2,795 results match your criteria Mixed Connective-Tissue Disease


Metabolic disturbances in urinary and plasma samples from seven different systemic autoimmune diseases detected by HPLC-ESI-QTOF-MS.

J Proteome Res 2020 May 28. Epub 2020 May 28.

Systemic Autoimmune Diseases (SADs) are characterized by dysfunction of the immune system, which cause damage in several tissues and organs. Among these pathologies are systemic lupus erythematosus, systemic sclerosis or scleroderma, Sjögren's syndrome, rheumatoid arthritis, the primary antiphospholipid syndrome, mixed connective tissue disease, and undifferentiated connective tissue disease (UCTD). Early diagnosis is difficult due to similarity in symptoms, signs, and clinical test results. Read More

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http://dx.doi.org/10.1021/acs.jproteome.0c00179DOI Listing

[Surgical Treatment of Lung Cancer Combined with Interstitial Lung Disease].

Zhongguo Fei Ai Za Zhi 2020 May;23(5):343-350

Department of Thoracic Surgery, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing 100730, China.

Background: Interstitial lung disease (ILD) is a group of diffuse lung diseases that mainly involve the interstitial and alveolar cavities and result in loss of alveolar-capillary functional units, leading to restrictive ventilatory dysfunction and diffusion impairment. There was an increased incidence of lung cancer on the basis of ILD, and perioperative risk of patients with lung cancer combined with ILD (LC-ILD) was significantly increased. The aim of this study is to summarize the safety and experience of surgical treatment of LC-ILD. Read More

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http://dx.doi.org/10.3779/j.issn.1009-3419.2020.104.19DOI Listing

An Autopsy Case of Mixed Connective Tissue Disease Complicated by Thrombotic Thrombocytopenic Purpura.

Intern Med 2020 15;59(10):1315-1321. Epub 2020 May 15.

Department of Rheumatology, Fukushima Medical University School of Medicine, Japan.

We herein report a patient with mixed connective tissue disease (MCTD) who had been stable for years but suddenly developed thrombotic thrombocytopenic purpura (TTP). The patient showed a clinical pentad of signs of TTP, low activity of ADAMTS13, and positivity of anti-ADAMTS13 antibodies. She did not respond to plasma exchange or steroid therapy and died five days after admission. Read More

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http://dx.doi.org/10.2169/internalmedicine.3939-19DOI Listing

Clinical and Immunological Profile of Mixed Connective Tissue Disease and a Comparison of Four Diagnostic Criteria.

Int J Rheumatol 2020 29;2020:9692030. Epub 2020 Jan 29.

Department of Medicine, Christian Medical College, Vellore 632004, India.

Mixed connective tissue disease (MCTD) was initially described as a chronic immune-mediated disease with overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis. We conducted a cross-sectional study to describe the clinical and immunological profile of patients with MCTD and to compare the four diagnostic criteria, namely, Sharp, Kasukawa, Alarcón-Segovia, and Khan criteria. A total of 291 patients who were admitted from June 2007 to June 2017 and fulfilled the inclusion criteria were included in the study. Read More

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http://dx.doi.org/10.1155/2020/9692030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7204172PMC
January 2020

A hierarchical bivariate meta-analysis of diagnostic test accuracy to provide direct comparisons of immunoassays vs. indirect immunofluorescence for initial screening of connective tissue diseases.

Clin Chem Lab Med 2020 Apr 30. Epub 2020 Apr 30.

Department of Microbiology, Immunology and Transplantation, Clinical and Diagnostic Immunology, KU Leuven, Leuven, Belgium.

Objectives To compare indirect immunofluorescence (IIF) for antinuclear antibodies (ANA) against immunoassays (IAs) as an initial screening test for connective tissue diseases (CTDs). Methods A systematic literature review identified cross-sectional or case-control studies reporting test accuracy data for IIF and enzyme-linked immunosorbent assays (ELISA), fluorescence enzyme immunoassay (FEIA), chemiluminescent immunoassay (CLIA) or multiplex immunoassay (MIA). The meta-analysis used hierarchical, bivariate, mixed-effect models with random-effects by test. Read More

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http://dx.doi.org/10.1515/cclm-2020-0094DOI Listing

Belimumab Concentrations in Maternal Serum and Breast Milk During Breastfeeding and the Safety Assessment of the Infant: A Case Study.

Breastfeed Med 2020 Apr 23. Epub 2020 Apr 23.

Japan Drug Information Institute in Pregnancy, National Center for Child Health and Development, Tokyo, Japan.

