
[Is] ISSN:1931-3543
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: We aimed to examine short- and long-term mortality in a mixed population of patients with interstitial lung disease (ILD) with acute respiratory failure, and to identify those at lower vs higher risk of in-hospital death. METHODS: We conducted a single-center retrospective cohort study of 126 consecutive adults with ILD admitted to an ICU for respiratory failure at a tertiary care hospital between 2010 and 2014 and who did not undergo lung transplantation during their hospitalization. We examined associations of ICU-day 1 characteristics with in-hospital and 1-year mortality, using Poisson regression, and examined survival using Kaplan-Meier curves. We created a risk score for in-hospital mortality, using a model developed with penalized regression. RESULTS: In-hospital mortality was 66%, and 1-year mortality was 80%. Those with connective tissue disease-related ILD had better short-term and long-term mortality compared with unclassifiable ILD (adjusted relative risk, 0.6; 95% CI, 0.3-0.9; and relative risk, 0.6; 95% CI, 0.4-0.9, respectively). Our prediction model includes male sex, interstitial pulmonary fibrosis diagnosis, use of invasive mechanical ventilation and/or extracorporeal life support, no ambulation within 24 h of ICU admission, BMI, and Simplified Acute Physiology Score-II. The optimism-corrected C-statistic was 0.73, and model calibration was excellent (P = .99). In-hospital mortality rates for the low-, moderate-, and high-risk groups were 33%, 65%, and 96%, respectively. CONCLUSIONS: We created a risk score that classifies patients with ILD with acute respiratory failure from low to high risk for in-hospital mortality. The score could aid providers in counseling these patients and their families.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180305
[Lr] Last revision date:180305
[St] Status:Publisher

  6 / 2951 MEDLINE  
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[PMID]: 29490304
[Au] Autor:Montani D; Henry J; O'Connell C; Jaïs X; Cottin V; Launay D; Habib G; Bourdin A; Jevnikar M; Savale L; Rottat L; Simonneau G; Sitbon O; Humbert M; Allanore Y
[Ad] Address:Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France.
[Ti] Title:Association between Rheumatoid Arthritis and Pulmonary Hypertension: Data from the French Pulmonary Hypertension Registry.
[So] Source:Respiration;, 2018 Feb 28.
[Is] ISSN:1423-0356
[Cp] Country of publication:Switzerland
[La] Language:eng
[Ab] Abstract:BACKGROUND: Precapillary pulmonary hypertension (PH), and particularly pulmonary arterial hypertension (PAH), is a life-threatening complication of connective tissue diseases (systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease). The relationship between PH and rheumatoid arthritis (RA) has not been clearly established. OBJECTIVES: The aim of the study was to evaluate the relationship between precapillary PH and RA. METHODS: We identified patients with PH and suspected RA included in the French PH Registry between 1 May 2004 and 31 December 2012 and evaluated the prevalence of confirmed RA in this population. RA phenotypes, clinical, functional, and hemodynamic data, and patient outcomes were recorded. RESULTS: RA was confirmed in 20 patients (70% female; mean age 52 years) with precapillary PH, including 10 patients with PAH, 6 with severe PH due to lung disease, and 4 with chronic thromboembolic PH. The prevalence of RA was 0.35% (95% CI: 0.23-0.54) in the French PH Registry and 0.58% (95% CI: 0.30-1.11) in idiopathic PAH, comparable to that in the general population. The RA phenotype was characterized by the presence of specific RA autoantibodies and joint erosions in 75% of the patients. The outcomes of PH in the RA patients were unremarkable compared to those in other patients from the registry, and RA therapies had no major impact on the cardiopulmonary parameters. CONCLUSION: When precapillary PH occurs in RA patients, all PH subsets may be identified. The RA prevalence in the French PH Registry is similar to that in the general population, which does not support a specific association or an indication for PH screening in RA patients.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180228
[Lr] Last revision date:180228
[St] Status:Publisher
[do] DOI:10.1159/000485631

