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    Vasculitic and autoimmune wounds.
    J Vasc Surg Venous Lymphat Disord 2017 Mar 14;5(2):280-292. Epub 2016 Dec 14.
    Division of Rheumatology, The George Washington University, School of Medicine and Health Sciences, Washington, D.C.
    Objective: Chronic wounds are a major cause of morbidity and mortality. Approximately 20% to 23% of nonhealing wounds that are refractory to vascular intervention have other causes, including vasculitis, pyoderma gangrenosum, and other autoimmune diseases. The purpose of this article was to review the literature across medical and surgical specialties with regard to refractory chronic wounds associated with vasculitis and autoimmune diseases and to delineate clinical outcomes of these wounds in response to vascular and other interventions. Read More

    Pulmonary hypertension in connective tissue diseases: an update.
    Int J Rheum Dis 2017 Feb 16. Epub 2017 Feb 16.
    Department ofClinical Immunology & Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India.
    Pulmonary hypertension (PH) is a relatively commoner complication of systemic sclerosis (SSc) with estimated prevalence ranging between 8% and 12% as compared to much lower figures in other connective tissue diseases (CTD). It is a major cause of morbidity and mortality in CTDs. PH is classified into five major groups. Read More

    Treatment of Vasodilator-resistant Mixed Connective Tissue Disease-associated Pulmonary Arterial Hypertension with Glucocorticoid and Cyclophosphamide.
    Intern Med 2017 15;56(4):445-448. Epub 2017 Feb 15.
    Division of Rheumatology, Endocrinology and Nephrology, Hokkaido University Graduate School of Medicine, Japan.
    Pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MTCD), in contrast to other types of PAH, may respond to immunosuppressive therapy. Most PAH cases with an immunosuppressant response were in the early stages of the disease (WHO functional class III or less). The present case was a 34-year-old woman with MCTD-associated PAH (WHO functional class IV) who was resistant to a combination of three vasodilators. Read More

    Cardiovascular magnetic resonance imaging pattern at the time of diagnosis of treatment naïve patients with connective tissue diseases.
    Int J Cardiol 2017 Feb 1. Epub 2017 Feb 1.
    Onassis Cardiac Surgery Center, Athens, Greece.
    Background-aim: Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis.

    Patients-methods: CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]. Read More

    Survival and outcomes after lung transplantation for non-scleroderma connective tissue-related interstitial lung disease.
    J Heart Lung Transplant 2016 Dec 31. Epub 2016 Dec 31.
    Division of Pulmonary and Critical Care, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania; Division of Rheumatology, Brigham and Women's Hospital, Boston, Massachusetts. Electronic address:
    Background: Patients with non-scleroderma connective tissue-related lung disease (NS-CTLD), including rheumatoid arthritis, idiopathic inflammatory myopathies, Sjögren syndrome, mixed connective tissue disease, and systemic lupus erythematosus, may be at risk for worse outcomes after lung transplantation because of immune dysregulation or extrapulmonary manifestations of their underlying disease. We compared survival, acute and chronic rejection, and extrapulmonary organ dysfunction after transplantation in patients with NS-CTLD and idiopathic pulmonary fibrosis (IPF).

    Methods: This was a retrospective cohort study of patients with NS-CTLD and IPF who were listed in the Scientific Registry of Transplant Recipients and underwent lung transplantation from May 5, 2005, to March 1, 2016. Read More

    Hypercalcemia due to Primary Hepatic Lymphoma.
    Case Rep Hematol 2016 25;2016:1876901. Epub 2016 Dec 25.
    Department of Hematology and Oncology, University of Massachusetts Medical School, Worcester, MA, USA.
    A 65-year-old female with a history of mixed connective tissue disease and pulmonary fibrosis on azathioprine, hydroxychloroquine, and prednisone (osteoporosis on teriparatide) presented with a 1-month history of hypercalcemia. After discontinuation of teriparatide, the patient's hypercalcemia persisted. Further evaluation revealed primary hepatic lymphoma as the source of her hypercalcemia. Read More

    Successful treatment of Type B insulin resistance with mixed connective tissue disease by pulse glucocorticoids and cyclophosphamide.
    J Diabetes Investig 2017 Jan 13. Epub 2017 Jan 13.
    Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
    Introduction: Type B insulin resistance syndrome is a very rare condition caused by autoantibodies against the insulin receptor. We report the successful treatment of a patient with refractory type B insulin resistance with pulse glucocorticoids and cyclophosphamide.