Belimumab is a recombinant human immunoglobulin G1 lambda monoclonal antibody that binds soluble B lymphocyte stimulator protein with high affinity and inhibits its biological activity. Belimumab is not recommended for breastfeeding women due to insufficient data about its excretion into breast milk. In this study, we measured belimumab concentrations in the breast milk of one nursing mother diagnosed with mixed connective tissue disease (MCTD) and evaluated the health of her breastfed infant. Read More

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http://dx.doi.org/10.1089/bfm.2020.0068DOI Listing

[Pneumococcal septic arthritis in a patient with mixed connective tissue disease].

Rev Esp Quimioter 2020 Jun 15;33(3):221-222. Epub 2020 Apr 15.

Jorge Ligero López, Servicio de Microbiología. Hospital Universitario Severo Ochoa, Av. de Orellana, s/n, 28911 Leganés, Madrid. Spain.

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http://dx.doi.org/10.37201/req/004.2020DOI Listing

Worldwide trends in all-cause mortality of auto-immune systemic diseases between 2001 and 2014.

Autoimmun Rev 2020 Jun 29;19(6):102531. Epub 2020 Mar 29.

Centre National de Référence des Maladies Auto-Immunes et Systémiques Rares, Est/Sud-Ouest (RESO), France; Service de rhumatologie, Centre Hospitalier Universitaire de Strasbourg, 1 avenue Molière, 67098 Strasbourg, France; Immuno-rhumatologie moléculaire, INSERM UMR-S 1109, Strasbourg, France. Electronic address:

Aim: To describe changes in the 2001-2014 mortality of 6 autoimmune systemic diseases (AISDs), namely Systemic Lupus Erythematosus (SLE), Systemic Sclerosis (SSc), Idiopathic Inflammatory Myopathies (IIM), Sjögren's Syndrome (SS), Mixed Connective Tissue Disease (MCTD) and ANCA-associated vasculitis (AAV) at the country-, continent-, and world-levels.

Methods: Mortality data were retrieved from the World Health Organization (WHO) mortality database for each disease, based on ICD-10 codes. We computed age-standardized mortality rate (ASMR) as the estimated number of deaths per million inhabitants and its 95% confidence interval (95%CI). Read More

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http://dx.doi.org/10.1016/j.autrev.2020.102531DOI Listing

[Clinical analysis of 12 cases of mixed connective tissue disease-associated trigeminal neuropathy].

Zhonghua Yi Xue Za Zhi 2020 Mar;100(12):938-941

Department of Rheumatology, People's Hospital of Peking University, Beijing 100044, China.

The aim of present study is to analyze clinical and laboratory features of mixed connective tissue disease (MCTD)-associated trigeminal neuropathy (TN). Clinical records of 12 cases of MCTD complicated with TN diagnosed in Peking University People's Hospital from January 2008 to October 2019 were analyzed retrospectively. The present study included 12 cases, 1 males and 11 females, average age was(40±13)years. Read More

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http://dx.doi.org/10.3760/cma.j.cn112137-20191113-02471DOI Listing

"Mixed connective tissue disease": a condition in search of an identity.

Clin Exp Med 2020 May 4;20(2):159-166. Epub 2020 Mar 4.

Division of Medicine, Centre for Rheumatology, University College of London, Room 424, 4th Floor, Rayne Building, 5 University Street, London, WC1E 6JF, UK.

Mixed connective tissue disease was first described as a new autoimmune rheumatic disease in 1972 based on the claim of a distinct clinical picture associated with anti-RNP antibody positivity. Subsequently, this new entity has divided opinions in the rheumatology community. We have reviewed recent cohort studies with more than 100 patients, comparing the clinical and immunological features, treatment, prognosis and evolution to well-defined autoimmune rheumatic diseases. Read More

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http://dx.doi.org/10.1007/s10238-020-00606-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7181542PMC

Performance of the 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus in Asian patients: a single-centre retrospective cohort study in Korea.

Clin Exp Rheumatol 2020 Feb 4. Epub 2020 Feb 4.

Div. of Rheumatology, Dept. of Internal Medicine, and Dept. of Molecular Medicine and Biopharmaceutical Sciences, Graduate School of Convergence Science and Technology and College of Medicine, Seoul National Univ. Hosp., Seoul, Korea.

Objectives: To evaluate the performance of the 2019 European League against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for systemic lupus erythematosus (SLE) in Asian patients.

Methods: We conducted an electronic medical chart review of patients with SLE and defined rheumatic diseases. Classification criteria of the 1997 ACR, 2012 Systemic Lupus International Collaborating Clinics (SLICC), and 2019 EULAR/ACR were examined based on sensitivity, specificity, positive predictive value, negative predicted value, and accuracy using clinical diagnosis as the gold standard. Read More

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February 2020

Targeted therapies in systemic sclerosis, myositis, antiphospholipid syndrome, and Sjögren's syndrome.

Best Pract Res Clin Rheumatol 2020 Feb 15:101485. Epub 2020 Feb 15.

