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Undifferentiated Connective Tissue Disease

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https://www.readbyqxmd.com/read/29745875/interstitial-lung-disease-in-patients-with-mixed-connective-tissue-disease-pilot-study-on-predictors-of-lung-involvement
#1
Neha Narula, Tathagat Narula, Isabel Mira-Avendano, Benjamin Wang, Andy Abril
OBJECTIVES: Mixed connective tissue disease (MCTD) is an immune-mediated systemic disorder characterised by serum autoantibodies against U1-ribonucleoprotein and diverse multisystemic clinical manifestations. Approximately 50% of patients with MCTD develop a radiologic pattern of interstitial lung disease (ILD). Our single centre, cross-sectional study sought to identify clinical and serologic associations of ILD in patients with MCTD which may serve as predictors of lung disease and prognosis...
May 8, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29713867/treatment-of-adult-idiopathic-inflammatory-myopathies-with-conventional-immunosuppressive-drugs-results-of-a-retrospective-study
#2
G Keyßer, S Zierz, M Kornhuber
OBJECTIVES: To gain information about the efficacy of immunosuppressive drugs as first-, second-, and third-line treatment of idiopathic inflammatory myopathies (IIM). METHODS: 112 treatment cycles of 63 patients with dermatomyositis (n = 23), polymyositis (n = 33), overlap syndromes (n = 4), and undifferentiated connective tissue diseases (n = 3) were analyzed by retrospective chart analysis. Data regarding muscle strength, muscle enzymes, treatment duration, and treatment discontinuation were collected...
April 30, 2018: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/29667101/clinical-features-risk-factors-and-outcomes-of-patients-with-interstitial-pneumonia-with-autoimmune-features-a-population-based-study
#3
Jinghong Dai, Lei Wang, Xin Yan, Hui Li, Kefeng Zhou, Jian He, Fanqing Meng, Siyi Xu, Geyu Liang, Hourong Cai
To investigate the clinical features, risk factors and outcomes of patients with interstitial pneumonia with autoimmune features (IPAF). A total of 1429 patients with idiopathic interstitial pneumonia (IIP) and undifferentiated connective tissue disease-associated interstitial lung disease (UCTD-ILD) were screened to identify patients who met IPAF criteria. Clinical, serological, and morphological features of patients with IPAF were characterized. Outcomes between patients with IPAF, UCTD-ILD, and IIP who were divided into idiopathic pulmonary fibrosis (IPF) and non-IPF groups were compared using survival as an endpoint...
April 18, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29618128/anti-dfs70-autoantibodies-in-undifferentiated-connective-tissue-diseases-subjects-what-s-on-the-horizon
#4
Maria Infantino, Ora Shovman, Dolores Pérez, Mariangela Manfredi, Valentina Grossi, Maurizio Benucci, Francesca Li Gobbi, Francesca Bandinelli, Arianna Damiani, Paolo Moscato, Danielle Azoulay, Boris Gilburd, Yehuda Shoenfeld
Objective: The main objective was to determine the prevalence of anti-dense fine speckled (DFS70) antibodies in a stable population of undifferentiated connective tissue disease (UCTD) to better define their potential role. Methods: Immunological and clinical records of 91 long-standing UCTD patients were studied. DFS pattern was determined using the IIF ANA test on HEp-2 cells and anti-DFS70 antibodies were tested by chemiluminescence assay and by DFS70 line immunoassay...
April 3, 2018: Rheumatology
https://www.readbyqxmd.com/read/29465359/socio-economic-inequalities-in-occurrence-and-health-care-costs-in-rheumatic-and-musculoskeletal-diseases-results-from-a-spanish-population-based-study-including-1-9-million-persons
#5
Polina Putrik, Sofia Ramiro, Juan F Orueta, Andras Keszei, Edurne Alonso Moran, Roberto Nuño Solinis, Annelies Boonen
OBJECTIVES: To explore and compare the impact of socio-economic deprivation on the occurrence of the major rheumatic and musculoskeletal diseases (RMDs) and health care costs. METHODS: Data on diagnoses, socio-demographics and health care costs of the entire adult population of the Basque Country (Spain) was used. Area deprivation index included five categories (1 to 5 (most deprived)). Cost categories included primary and specialist care, emergency room, hospitalisations, and drug prescriptions...