    Materials And Methods: The medical record of a patient with type B insulin resistance was reviewed. Read More

    Mixed Connective Tissue Disorder Complicated by Polymyositis, Sjogren's Syndrome, Pleural Effusion and Pericarditis.
    Cureus 2016 Dec 1;8(12):e906. Epub 2016 Dec 1.
    Department of Internal Medicine, Carthage Area Hospital, New York, USA.
    We report a case of a 24-year-old female with a history of asthma and gastroesophageal reflux disease (GERD). She presented to the emergency room with severe chest pain, chest tightness, and shortness of breath following an upper respiratory tract infection. The patient reported that she had a cough and runny nose one week prior to this presentation, followed by a sudden sharp pain in the center of the chest 8/10 in intensity on the visual analog scale and pleuritic in nature, which aggravated by deep breathing and lying down flat. Read More

    Intravenous Immunoglobulin for Mixed Connective Tissue Disease Presenting With Bilateral Trigeminal Neuropathy.
    Am J Ther 2016 Dec 26. Epub 2016 Dec 26.
    1Division of Rheumatology, Yale University, New Haven, CT 2Division of Rheumatology, University of Nebraska Medical Center, Omaha, NE 3Department of Neurological Sciences, University of Nebraska Medical Center, Omaha, NE.

    Physical and cultural determinants of postpartum pelvic floor support and symptoms following vaginal delivery: a protocol for a mixed-methods prospective cohort study.
    BMJ Open 2017 Jan 10;7(1):e014252. Epub 2017 Jan 10.
    Department of Obstetrics and Gynecology, School of Medicine, University of Utah, Salt Lake City, Utah, USA.
    Introduction: Pelvic floor disorders (PFDs), including pelvic organ prolapse (POP), stress and urgency urinary incontinence, and faecal incontinence, are common and arise from loss of pelvic support. Although severe disease often does not occur until women become older, pregnancy and childbirth are major risk factors for PFDs, especially POP. We understand little about modifiable factors that impact pelvic floor function recovery after vaginal birth. Read More

    Adhesive arachnoiditis in mixed connective tissue disease: a rare neurological manifestation.
    BMJ Case Rep 2016 Dec 16;2016. Epub 2016 Dec 16.
    Rheumatology Department, University Hospital Limerick, Limerick, Ireland.
    The overall incidence of neurological manifestations is relatively low among patients with mixed connective tissue disease (MCTD). We recently encountered a case of autoimmune adhesive arachnoiditis in a young woman with 7 years history of MCTD who presented with severe back pain and myeloradiculopathic symptoms of lower limbs. To the best of our knowledge, adhesive arachnoiditis in an MCTD patient has never been previously reported. Read More

    Azathioprine response in patients with fibrotic connective tissue disease-associated interstitial lung disease.
    Respir Med 2016 Dec 4;121:117-122. Epub 2016 Nov 4.
    Department of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, United States.
    Background: Azathioprine is a commonly prescribed therapy for connective tissue disease-associated interstitial lung disease (CTD-ILD). Combination therapy that included azathioprine was recently shown to increase the risk of death and hospitalization in patients with idiopathic pulmonary fibrosis. Whether azathioprine increases the risk of adverse outcomes in patients with fibrotic CTD-ILD, including those with CTD-associated usual interstitial pneumonia (UIP), remains unknown. Read More

    Myocardial perfusion in peripheral Raynaud's phenomenon. Evaluation using stress cardiovascular magnetic resonance.
    Int J Cardiol 2017 Feb 12;228:444-448. Epub 2016 Nov 12.
    Onassis Cardiac Surgery Center, Athens, Greece.
    Background: Peripheral Raynaud's phenomenon (RP) is either primary (PRP), without any coexisting disease or secondary (SRP), due to connective tissue diseases (CTD). We hypothesized that adenosine stress cardiovascular magnetic resonance (CMR) can assess myocardial perfusion in a population of PRP and SRP.