Dept of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, Utrecht University, the Netherlands. Electronic address:

Targeted therapies using biological disease-modifying antirheumatic drugs (bDMARDs) and small molecule synthetic drugs have revolutionized rheumatological practice. Initially developed for the treatment of immune arthritis (rheumatoid arthritis, psoriatic arthritis, and spondylarthritis), both bDMARDs and small molecule synthetic drugs are now increasingly entering the space of connective tissue disease (CTD) treatment. Recent clinical trial data in systemic sclerosis (SSc) have been particularly encouraging with positive effects on outcomes having been observed with nintedanib preventing the decline of lung function in patients with SSc-related interstitial lung disease. Read More

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http://dx.doi.org/10.1016/j.berh.2020.101485DOI Listing
February 2020

Clinical characteristics and survival in systemic sclerosis-mixed connective tissue disease and systemic sclerosis-overlap syndrome.

Arthritis Care Res (Hoboken) 2020 Feb 14. Epub 2020 Feb 14.

Department of Rheumatology, St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia.

Objectives: To describe the clinical characteristics and outcomes of systemic sclerosis -mixed connective tissue disease (SSc-MCTD) and SSc-overlap.

Methods: We included patients from the Australian Scleroderma Cohort Study who met ACR/EULAR criteria for SSc. Three mutually exclusive groups were created: SSc-MCTD, SSc-overlap and SSc-only. Read More

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http://dx.doi.org/10.1002/acr.24167DOI Listing
February 2020

Clinical Outcomes of Hip Arthroscopy in Patients With Systemic Inflammatory Diseases Compared With Matched Controls at a Minimum of 2-Year Follow-Up.

Arthroscopy 2020 May 6;36(5):1345-1352. Epub 2020 Feb 6.

NYU Langone Orthopedic Hospital, New York, New York, U.S.A.

Purpose: To evaluate postoperative outcomes and preoperative risk factors for patients with underlying systemic inflammatory disorders after hip arthroscopy.

Methods: A retrospective analysis of patients who had undergone hip arthroscopy, with a history of systemic inflammatory disease, was performed. This included patients with a diagnosis of lupus, a positive antinuclear antibody test, rheumatoid arthritis, psoriatic arthritis, sarcoidosis, inflammatory bowel disease, Reiter syndrome, and mixed connective tissue disease. Read More

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http://dx.doi.org/10.1016/j.arthro.2020.01.017DOI Listing

Therapeutic Options for the Treatment of Interstitial Lung Disease Related to Connective Tissue Diseases. A Narrative Review.

J Clin Med 2020 Feb 3;9(2). Epub 2020 Feb 3.

Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, 41121 Modena, Italy.

Interstitial lung disease (ILD) is one of the most serious pulmonary complications of connective tissue diseases (CTDs) and it is characterized by a deep impact on morbidity and mortality. Due to the poor knowledge of CTD-ILD's natural history and due to the difficulties related to design of randomized control trials, there is a lack of prospective data about the prevalence, follow-up, and therapeutic efficacy. For these reasons, the choice of therapy for CTD-ILD is currently very challenging and still largely based on experts' opinion. Read More

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http://dx.doi.org/10.3390/jcm9020407DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7073957PMC
February 2020

Pulmonary infection due to Acrophialophora fusispora in a patient with underlying mixed connective tissue disease and chronic pulmonary aspergillosis: A case report and review of literature.

J Mycol Med 2020 Jun 22;30(2):100932. Epub 2020 Jan 22.

Department of Microbiology, All India Institute of Medical Sciences, Phase 2 Industrial Area, 342005 Jodhpur, Rajasthan, India.

Acrophialophora fusispora is a soil-borne fungus rarely implicated in human infections. Here, we report a case of pulmonary infection due to A. fusispora in a 59-year-old male who presented with productive cough and gradually progressive dyspnoea for 20 days. Read More

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http://dx.doi.org/10.1016/j.mycmed.2020.100932DOI Listing

Cardiac Catheterization versus Echocardiography for Monitoring Pulmonary Pressure: A Prospective Study in Patients with Connective Tissue Disease-Associated Pulmonary Arterial Hypertension.

Diagnostics (Basel) 2020 Jan 19;10(1). Epub 2020 Jan 19.

First Department of Propaedeutic Internal Medicine and Joint Rheumatology Program, Medical School, National and Kapodistrian University of Athens, 157 72 Athens, Greece.

Standard echocardiography is important for pulmonary arterial hypertension (PAH) screening in patients with connective tissue disease (CTD), but PAH diagnosis and monitoring require cardiac catheterization. Herein, using cardiac catheterization as reference, we tested the hypothesis that follow-up echocardiography is adequate for clinical decision-making in these patients. We prospectively studied 69 consecutive patients with CTD-associated PAH. Read More

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http://dx.doi.org/10.3390/diagnostics10010049DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7168199PMC
January 2020

Circulating NEDD9 is increased in pulmonary arterial hypertension: A multicenter, retrospective analysis.