  7 / 2951 MEDLINE  
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[PMID]: 29367532
[Au] Autor:Michitsuji T; Horai Y; Sako A; Asano T; Iwanaga N; Izumi Y; Kawakami A
[Ad] Address:Department of General and Internal Medicine, National Hospital Organization Nagasaki Medical Center.
[Ti] Title:[A case of mixed connective tissue disease positive for proteinase 3 antineutrophil cytoplasmic antibody in a patient with slowly progressive type 1 diabetes mellitus and chronic thyroiditis].
[So] Source:Nihon Rinsho Meneki Gakkai Kaishi;40(6):467-470, 2017.
[Is] ISSN:1349-7413
[Cp] Country of publication:Japan
[La] Language:jpn
[Ab] Abstract:  A female in her sixties with slowly progressive type 1 diabetes mellitus (SPT1DM) and chronic thyroiditis was referred to our rheumatology department with swelling in her fingers. A prominent atherosclerotic lesion was revealed upon brain magnetic resonance imaging, and she was found to have mixed connective tissue disease (MCTD) positive for proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA). This rare case of MCTD accompanying SPT1DM and PR3-ANCA suggested that a synergy between MCTD and PR3-ANCA triggers atherosclerosis.
[Mh] MeSH terms primary: Antibodies, Antineutrophil Cytoplasmic
Atherosclerosis/etiology
Diabetes Mellitus, Type 1/complications
Hashimoto Disease/complications
Mixed Connective Tissue Disease/diagnosis
Mixed Connective Tissue Disease/immunology
Myeloblastin/immunology
Thyroiditis/complications
[Mh] MeSH terms secundary: Aged
Atherosclerosis/diagnostic imaging
Atherosclerosis/immunology
Brain/diagnostic imaging
Disease Progression
Female
Humans
Magnetic Resonance Imaging
Middle Aged
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Antibodies, Antineutrophil Cytoplasmic); EC 3.4.21.76 (Myeloblastin)
[Em] Entry month:1802
[Cu] Class update date: 180227
[Lr] Last revision date:180227
[Js] Journal subset:IM
[Da] Date of entry for processing:180126
[St] Status:MEDLINE
[do] DOI:10.2177/jsci.40.467

  8 / 2951 MEDLINE  
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[PMID]: 29474209
[Au] Autor:Hoffman TW; van Moorsel CHM; Borie R; Crestani B
[Ad] Address:Interstitial Lung Diseases Center of Excellence, Department of Pulmonology, St. Antonius Hospital, Nieuwegein, The Netherlands.
[Ti] Title:Pulmonary phenotypes associated with genetic variation in telomere-related genes.
[So] Source:Curr Opin Pulm Med;, 2018 Feb 21.
[Is] ISSN:1531-6971
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:PURPOSE OF REVIEW: Genomic mutations in telomere-related genes have been recognized as a cause of familial forms of idiopathic pulmonary fibrosis (IPF). However, it has become increasingly clear that telomere syndromes and telomere shortening are associated with various types of pulmonary disease. Additionally, it was found that also single nucleotide polymorphisms (SNPs) in telomere-related genes are risk factors for the development of pulmonary disease. This review focuses on recent updates on pulmonary phenotypes associated with genetic variation in telomere-related genes. RECENT FINDINGS: Genomic mutations in seven telomere-related genes cause pulmonary disease. Pulmonary phenotypes associated with these mutations range from many forms of pulmonary fibrosis to emphysema and pulmonary vascular disease. Telomere-related mutations account for up to 10% of sporadic IPF, 25% of familial IPF, 10% of connective-tissue disease-associated interstitial lung disease, and 1% of COPD. Mixed disease forms have also been found. Furthermore, SNPs in TERT, TERC, OBFC1, and RTEL1, as well as short telomere length, have been associated with several pulmonary diseases. Treatment of pulmonary disease caused by telomere-related gene variation is currently based on disease diagnosis and not on the underlying cause. SUMMARY: Pulmonary phenotypes found in carriers of telomere-related gene mutations and SNPs are primarily pulmonary fibrosis, sometimes emphysema and rarely pulmonary vascular disease. Genotype-phenotype relations are weak, suggesting that environmental factors and genetic background of patients determine disease phenotypes to a large degree. A disease model is presented wherever genomic variation in telomere-related genes cause specific pulmonary disease phenotypes whenever triggered by environmental exposure, comorbidity, or unknown factors.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180223
[Lr] Last revision date:180223
[St] Status:Publisher
[do] DOI:10.1097/MCP.0000000000000475