    Patients-methods: Twenty CTDs, aged 30. Read More

    Immunity and early atherosclerosis in the course of systemic lupus erythematosus, mixed connective tissue disease and antiphospholipid syndrome.
    Reumatologia 2016 5;54(4):187-195. Epub 2016 Oct 5.
    Department of Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland.
    Atherosclerosis is a chronic inflammatory disease of the arteries associated with various risk factors that promote lipid abnormalities, development and progression of atherosclerotic lesions, plaque rupture, and vascular thrombosis. Atherosclerosis is accelerated in autoimmune diseases. Non-invasive investigations showed increased intima-media thickness (IMT), carotid plaque, and coronary artery calcifications in patients with antiphospholipid syndrome, systemic lupus erythematosus and mixed connective tissue disease compared to controls. Read More

    How I treat cryoglobulinemia.
    Blood 2017 Jan 31;129(3):289-298. Epub 2016 Oct 31.
    Division of Hematology, Mayo Clinic, Rochester, MN; and.
    Cryoglobulinemia is a distinct entity characterized by the presence of cryoglobulins in the serum. Cryoglobulins differ in their composition, which has an impact on the clinical presentation and the underlying disease that triggers cryoglobulin formation. Cryoglobulinemia is categorized into two main subgroups: type I, which is seen exclusively in clonal hematologic diseases, and type II/III, which is called mixed cryoglobulinemia and is seen in hepatitis C virus infection and systemic diseases such as B-cell lineage hematologic malignancies and connective tissue disorders. Read More

    Prevalence of mixed connective tissue disease in a population-based registry of American Indian/Alaska Native people in 2007.
    Arthritis Care Res (Hoboken) 2016 Oct 27. Epub 2016 Oct 27.
    Rheumatology, Department of Medicine, Emory University.
    Objective: The objective of this surveillance project was to determine the prevalence of mixed connective tissue disease (MCTD) in 2007 in the Indian Health Service (IHS) active clinical population from 3 regions of the United States.

    Methods: The IHS Lupus Registry was designed to identify possible MCTD cases in addition to lupus. The population denominator for this report includes American Indian or Alaska Native adults within the IHS active clinical population in 2007, residing in select communities in 3 regions of the US. Read More

    Effects of rituximab in connective tissue disorders related interstitial lung disease.
    Clin Exp Rheumatol 2016 Sep-Oct;34 Suppl 100(5):181-185. Epub 2016 Oct 14.
    Paris Descartes University, Rheumatology A Department, APHP, Cochin Hospital, Paris, France.
    Objectives: Interstitial lung disease (ILD) is a key prognostic factor in connective tissue disorders (CTDs). The aim of our study was to assess the changes in pulmonary functional tests (PFTs) in various CTDs, including anti-synthetase syndrome (SYN), systemic sclerosis (SSc) and mixed connective tissue disorder (MCTD), following the use of rituximab therapy.

    Methods: A multicentre retrospective analysis of patients with ILD secondary to SYN (n=15), MCTD (n=6) and SSc (n=23). Read More

    Tocilizumab in the treatment of mixed connective tissue disease and overlap syndrome in children.
    RMD Open 2016 15;2(2):e000271. Epub 2016 Sep 15.
    Department of Pediatric Rheumatology, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Claude Bernard University Lyon 1, University of Lyon, Lyon, France; INSERM U1111.
    Arthritis is one of the main manifestations of mixed connective tissue disease (MCTD) and overlap syndrome in children and can be responsible for functional disability. We report on 2 children with arthritis that were dramatically improved by a treatment with interleukin-6 (IL-6) blockers in the context of connective tissue disease. However, in both cases, other systemic autoimmune symptoms were not modified by the treatment and autoantibodies tend to increase, suggesting a differential effect of IL-6 inhibition on articular inflammation and systemic autoimmunity. Read More