J Heart Lung Transplant 2020 Apr 31;39(4):289-299. Epub 2019 Dec 31.

Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts; Division of Cardiovascular Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts. Electronic address:

Background: Pulmonary arterial hypertension (PAH) is a highly morbid disease characterized by elevated pulmonary vascular resistance (PVR) and pathogenic right ventricular remodeling. Endothelial expression of the prometastatic protein NEDD9 is increased in fibrotic PAH arterioles, and NEDD9 inhibition decreases PVR in experimental PAH. We hypothesized that circulating NEDD9 is increased in PAH and informs the clinical profile of patients. Read More

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http://dx.doi.org/10.1016/j.healun.2019.12.002DOI Listing

The Serum Cell-Free microRNA Expression Profile in MCTD, SLE, SSc, and RA Patients.

J Clin Med 2020 Jan 7;9(1). Epub 2020 Jan 7.

Department of Molecular Biology, National Institute of Geriatrics, Rheumatology, and Rehabilitation, Warsaw 02-637, Poland.

Mixed connective tissue disease (MCTD) is a rare disorder characterized by symptoms that overlap two or more Autoimmune Connective Tissue Diseases (ACTDs). The aim of this study was to determine whether miRNAs participating in the TLRs signaling pathway could serve as biomarkers differentiating MCTD or other ACTD entities from a healthy control group and between groups of patients. Although the selected miRNA expression level was not significantly different between MCTD and control, we observed that miR-126 distinguishes MCTD patients from all other ACTD groups. Read More

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http://dx.doi.org/10.3390/jcm9010161DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7020053PMC
January 2020

2019 Diagnostic criteria for mixed connective tissue disease (MCTD): From the Japan research committee of the ministry of health, labor, and welfare for systemic autoimmune diseases.

Mod Rheumatol 2020 Jan 7:1-5. Epub 2020 Jan 7.

Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.

To update and revise the diagnostic criteria for mixed connective tissue disease (MCTD) issued by the Japan Research Committee of the Ministry of Health, Labor, and Welfare (MHLW), a round table discussion by experts from rheumatology, dermatology, and pediatric medicine was conducted in multiple occasions. The definition of MCTD, and items included in the diagnostic criteria were generated by consensus method and evaluation using clinical data of typical and borderline cases of MCTD, by applying to the diagnostic criteria for MCTD proposed in 1996 and 2004 by the Research Committee of MHLW. To the end, all committee members reached consensus. Read More

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http://dx.doi.org/10.1080/14397595.2019.1709944DOI Listing
January 2020

Somatosensory profile of a patient with mixed connective tissue disease and Sjögren syndrome.

J Am Dent Assoc 2020 Feb 23;151(2):145-151. Epub 2019 Dec 23.

Background And Overview: The authors report the case of a patient with mixed connective tissue disease (MCTD) and Sjögren syndrome, showing signs and symptoms of bilateral trigeminal neuropathy and aseptic meningitis. The patient was assessed by means of quantitative sensory testing (QST) according to the German Research Network on Neuropathic Pain standards, in both the gingiva and forearm, and the results were compared with those of healthy control participants.

Case Description: A 27-year-old woman, who had received a diagnosis of MCTD and Sjögren syndrome from a rheumatologist, sought treatment at an orofacial pain clinic for bilateral electriclike pain in the maxillary anterior gingiva, eyelids, and cheeks. Read More

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http://dx.doi.org/10.1016/j.adaj.2019.09.001DOI Listing
February 2020

Heart-lung transplantation: A viable option for connective tissue diseases.

Clin Transplant 2020 Feb 9;34(2):e13776. Epub 2020 Jan 9.

Division of Cardiology, Department of Medicine, University of California San Francisco, San Francisco, California.

Background: While lung transplantation (LTx) has been effective for connective tissue disease (CTD) patients with pulmonary involvement, outcomes for heart-lung transplantation (HLTx) are less defined. The aim of this study is to evaluate HLTx in CTD patients utilizing the UNOS database.

Methods: HLTx patients with CTD (HLTx-CTD) were compared to both LTx patients with CTD (LTx-CTD) and HLTx patients with all other indications (HLTx-OI) from 1999 to 2018. Read More

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http://dx.doi.org/10.1111/ctr.13776DOI Listing
February 2020

Extremely high levels of multiple cytokines in the cord blood of neonates born to mothers with systemic autoimmune diseases.

Cytokine 2020 Mar 19;127:154926. Epub 2019 Dec 19.

Department of Obstetrics and Gynecology, The University of Tokyo Hospital, Tokyo, Japan.