  9 / 2951 MEDLINE  
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[PMID]: 29384899
[Au] Autor:Zhang J; Huang X; Zhang X; Zhu Y; Liao K; Ma J; Wang G; Guo Y; Xie C
[Ad] Address:Department of Respiratory Medicine.
[Ti] Title:Coinfection of disseminated Talaromyces marneffei and Mycobacteria kansasii in a patient with papillary thyroid cancer: A case report.
[So] Source:Medicine (Baltimore);96(52):e9072, 2017 Dec.
[Is] ISSN:1536-5964
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:RATIONALE: Recently, Talaromyces marneffei (T. marneffei) has been reported in human immunodeficiency virus (HIV)-negative patient with underlying diseases, such as oral cancer, colon cancer, haematological malignancies, connective tissue disease, diabetes mellitus, and corticosteroids or immunosuppressive agents. Similar to HIV-positive ones, such patients were observed with CD4 lymphocytopenia. PATIENT CONCERNS: We reported a case of a 45-year-old woman who was diagnosed with disseminated T. marneffei and Mycobacteria kansasii (M. kansasii) with papillary thyroid cancer as the underlying disease. T-cell subsets counts, CD4 T-cell%, CD8 T-cell%, CD4/CD8 ratio, and NK cell% were all turned out to be normal. DIAGNOSES: Based on bronchoalveolar lavage fluid and skin lesions secretion cultures, blood culture, the patient was diagnosed with disseminated T. marneffei and M. kansasii. Pathological examination reported papillary thyroid cancer with cervical lymph node metastasis. INTERVENTIONS: The patient received the combined and longer antifungal therapy and drug regimens for M. kansasii. She had total thyroidectomy with radical neck dissection to treat the papillary thyroid cancer. OUTCOMES: The patient had a favorable outcome for 19 months without recurrence. LESSONS: T. marneffei could infect non-HIV individuals with underlying disease under the condition of normal T-cell counts. The symptoms were lack of specificity and were more likely to be misdiagnosed. Such patients with unidentified T-cell dysfunction or other unidentified primary immunodeficiency disorders may prone to coinfect with other opportunistic pathogens, such as M. kansasii. Compared with HIV-positive ones, they need combined and much longer antifungal therapy.
[Mh] MeSH terms primary: Carcinoma, Papillary/microbiology
Coinfection
Mycobacterium Infections, Nontuberculous/complications
Mycobacterium kansasii
Mycoses/complications
Talaromyces
Thyroid Neoplasms/microbiology
[Mh] MeSH terms secundary: Anti-Bacterial Agents/therapeutic use
Antifungal Agents/therapeutic use
Carcinoma, Papillary/pathology
Carcinoma, Papillary/therapy
Female
Humans
Middle Aged
Mycobacterium Infections, Nontuberculous/diagnosis
Mycobacterium Infections, Nontuberculous/therapy
Mycoses/diagnosis
Mycoses/therapy
Neck Dissection
Thyroid Neoplasms/pathology
Thyroid Neoplasms/therapy
Thyroidectomy
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Anti-Bacterial Agents); 0 (Antifungal Agents)
[Em] Entry month:1802
[Cu] Class update date: 180209
[Lr] Last revision date:180209
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:180201
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009072

  10 / 2951 MEDLINE  
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[PMID]: 29413506
[Au] Autor:Bazan IS; Mensah KA; Rudkovskaia AA; Adonteng-Boateng PK; Herzog EL; Buckley L; Fares WH
[Ad] Address:Yale University, School of Medicine, Section of Pulmonary, Critical Care & Sleep Medicine, New Haven, CT, USA.
[Ti] Title:Pulmonary arterial hypertension in the setting of scleroderma is different than in the setting of lupus: A review.
[So] Source:Respir Med;134:42-46, 2018 Jan.
[Is] ISSN:1532-3064
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Pulmonary hypertension (PH) is a clinical syndrome that is subdivided into five groups per the World Health Organization (WHO) classification, based largely on hemodynamic and pathophysiologic criteria. WHO Group 1 PH, termed pulmonary arterial hypertension (PAH), is a clinically progressive disease that can eventually lead to right heart failure and death, and it is hemodynamically characterized by pre-capillary PH and increased pulmonary vascular resistance in the absence of elevated left ventricular filling pressures. PAH can be idiopathic, heritable, or associated with a variety of conditions. Connective tissue diseases make up the largest portion of these associated conditions, most commonly systemic sclerosis (SSc), followed by mixed connective tissue disease and systemic lupus erythematous. These etiologies (namely SSc and Lupus) have been grouped together as connective tissue disease-associated PAH, however emerging evidence suggests they differ in pathogenesis, clinical course, prognosis, and treatment response. This review highlights the differences between SSc-PAH and Lupus-PAH. After introducing the diagnosis, screening, and pathobiology of PAH, we discuss connective tissue disease-associated PAH as a group, and then explore SSc-PAH and SLE-PAH separately, comparing these 2 PAH etiologies.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1802
[Cu] Class update date: 180207
[Lr] Last revision date:180207
[St] Status:In-Data-Review


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