    Imaging of Pulmonary Manifestations of Connective Tissue Diseases.
    Radiol Clin North Am 2016 Nov 12;54(6):1015-1031. Epub 2016 Aug 12.
    Department of Radiology, University of Washington, 1959 Northeast Pacific Street, Seattle, WA 98195, USA.
    Connective tissue diseases (CTDs) are a heterogeneous group of conditions characterized by circulating autoantibodies and autoimmune-mediated organ damage. Common CTDs with lung manifestations are rheumatoid arthritis, scleroderma or systemic sclerosis, Sjögren syndrome, polymyositis/dermatomyositis, systemic lupus erythematosis, mixed connective tissue disease, and undifferentiated connective tissue disease. The most common histopathologic patterns of CTD-related interstitial lung disease are nonspecific interstitial pneumonia, usual interstitial pneumonia, organizing pneumonia, and lymphoid interstitial pneumonia. Read More

    Clinical features and natural history of interstitial pneumonia with autoimmune features: A single center experience.
    Respir Med 2016 Oct 3;119:150-154. Epub 2016 Sep 3.
    Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USA. Electronic address:
    Objective: To describe the clinical phenotype and natural history of a cohort of patients with interstitial pneumonia with autoimmune features (IPAF).

    Methods: A retrospective, single center study of 56 patients with IPAF evaluated between February 2008 and August 2014. All clinical data were extracted from the electronic medical record and longitudinal changes in forced vital capacity (FVC) were analyzed with mixed-effects, piecewise linear regression models that considered time as a continuous factor. Read More

    Clinical association of mixed connective tissue disease and granulomatosis with polyangiitis: a case report and systematic screening of anti-U1RNP and anti-PR3 auto-antibody double positivity in ten European hospitals.
    Immunol Res 2016 Dec;64(5-6):1243-1246
    Department of Rheumatology, Lapeyronie Hospital, Montpellier University, Montpellier, France.
    We report here the case of a 50-years-old man treated for mixed connective tissue disease (MCTD) positive for anti-U1 ribonucleoprotein (U1RNP) antibodies who secondarily developed a granulomatosis with polyangiitis (GPA) associated with anti-proteinase 3 anti-neutrophil cytoplasmic antibodies (PR3-ANCA). We then evaluated the frequency of the association between anti-U1RNP and anti-PR3-ANCA antibodies by a systematic retrospective study in ten European hospitals. Overall, out of 11,921 samples analyzed for both auto-antibodies, 18 cases of anti-U1RNP and anti-PR3-ANCA double positivity were found and only one patient presented with both MCTD and GPA symptoms. Read More

    Ophthalmic Manifestations in Chronic Inflammatory Rheumatic Diseases at a Referral Hospital of Yaounde, Cameroon.
    Ocul Immunol Inflamm 2016 Sep 6:1-6. Epub 2016 Sep 6.
    b Faculty of Health Sciences , Université de Yaoundé 1 , Cameroon.
    Purpose: To determine the profile of ophthalmic manifestations in chronic inflammatory rheumatic diseases (CIRD).

    Methods: Observational study at the Yaounde Central Hospital and Innel Medical Centre (2004 to 2012).

    Results: The study population (n = 36) consisted of 14 men and 22 women with average age of 47. Read More

    [Intractable Myasthenia Gravis Accompanied with Thymoma;Report of a Case].
    Kyobu Geka 2016 Sep;69(10):881-4
    Department of Thoracic Surgery, Nagoya University, Nagoya, Japan.
    A 46-year-old female visited a hospital due to pelvic inflammatory disease (PID) and then her chest computed tomography revealed an abnormal shadow in the upper mediastinum. Four months later,she complained muscle weakness with her limbs, dysphagia, and ptosis of her eyelids. Total thymectomy was performed through a median sternotomy for mass lesion, which was pathologically proven to be type B1 thymoma. Read More

    Pathways Linked to Internuclear Ophthalmoplegia on Diffusion-Tensor Imaging in a Case with Midbrain Infarction.
    J Stroke Cerebrovasc Dis 2016 Nov 24;25(11):2575-2579. Epub 2016 Aug 24.
    Department of Neurology, Juntendo University School of Medicine, Tokyo, Japan.
    Background: Midbrain infarction shows diverse patterns of ophthalmoplegia; however, the association of ophthalmoplegia with a precise microanatomy has not been fully studied. Here, we report a patient with characteristic ophthalmoplegia and explore the associated pathologic fiber tracts using diffusion-tensor imaging (DTI).