Most infants born to mothers with autoimmune diseases are thought to be entirely healthy. However, the immunological conditions have not been examined thoroughly. Fourteen neonates born to mothers with systemic autoimmune diseases, namely systemic lupus erythematosus, mixed connective tissue disease, Sjögren's syndrome, rheumatoid arthritis, and systemic sclerosis, were included. Read More

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http://dx.doi.org/10.1016/j.cyto.2019.154926DOI Listing

Psychological distress and quality of life are improved in autoimmune patients through Tandem-Psychotherapy, combining individual hypnosis and eye movement desensitization and reprocessing (EMDR) treatment for trauma, followed by supportive-expressive group therapy.

Clin Rheumatol 2020 Apr 19;39(4):1331-1339. Epub 2019 Dec 19.

Ambulatorio Medicina Integrata, "Andrea Gallino" Hospital, Genoa, Italy.

Objective: Autoimmune diseases are associated with psychological distress, resulting in greatly impaired quality of life. Tandem-Psychotherapy comprises trauma-focused psychotherapy with hypnosis and eye movement desensitization and reprocessing (EMDR), followed by supportive-expressive group therapy. The objective was to evaluate whether Tandem-Psychotherapy could reduce psychological distress and improve quality of life. Read More

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http://dx.doi.org/10.1007/s10067-019-04862-1DOI Listing

Increased Endothelial Activation in Patients with Mixed Connective Tissue Disease.

J Stroke Cerebrovasc Dis 2020 Feb 9;29(2):104563. Epub 2019 Dec 9.

Department of Neurology, Oslo University Hospital Rikshospitalet, Oslo Norway. Electronic address:

Objective: Juvenile-onset mixed connective tissue disease (JMCTD) is a chronic inflammatory disease. We have previously demonstrated preclinical atherosclerosis in these patients, now exploring this further by assessing markers of endothelial dysfunction.

Methods: Thirty-three patients with JMCTD and 33 age-and sex-matched controls were included. Read More

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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2019.104563DOI Listing
February 2020

Macitentan for the treatment of severe digital ulcers in a patient with mixed connective tissue disease: avoiding drug interactions.

Clin Exp Rheumatol 2020 Jan-Feb;38(1):171. Epub 2019 Nov 20.

Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitario La Paz, Madrid, Spain.

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[A multicenter study of the condition of children's rheumatic disease associated medical resources in Fujian province].

Zhonghua Er Ke Za Zhi 2019 Dec;57(12):913-916

Department of Pediatrics, the First Affiliated Hospital of Xiamen University, Xiamen 361000, China.

To investigate general condition of children's rheumatic disease associated medical resources in Fujian Province. This questionnaire-based survey was conducted in 19 hospitals in Fujian province from December 2, 2018 to May 1, 2019. The questionnaire was designed to survey the general condition of the medical resources and the hospitalization of patients with rheumatic diseases from January 1, 2014 to December 1, 2018. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2019.12.004DOI Listing
December 2019

Interferons () Genetic Variants in Patients with Mixed Connective Tissue Disease (MCTD).

J Clin Med 2019 Nov 21;8(12). Epub 2019 Nov 21.

Department of Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, 02-637 Warsaw, Poland.

Mixed connective tissue disease (MCTD) is a rare complex autoimmune disease in which autoantigens are recognized by endosomal TLRs. Their activation induces a higher secretion of the type I interferons, IFN-γ and the up-regulation of the INF-inducible genes. The present study aimed to investigate whether SNPs that are located in the IFN-A, IFN-B, and IFN-G genes are associated with MCTD. Read More

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http://dx.doi.org/10.3390/jcm8122046DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6947393PMC
November 2019

Systemic Autoimmune Disease Among Adults Exposed to the September 11, 2001 Terrorist Attack.

Arthritis Rheumatol 2020 May 1;72(5):849-859. Epub 2020 Apr 1.

New York City Department of Health and Mental Hygiene, New York, New York.

Objective: Autoimmune disease is an emerging condition among persons exposed to the September 11, 2001 attack on the World Trade Center (WTC). Components of the dust cloud resulting from the collapse of the WTC have been associated with development of a systemic autoimmune disease, as has posttraumatic stress disorder (PTSD). We undertook this study to determine whether dust exposure and PTSD were associated with an increased risk of systemic autoimmune disease in a 9/11-exposed cohort. Read More

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http://dx.doi.org/10.1002/art.41175DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7216890PMC

Congestive hepatopathy and acute pancreatitis as severe complications of mixed connective tissue disease.

Clin J Gastroenterol 2020 Jun 19;13(3):434-442. Epub 2019 Nov 19.

Department of Gastroenterology, Nagano Red Cross Hospital, Nagano, Japan.

