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Mixed connective tissue disease

Shantanu Dixit, Chaithra Kalkur, Atul P Sattur, Michael M Bornstein, Fred Melton
BACKGROUND: Scleroderma is a chronic connective tissue disorder with unknown etiology. It is characterized by excessive deposition of extracellular matrix in the connective tissues causing vascular disturbances which can result in tissue hypoxia. These changes are manifested as atrophy of the skin and/or mucosa, subcutaneous tissue, muscles, and internal organs. Such changes can be classified into two types, namely, morphea (localized) and diffuse (systemic). Morphea can manifest itself as hemifacial atrophy (Parry-Romberg syndrome) although this remains debatable...
October 24, 2016: Journal of Medical Case Reports
Gemma Lepri, Jerome Avouac, Paolo Airò, Francisco Anguita Santos, Silvia Bellando-Randone, Jelena Blagojevic, Francisco Garcia Hernàndez, Jose Antonio Gonzalez Nieto, Serena Guiducci, Suzana Jordan, Vidya Limaye, Britta Maurer, Albert Selva-O'Callaghan, Valeria Riccieri, Oliver Distler, Marco Matucci-Cerinic, Yannick Allanore
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)...
September 2016: Clinical and Experimental Rheumatology
Natalia Cabrera, Agnes Duquesne, Marine Desjonquères, Jean-Paul Larbre, Jean-Christophe Lega, Nicole Fabien, Alexandre Belot
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...
2016: RMD Open
Aasritha Mekala, Y S Sarma, S V Ramana Murthy
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
P Lakshmi Raghavendra, Uma Ma, Srinivasa Rao, Yjv Reddy
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Jitesh Ahuja, Deepika Arora, Jeffrey P Kanne, Travis S Henry, J David Godwin
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...
November 2016: Radiologic Clinics of North America
Masanobu Sakaguchi, Tomoki Takeda, Takao Kodera, Kazuhiro Murakami, Masahiro Oka
No abstract text is available yet for this article.
September 29, 2016: European Journal of Dermatology: EJD
Amandine Tubery, Françoise Fortenfant, Bernard Combe, Isabelle Abreu, Xavier Bossuyt, Pascale Chretien, Sophie Desplat-Jégo, Nicole Fabien, Sophie Hue, Catherine Johanet, Daniela Lakomy, Thierry Vincent, Claire I Daïen
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...
September 12, 2016: Immunologic Research
Christelle Domngang Noche, Giles Kagmeni, Viola Dohvoma, Assumpta Lucienne Bella, Come Ebana Mvogo, Madeleine Singwe-Ngandeu
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 47.9 ± 17.2 years. Cases of CIRD were rheumatoid arthritis (n = 16), systemic lupus erythematosus (n = 8), ankylosing spondylitis (n = 8), mixed connective tissue disease (n = 2), scleroderma (n = 1), and juvenile idiopathic arthritis (n = 1)...
September 6, 2016: Ocular Immunology and Inflammation
Akira Naomi, Yoshinori Oyamatsu, Kunio Narita, Masato Nakayama, Shoki Maeda
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. Postoperative myasthenia gravis (MG) crisis, which led to respiratory failure requiring intubation and mechanical ventilation, developed and laboratory tests showed elevated serum anti-AChR Ab(130 nmol/l), antinuclear antibody( ×640 serum dilution, speckled pattern) and anti-RNP Ab(129...
September 2016: Kyobu Geka. the Japanese Journal of Thoracic Surgery
Haruka Takeshige, Yuji Ueno, Koji Kamagata, Fuyuko Sasaki, Kazuo Yamashiro, Ryota Tanaka, Shigeki Aoki, Nobutaka Hattori
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...
August 24, 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Mariko Ogawa-Momohara, Yoshinao Muro, Akihiro Hirashiki, Yasushi Fujimoto, Takahisa Kondo, Masashi Akiyama
July 26, 2016: Clinical and Experimental Rheumatology
Sankaran Sriram, Rajeswari Sankaralingam, Madeshwaran Mani, Tiruchengode Natesan Tamilselvam
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...
July 26, 2016: International Journal of Rheumatic Diseases
A E Nica, L M Alexa, A O Ionescu, O Andronic, D N Păduraru
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. Treatment of patients with MCTD must be performed by both medical and surgical multidisciplinary teams in order to provide a management suitable for the patients' needs...
April 2016: Journal of Medicine and Life
Denis M Medeiros
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. The master controller of mitochondrial biogenesis, PGC1-α expression and protein, along with other proteins and transcriptional factors that play a role are upregulated...
July 21, 2016: Biological Trace Element Research
Natalia C O Ciang, Nídia Pereira, David A Isenberg
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. However, soon after the original description, questions about the existence of such a syndrome as well as disputes over the features initially described began to surface. The conundrum of whether MCTD is a distinct disease entity remains controversial...
July 19, 2016: Rheumatology
Ragnar Gunnarsson, Siri Opsahl Hetlevik, Vibke Lilleby, Øyvind Molberg
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...
February 2016: Best Practice & Research. Clinical Rheumatology
Vivek Thakkar, Edmund M T Lau
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...
February 2016: Best Practice & Research. Clinical Rheumatology
Małgorzata Bartosiewicz
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. Lung involvement is an increasing cause of morbidity and mortality in the connective tissue diseases. Clinical course is highly variable - can range from mild to rapidly progressive, some processes are reversible, while others are irreversible...
2016: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
I Chaniotakis, C G Gartzonika, G Gaitanis, S Levidiotou-Stefanou, I D Bassukas
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...
September 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
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