    Methods: A 21-year-old woman with an 11-year history of mixed connective tissue disease (MCTD) abruptly developed bilateral internuclear ophthalmoplegia (INO) with upward gaze and convergence palsies. Read More

    Ortner's syndrome caused by pulmonary arterial hypertension associated with mixed connective tissue disease.
    Clin Exp Rheumatol 2016 Nov-Dec;34(6):1125. Epub 2016 Jul 26.
    Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
    ****************************************************************************. Read More

    Autologous platelet rich plasma in the management of non-healing vasculitic ulcers.
    Int J Rheum Dis 2016 Jul 26. Epub 2016 Jul 26.
    Institute of Rheumatology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, India.
    Aim: To determine the role and efficacy of platelet rich plasma (PRP) in the healing of recalcitrant vasculitic ulcers and to study the rapidity of ulcer healing with respect to platelet concentration in the prepared PRP.

    Methods: This is a pilot study which included 20 patients with biopsy-proven vasculitic ulcers after excluding human immunodeficiency virus, uncontrolled diabetes mellitus, hypertension, obesity, active smoking and platelet abnormalities in peripheral smear. PRP was prepared from whole blood by centrifugation and activated using 10% calcium chloride. Read More

    Esophageal disorders in mixed connective tissue diseases.
    J Med Life 2016 Apr-Jun;9(2):141-3
    "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania; III rd Department of General Surgery, University Emergency Hospital Bucharest, Romania.
    Extra Musculoskeletal manifestations are a distinct clinical entity that refers to a combination of clinical features, which are found in multiple rheumatic diseases. Besides the standard manifestations, other organs can be damaged such as the vascular system, skin, gastrointestinal tract, musculoskeletal system, cardiopulmonary system, hematologic system, kidneys, and the central nervous system. Among the gastrointestinal MCTD symptoms, the most frequent are the esophageal ones. Read More

    Perspectives on the Role and Relevance of Copper in Cardiac Disease.
    Biol Trace Elem Res 2017 Mar 21;176(1):10-19. Epub 2016 Jul 21.
    Division of Biochemistry and Molecular Biology, School of Graduate Studies, University of Missouri Kansas City, 300F Administrative Center, 5115 Oak Street, Kansas City, MO, 64110-2499, USA.
    Cardiac hypertrophy as a result of dietary copper deficiency has been studied for 40 plus years and is the subject of this review. While connective tissue anomalies occur, a hallmark pathology is cardiac hypertrophy, increased mitochondrial biogenesis, with disruptive cristae, vacuolization of mitochondria, and deposition of lipid droplets. Electrocardiogram abnormalities have been demonstrated along with biochemical changes especially as it relates to the copper-containing enzyme cytochrome c oxidase. Read More

    Mixed connective tissue disease-enigma variations?
    Rheumatology (Oxford) 2016 Jul 19. Epub 2016 Jul 19.
    Division of Rheumatology, Department of Medicine, Queen Elizabeth Hospital, Hong KongInternal Medicine Department, Hospital Pedro Hispano, Matosinhos, PortugalCentre for Rheumatology, Division of Medicine, University College London, London, UK
    In 1972, Sharp et al. described a new autoimmune rheumatic disease that they called MCTD, characterized by overlapping features of SSc, SLE, PM/DM, high levels of anti-U1snRNP and low steroid requirements with good prognosis. MCTD was proposed as a distinct disease. Read More

    Mixed connective tissue disease.
    Best Pract Res Clin Rheumatol 2016 Feb 12;30(1):95-111. Epub 2016 Apr 12.
    Rheumatology Unit, Oslo University Hospital Rikshospitalet, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
    The concept of mixed connective tissue disease (MCTD) as a separate connective tissue disease (CTD) has persisted for more than four decades. High titers of antibodies targeting the U1 small nuclear ribonucleoprotein particle (U1 snRNP) in peripheral blood are a sine qua non for the diagnosis of MCTD, in addition to distinct clinical features including Raynaud's phenomenon (RP), "puffy hands," arthritis, myositis, pleuritis, pericarditis, interstitial lung disease (ILD), and pulmonary hypertension (PH). Recently, population-based epidemiology data from Norway estimated the point prevalence of adult-onset MCTD to be 3. Read More