Mixed connective tissue disease (MCTD) causes multiple organ dysfunctions, such as joint swelling, pulmonary fibrosis and hypertension, and serositis, but hepatopancreatic complications are rare. Here, we report a case of young man who exhibited acute severe liver dysfunction. He also had impaired cardiac function: both ventriculi were hypokinetic, but pulmonary hypertension and pericarditis were not observed. Read More

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http://dx.doi.org/10.1007/s12328-019-01059-6DOI Listing

Myocarditis as a lupus challenge: two case reports.

J Med Case Rep 2019 Nov 20;13(1):343. Epub 2019 Nov 20.

Department of Rheumatology, Internal Medicine, Tawam Hospital, Al Ain, United Arab Emirates.

Background: Myocarditis is an uncommon manifestation of systemic lupus erythematosus in which the clinical presentation can range from subclinical to life-threatening. We report cases of two patients who presented to our hospital with myocarditis as an initial manifestation of systemic lupus erythematosus despite negative results of extensive workup that excluded other diagnoses. The mainstays of treatment are corticosteroids, immunosuppressive agents, and anti-heart failure medications, with use of the latter being case-specific. Read More

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http://dx.doi.org/10.1186/s13256-019-2242-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6864968PMC
November 2019

Individuals With Scleroderma May Have Increased Risk of Sleep-Disordered Breathing.

J Clin Sleep Med 2019 11;15(11):1665-1669

Division of Pulmonary Medicine, Mayo Clinic Hospital, Phoenix, Arizona.

Study Objectives: Scleroderma is associated with abnormal skin thickening, interstitial lung disease, pulmonary hypertension, and abnormalities of the upper airway. These changes can cause cardiopulmonary complications, potentially including sleep-disordered breathing. The objective of this study is to examine the risk of sleep-disordered breathing in patients with scleroderma. Read More

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http://dx.doi.org/10.5664/jcsm.8036DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6853384PMC
November 2019
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Drop Head Syndrome as a Rare Complication in Mixed Connective Tissue Disease.

Intern Med 2020 Mar 18;59(5):729-732. Epub 2019 Nov 18.

Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan.

A 54-year-old woman developed drop head syndrome (DHS), Raynaud's phenomenon and creatine kinase (CK) elevation. She did not meet the international classification criteria of dermatomyositis/polymyositis, as we observed no muscle weakness, grasping pain or electromyography abnormality in her limbs, and anti-aminoacyl tRNA synthetase (ARS) antibody was negative. Cervical magnetic resonance imaging and a muscle biopsy of the trapezius muscle revealed myositis findings as the only clinical observations in muscle. Read More

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http://dx.doi.org/10.2169/internalmedicine.3626-19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7086316PMC

Clinical Impact of Nailfold Capillaroscopy in Daily Clinical Practice.

Reumatol Clin 2019 Nov 7. Epub 2019 Nov 7.

Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España.

Introduction: Nailfold capillaroscopy (NC) is useful in the evaluation of Raynaud's phenomenon, associated with some connective tissue diseases and in the follow-up of patients with systemic sclerosis. Our study evaluates the impact of NC in the diagnosis, according to the reason for the request and profile of autoantibodies in daily clinical practice.

Material And Methods: All patients that undergone at least one NC between June 2012 and December 2017 were included. Read More

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http://dx.doi.org/10.1016/j.reuma.2019.07.008DOI Listing
November 2019

Pain, functional disability, and their Association in Juvenile Fibromyalgia Compared to other pediatric rheumatic diseases.

Pediatr Rheumatol Online J 2019 Nov 6;17(1):72. Epub 2019 Nov 6.

Hackensack University Medical Center, 30 Prospect Avenue, WFAN, PC360, Hackensack, NJ, 07601, USA.

Background: Severe pain and impairments in functioning are commonly reported for youth with juvenile fibromyalgia. The prevalence and impact of pain in other diseases commonly managed in pediatric rheumatology comparatively have been rarely systematically studied. The objective of the current study was to determine the extent to which high levels of pain and functional limitations, and the strength of their association, are unique to youth with juvenile primary fibromyalgia syndrome/JPFS) relative to other pediatric rheumatic diseases. Read More

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http://dx.doi.org/10.1186/s12969-019-0375-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6836648PMC
November 2019
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Ultrasound salivary gland involvement in Sjogren's syndrome vs. other connective tissue diseases: is it autoantibody and gland dependent?

Clin Rheumatol 2020 Apr 1;39(4):1207-1215. Epub 2019 Nov 1.

Department of Rheumatology, Bone and Joint Research Unit, Hospital Universitario Fundación Jiménez Díaz, IIS Fundación Jiménez Díaz, Universidad Autónoma de Madrid, Av. Reyes Católicos, 2, 28040, Madrid, Spain.