    Connective tissue disease-related pulmonary arterial hypertension.
    Best Pract Res Clin Rheumatol 2016 Feb 29;30(1):22-38. Epub 2016 May 29.
    Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney Medical School, University of Sydney, Camperdown, Australia. Electronic address:
    Over the past two decades, there have been several advances in the assessment and management of connective tissue disease-related pulmonary arterial hypertension (CTD-PAH) that improved outcomes of the treatment of this lethal disease, and this will be the focus of this study. Systemic sclerosis is the leading cause of CTD-PAH, followed by systemic lupus erythematosus, mixed connective tissue disease, idiopathic inflammatory myositis, rheumatoid arthritis, and Sjogren's syndrome. Clinical registries have been invaluable in informing about the burden of disease, risk and prognostic factors, and temporal trends with respect to treatment and outcome in CTD-PAH. Read More

    [Pulmonary involvement in connective tissue disease].
    Wiad Lek 2016 ;69(2 Pt 1):130-8
    I Klinika Chorób Płuc Instytutu Gruźlicy i Chorób Płuc ul. Płocka 26, 01-138 Warszawa tel.: 22 4312143 fax: 22 4312443 e-mail:
    The connective tissue diseases are a variable group of autoimmune mediated disorders characterized by multiorgan damage. Pulmonary complications are common, usually occur after the onset of joint symptoms, but can also be initially presenting complaint. The respiratory system may be involved in all its component: airways, vessels, parenchyma, pleura and respiratory muscles. Read More

    Causality evaluation of bacterial species isolated from patients with community-acquired lower leg cellulitis.
    J Eur Acad Dermatol Venereol 2016 Sep 12;30(9):1583-9. Epub 2016 Jul 12.
    Department of Skin and Venereal Diseases, Faculty of Medicine, School of Health Sciences, University of Ioannina and University Hospital of Ioannina, Ioannina, Greece.
    Introduction: Lower leg cellulitis is a diffuse inflammation of the cutaneous connective tissue following invasion of microorganisms and with potential to recur. The causative agent is not routinely identified in clinical practice, and the empirical therapy initiated primarily targets the 'conventional' disease pathogens, Streptococcus pyogenes and Staphylococcus aureus.

    Objective: To evaluate at case level, the role of bacterial species isolated from lesional skin in the pathogenesis of community-acquired lower leg cellulitis. Read More

    Left ventricular diastolic dysfunction and increased left ventricular mass index related to pulmonary hypertension in patients with systemic autoimmune disease without pericardial effusion.
    Int J Cardiol 2016 Oct 18;220:268-72. Epub 2016 Jun 18.
    Department of Cardiovascular Medicine, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba City, Chiba 260-8670, Japan.
    Purpose: We investigated the relationship of left ventricular (LV) diastolic dysfunction and LV mass index (LVMI) against pulmonary hypertension (PH) in systemic autoimmune disease (SAD).

    Methods: A total of 84 SAD patients (68 females; 53±17years; systemic lupus erythematosus, 27%; scleroderma, 17%; vasculitis, 16%; mixed connective tissue disease, 13% and polymyositis/dermatomyositis complex, 10%) without significant pericardial effusion (PE) on TTE (Vivid E9, GE) were analyzed. On TTE, PH was defined as peak tricuspid regurgitation velocity (TRV) of ≥2. Read More

    Case Report of Necrotizing Fasciitis Associated with Streptococcus pneumoniae.
    Can J Infect Dis Med Microbiol 2016 30;2016:6872739. Epub 2016 Mar 30.
    Department of Pathology and Molecular Medicine, McMaster University, Hamilton, ON, Canada L8S 4L8.
    Necrotizing fasciitis, caused by Streptococcus pneumoniae, is an extremely rare and life-threatening bacterial soft tissue infection. We report a case of early necrotizing fasciitis associated with Streptococcus pneumoniae infection in a 26-year-old man who was immunocompromised with mixed connective tissue disease. The patient presented with acute, painful, erythematous, and edematous skin lesions of his right lower back, which rapidly progressed to the right knee. Read More

    Can SLE classification rules be effectively applied to diagnose unclear SLE cases?
    Lupus 2016 Jun 27. Epub 2016 Jun 27.
    Department of Biological Sciences, Florida International University, Miami, FL, USA; International Forensic Research Institute, Florida International University, Miami, FL, USA
    Objective: The objective of this paper is to develop novel classification criteria to distinguish between unclear systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD) cases.

    Methods: A total of 205 variables from 111 SLE and 55 MCTD patients were evaluated to uncover unique molecular and clinical markers for each disease. Binomial logistic regressions (BLRs) were performed on currently used SLE and MCTD classification criteria sets to obtain six reduced models with power to discriminate between unclear SLE and MCTD patients that were confirmed by receiving operating characteristic (ROC) curve. Read More

    Prevalence of systemic autoimmune rheumatic diseases and clinical significance of ANA profile: data from a tertiary hospital in Shanghai, China.
    APMIS 2016 Sep 22;124(9):805-11. Epub 2016 Jun 22.
    Department of Laboratory Diagnostics, Changzheng Hospital, Second Military Medical University, Shanghai, China.
    It is necessary and useful to explore prevalence of various systemic autoimmune rheumatic diseases (SARDs) in patients with suspicion of having SARDs and to characterize antinuclear antibodies (ANA) profile for identifying different populations (SARDs and non-SARDs). A total of 5024 consecutive patients with available medical records were investigated, whose sera had been tested for ANA profile, including ANA, anti-dsDNA and anti-extractable nuclear antigen (ENA) antibodies, between 31 January 2012 and 26 March 2014. Only 594 (11. Read More

    Undifferentiated Connective Tissue Disease, Mixed Connective Tissue Disease, and Overlap Syndromes in Rheumatology.
    Mo Med 2016 Mar-Apr;113(2):136-40
    Autoimmune diseases often have overlapping symptoms and laboratory somewhat unfamiliar to the non-rheumatologist. Characteristic signs, symptoms, and autoantibodies define specific connective tissue diseases. Some patients have some characteristic symptoms, but cannot be definitively classified. Read More

    Long-term outcome in juvenile-onset mixed connective tissue disease: a nationwide Norwegian study.
    Ann Rheum Dis 2017 Jan 9;76(1):159-165. Epub 2016 Jun 9.
    Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
    Objectives: To describe the characteristics, outcome and predictive factors of juvenile mixed connective tissue disease (JMCTD) in a nationwide cohort of patients.

    Methods: We examined 55 patients with JMCTD after a mean disease duration of 16.2 years (SD 10. Read More

    Management of rheumatic and autoimmune blistering disease in pregnancy and postpartum.
    Clin Dermatol 2016 May-Jun;34(3):344-52. Epub 2016 Feb 9.
    Department of Dermatology, Perelman School of Medicine, Philadelphia, Pennsylvania; Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, Pennsylvania. Electronic address:
    The treatment of rheumatic and autoimmune skin disease in women who are pregnant or of childbearing potential can present challenges to the dermatologist. We discuss the current approaches to treating lupus erythematosus, antiphospholipid antibody syndrome, dermatomyositis, morphea and systemic sclerosis, mixed connective tissue disease, rheumatoid arthritis, and autoimmune blistering disease in such patients. In the appropriate setting, topical and systemic corticosteroids, hydroxychloroquine, dapsone, azathioprine, and ultraviolet B phototherapy may be safely and cautiously used during pregnancy. Read More

    Chronic Pruritus and Connective Tissue Disorders: Review, Gaps, and Future Directions.
    Am J Clin Dermatol 2016 Oct;17(5):445-449
    Department of Dermatology and Itch Center, Lewis Katz Medical School Temple University, 3322 North Broad Street, Medical Office Building, Suite 212, Philadelphia, PA, 19140, USA.
    Chronic itch is a common skin manifestation in many connective tissue diseases. However, the cause of chronic itch in these diseases is still not fully understood. The complex, and in some cases incomplete understanding of, pathogenesis in each condition makes it difficult to target specific mediators of chronic itch. Read More