This study aims to investigate ultrasound (US) findings on salivary glands (SG) in patients with Sjögren syndrome (SS) vs. other connective tissue diseases (CTDs) and to assess the relationship of SGUS abnormalities with autoantibody profile in both groups. We enrolled 81 patients, 45 diagnosed with SS (39 with primary SS, 6 with secondary SS) and 36 diagnosed with other CTDs. Read More

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http://dx.doi.org/10.1007/s10067-019-04780-2DOI Listing
April 2020
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Demographic and clinical predictors of progression and mortality in connective tissue disease-associated interstitial lung disease: a retrospective cohort study.

BMC Pulm Med 2019 Oct 31;19(1):192. Epub 2019 Oct 31.

Department of Medicine, University of British Columbia, Vancouver, Canada.

Background: Connective tissue disease-associated interstitial lung disease (CTD-ILD) is associated with reduced quality of life and poor prognosis. Prior studies have not identified a consistent combination of variables that accurately predict prognosis in CTD-ILD. The objective of this study was to identify baseline demographic and clinical characteristics that are associated with progression and mortality in CTD-ILD. Read More

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http://dx.doi.org/10.1186/s12890-019-0943-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6824100PMC
October 2019

Digital ulcers and acro-osteolysis in mixed connective tissue disease.

Rheumatology (Oxford) 2020 Jun;59(6):1460

Rheumatology Section, Clínica Universitaria Universidad Pontificia Bolivariana, Medellín, Colombia.

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http://dx.doi.org/10.1093/rheumatology/kez515DOI Listing

Whole-genome bisulfite sequencing in systemic sclerosis provides novel targets to understand disease pathogenesis.

BMC Med Genomics 2019 10 24;12(1):144. Epub 2019 Oct 24.

Lady Davis Institute for Medical Research, Jewish General Hospital, 3755 Côte Sainte-Catherine Road, Montreal, H3T 1E2, Canada.

Background: Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease whose pathogenesis remains incompletely understood. Increasing evidence suggests that both genetic susceptibilities and changes in DNA methylation influence pivotal biological pathways and thereby contribute to the disease. The role of DNA methylation in SSc has not been fully elucidated, because existing investigations of DNA methylation predominantly focused on nucleotide CpGs within restricted genic regions, and were performed on samples containing mixed cell types. Read More

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http://dx.doi.org/10.1186/s12920-019-0602-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6813992PMC
October 2019
1 Read

Pathological interpretation of connective tissue disease-associated lung diseases.

Authors:
Kun Young Kwon

Yeungnam Univ J Med 2019 01 15;36(1):8-15. Epub 2019 Jan 15.

Department of Pathology, Dongkang Hospital, Ulsan, Korea.

Connective tissue diseases (CTDs) can affect all compartments of the lungs, including airways, alveoli, interstitium, vessels, and pleura. CTD-associated lung diseases (CTD-LDs) may present as diffuse lung disease or as focal lesions, and there is significant heterogeneity between the individual CTDs in their clinical and pathological manifestations. CTD-LDs may presage the clinical diagnosis a primary CTD, or it may develop in the context of an established CTD diagnosis. Read More

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http://dx.doi.org/10.12701/yujm.2019.00101DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6784620PMC
January 2019
1 Read

Conversion surgery after extensive chemotherapy for stage IV mixed adenoneuroendocrine carcinoma (MANEC) of the gallbladder: clinical implications from the patterns of response and recurrence.

Clin J Gastroenterol 2020 Apr 15;13(2):240-246. Epub 2019 Oct 15.

Department of Surgery, Kindai University, Osaka, Japan.

A 53-year-old woman who was diagnosed as having advanced gallbladder cancer (T3N1M1, Stage IVB) showed a dramatic response to chemotherapy using gemcitabine + cisplatin and surgery with curative intent was attempted. Histopathological examination revealed residual tumor limited to the perimuscular connective tissue of the gallbladder (T2bN0M0, Stage IIB) and a mixture of poorly differentiated adenocarcinoma and neuroendocrine carcinoma (NEC) components was confirmed. At 6 months after the surgery, a small peritoneal nodule was detected and chemotherapy was resumed with gemcitabine, cisplatin, and S-1. Read More

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http://dx.doi.org/10.1007/s12328-019-01053-yDOI Listing
April 2020
1 Read

Long-term follow-up of nailfold videocapillaroscopic microvascular parameters in mixed connective tissue disease versus systemic sclerosis patients: a retrospective cohort study.

Clin Exp Rheumatol 2019 Jul-Aug;37 Suppl 119(4):102-107. Epub 2019 Oct 3.

Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genova, Italy.

Objectives: To retrospectively study nailfold videocapillaroscopy (NVC) changes in mixed connective tissue disease (MCTD) patients and to compare the capillary morphological abnormalities between patients affected by MCTD and systemic sclerosis (SSc) over time.