    Clinical and serological associations of autoantibodies to the Ku70/Ku80 heterodimer determined by a novel chemiluminescent immunoassay.
    Lupus 2016 Jul;25(8):889-96
    Cumming School of Medicine, University of Calgary, Calgary, Canada.
    Background: Autoantibodies targeting Ku, an abundant nuclear protein with DNA helicase activity, have been reported in patients with systemic autoimmune rheumatic diseases. Little is known about the clinical associations of anti-Ku antibodies, especially when novel diagnostic technologies are used. The objective of the present study was to analyse the prevalence of anti-Ku antibodies in different medical conditions using a novel chemiluminescent immunoassay. Read More

    Posterior reversible encephalopathy syndrome in a patient with mixed connective tissue disease: a case report.
    J Med Case Rep 2016 Jun 2;10(1):145. Epub 2016 Jun 2.
    Bone and Joint Reconstruction Research Center, Shafa Orthopedic Hospital, Iran University of Medical Sciences, Tehran, Iran.
    Background: Posterior reversible encephalopathy is a syndrome highly associated with hypertension and cytotoxic therapy. The syndrome typically presents with headache, visual abnormality, seizures and characteristic vasogenic edema on magnetic resonance imaging. The entity warrants a prompt diagnosis to avoid deteriorating consequences. Read More

    Is rituximab an effective treatment of refractory calcinosis?
    BMJ Case Rep 2016 May 31;2016. Epub 2016 May 31.
    Internal Medicine Department, Limoges University Hospital, Limoges, France.
    Calcinosis, the deposition of calcified material in soft tissues, is frequently seen in systemic sclerosis and dermatomyositis. Treatment options are limited, with disappointing results. Some recent case reports suggest that rituximab may be an attractive therapeutic option. Read More

    Successful Immunosuppressive Treatment of Mixed Connective Tissue Disease Complicated by Microscopic Polyangiitis.
    Tohoku J Exp Med 2016 ;239(2):111-6
    Department of Gastroenterology and Rheumatology, Fukushima Medical University School of Medicine.
    Mixed connective tissue disease (MCTD) is characterized by a combination of clinical features of systemic lupus erythematosus, systemic sclerosis, and polymyositis with elevated antibodies to U1 small nuclear ribonucleoprotein (U1-RNP). MCTD is often accompanied by interstitial lung disease as pulmonary involvement. On the other hand, microscopic polyangiitis (MPA) is a systemic autoimmune disease characterized by the inflammation of small vessels (arterioles, capillaries, and venules) mainly affecting the lung and kidney. Read More

    Comparison of Screening Dilution and Automated Reading for Antinuclear Antibody Detection on HEP2 Cells in the Monitoring of Connective Tissue Diseases.
    J Clin Lab Anal 2016 Sep 26;30(5):471-8. Epub 2016 May 26.
    Laboratoire d'Immunologie et d'Immunomonitoring, CIC 1408 INSERM, GIMAP EA3064, CHU de Saint-Etienne (France), Werfen Instrument Laboratory, France.
    Background: Indirect immunofluorescence plays a major role in the detection of antinuclear antibodies (ANAs) and follow-up of their titers in the context of connective tissue diseases. Given the numerous unfavorable features of the conventional manual reading of HEP2 slides (need of time and expert morphologists for the reading, lack of standardization, subjectivity of the interpretation), the biomedical industry has developed automated techniques of slide preparation and microscope reading.

    Methods: We collected 49 sera beforehand analyzed by the conventional reading of slides. Read More

    Association between antinuclear antibodies titers and connective tissue diseases in a Rheumatology Department.
    Reumatol Clin 2016 May 21. Epub 2016 May 21.
    Sección de Reumatología, Hospital General de Jerez, Jerez de la Frontera, España.
    Objective: To determine the dilution titles at antinuclear antibodies (ANA) by indirect immunofluorescence observed in cell substrate HEp-2 and its association with the diagnosis of systemic connective tissue disease in ANA test requested by a Rheumatology Unit.

    Method: Samples of patients attended for the first time in the rheumatology unit, without prior ANA test, between January 2010 and December 2012 were selected. The dilution titers, immunofluorescence patterns and antigen specificity were recorded. Read More

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