Methods: Ten MCTD patients on whom NVC had been performed, with a follow-up of three years, were selected. In addition, ten patients affected by SSc with similar age and disease duration of MCTD patients were enrolled to compare NVC abnormalities at baseline (T0). Read More

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October 2019
2 Reads

Suspected inflammatory rheumatic diseases in patients presenting with skin rashes.

Best Pract Res Clin Rheumatol 2019 08 1;33(4):101440. Epub 2019 Oct 1.

Clinic of Dermatology, University Hospital of Coimbra, Portugal; Faculty of Medicine, University of Coimbra, Coimbra, Portugal. Electronic address:

Skin lesions occur, often at very early stages, in many of the most frequent inflammatory rheumatic diseases such as in systemic lupus erythematosus (SLE), dermatomyositis (DM), systemic sclerosis (SSc), Sjögren's syndrome, rheumatoid arthritis (RA), and psoriatic arthritis. It is important to recognize the different specific cutaneous lesions in SLE (e.g. Read More

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http://dx.doi.org/10.1016/j.berh.2019.101440DOI Listing
August 2019
4 Reads

Characterizing Pruritus in Autoimmune Connective Tissue Diseases

J Drugs Dermatol 2019 Oct;18(10):995-998

Background: Autoimmune connective tissue diseases (ACTDs) are a broad range of diseases featured by immune dysregulation, and often have multisystem involvement with prominent skin manifestations. Pruritus is one of the most common symptoms in these diseases, with significant impact on the quality of life of patients. Objective: To characterize the frequency, location, severity, and timing relative to disease onset of pruritus in different ACTDs. Read More

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October 2019
4 Reads

Vaccination coverage in children with rheumatic diseases.

Clin Exp Rheumatol 2020 Jan-Feb;38(1):164-170. Epub 2019 Oct 2.

Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Centre Ljubljana, and Faculty of Medicine, University of Ljubljana, Slovenia.

Objectives: To assess vaccination status in a cohort of children with rheumatic diseases followed at the University Children's Hospital Ljubljana and to evaluate the most common reasons for vaccination dropout.

Methods: Patients with rheumatic diseases who were evaluated at the rheumatology outpatient clinic between January 2015 and January 2017 received a questionnaire about their vaccination status and reasons for potential vaccination dropout. Vaccination coverage for individual vaccines was determined at 5, 10, 18 years and at the time of their last clinic visit. Read More

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October 2019
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Is the T-G-CT-G SNRNP70 haplotype another proof that mixed connective tissue disease is distinct from systemic lupus erythematosus and systemic sclerosis? A novel gene variant in SNRNP70 gene.

Clin Exp Rheumatol 2019 Jul-Aug;37 Suppl 119(4):32-40. Epub 2019 Sep 17.

Department of Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland.

Objectives: U1-70K, encoded by the SNRNP70 gene, is a key early immunogen in connective tissue disease. The aim of the study was the genetic analysis of the SNRNP70 gene in mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) patients.

Methods: SNRNP70 genetic variants were detected using 3730 DNA Analyzer. Read More

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October 2019
4 Reads
2.724 Impact Factor

Etiological Spectrum of Non-compressive Myelopathies in Tertiary Care Centre.

J Assoc Physicians India 2019 Sep;67(9):14-16

Senior Resident.

Aims: To study the clinical, radiological, cerebrospinal fluid profile of noncompressive myelopathy and to study various etiologies of non-compressive myelopathies in causation of quadriplegia and paraplegia.

Study Design: Observational study.

Place And Duration Of Study: Department of Neurology, Govt. Read More

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September 2019
2 Reads

Chondral grafts for condylar reconstruction in treatment of temporomandibular joint arthritis in a mixed connective tissue disease patient.

Kaohsiung J Med Sci 2019 Dec 11;35(12):787-788. Epub 2019 Sep 11.

Department of Oral and Maxillofacial Surgery, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan.

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http://dx.doi.org/10.1002/kjm2.12128DOI Listing
December 2019
3 Reads

Tumor induced osteomalacia in head and neck region: single center experience and systematic review.

Endocr Connect 2019 Oct;8(10):1330-1353

Department of Endocrinology, Seth GS Medical College & KEM Hospital, Parel, Mumbai, India.

Tumor-induced osteomalacia in the head and neck region remains a challenging diagnosis to manage. Literature pertaining to management and outcome details remains sparse. We describe two cohorts: cohort 1 included seven patients from a single center in Western India with tumors located in paranasal sinuses (n = 3), intracranial (n = 2) and maxilla (n = 2). Read More

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http://dx.doi.org/10.1530/EC-19-0341DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6790902PMC
October 2019
3 Reads