January 31, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29447658/primary-anetoderma-with-undifferentiated-connective-tissue-disease
#6
Lana X Tong, Jenna Beasley, Shane Meehan, Evan Rieder, Miriam Pomeranz, Kristen Lo Sicco, Alisa Femia, Randie Kim, Andrew G Franks
Anetoderma is a rare benign elastolytic disorder that is characterized by focal loss of elastin fibers on histopathology and is often recalcitrant to treatment. We present a case of a patient with a 20-year history of pruritic and painful hyperpigmented atrophic papules clustered on the neck, axillae, inframammary folds, and right medial thigh. Although the histopathologyof her axillary lesions was consistent with anetoderma, her clinical presentation is unusual given the extent of involvement, reported pain and pruritus, and sharp demarcation of the distribution...
December 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/29395482/podocyte-infolding-glomerulopathy-pig-in-a-patient-with-undifferentiated-connective-tissue-disease-a-case-report
#7
Smita Mary Matthai, Anjali Mohapatra, Ashish J Mathew, Sanjeet Roy, Santosh Varughese, Debashish Danda, Veerasamy Tamilarasi
Podocyte infolding glomerulopathy (PIG) is a recently described pathologic entity characterized by diffuse podocyte infolding into the glomerular basement membrane (GBM) associated with ultrastructurally demonstrable microspherular aggregates. The clinical features, significance, and pathogenesis of this condition are still not well delineated because only a few cases have been documented to date, all from Japan. We report a case of PIG associated with undifferentiated connective tissue disease in an Indian woman who presented with nephrotic syndrome while undergoing treatment for an autoimmune disorder...
January 27, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29389023/innate-immunity-and-cellular-senescence-the-good-and-the-bad-in-the-developmental-and-aged-brain
#8
REVIEW
Antonietta Santoro, Chiara Carmela Spinelli, Stefania Martucciello, Stefania Lucia Nori, Mario Capunzo, Annibale Alessandro Puca, Elena Ciaglia
Ongoing studies evidence cellular senescence in undifferentiated and specialized cells from tissues of all ages. Although it is believed that senescence plays a wider role in several stress responses in the mature age, its participation in certain physiological and pathological processes throughout life is coming to light. The "senescence machinery" has been observed in all brain cell populations, including components of innate immunity (e.g., microglia and astrocytes). As the beneficial versus detrimental implications of senescence is an open question, we aimed to analyze the contribution of immune responses in regulatory mechanisms governing its distinct functions in healthy (development, organogenesis, danger patrolling events) and diseased brain (glioma, neuroinflammation, neurodeneration), and the putative connection between cellular and molecular events governing the 2 states...
March 2018: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/29320807/overlap-of-interstitial-pneumonia-with-autoimmune-features-with-undifferentiated-connective-tissue-disease-and-contribution-of-uip-to-mortality
#9
Bryan T Kelly, Teng Moua
BACKGROUND AND OBJECTIVE: Criteria for interstitial pneumonia with autoimmune features (IPAF) were recently established for research purposes in a joint statement from the European Respiratory Society (ERS) and American Thoracic Society (ATS). We reviewed the utility of these criteria in patients previously diagnosed as broadly defined undifferentiated connective tissue disease (UCTD) and noted overlapping IPAF findings. Additional review was given to IPAF patients with usual interstitial pneumonia (UIP) on histopathology or radiology in terms of survival and outcome...
January 10, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29184701/paneth-cells-in-intestinal-physiology-and-pathophysiology
#10
REVIEW
Nikolaus Gassler
Small intestinal mucosa is characterised by villus forming connective tissues with highly specialised surface lining epithelial cells essentially contributing to the establishment of the intestinal border. In order to perform these diverse functions, spatially distinct compartments of epithelial differentiation are found along the crypt-villus axis, including Paneth cells as a highly specialised cell type. Paneth cells locate in crypts and assist undifferentiated columnar cells, called crypt base columnar cells, and rapidly amplifying cells in the regeneration of absorptive and secretory cell types...
November 15, 2017: World Journal of Gastrointestinal Pathophysiology
https://www.readbyqxmd.com/read/28993902/cardiac-involvement-in-undifferentiated-connective-tissue-disease-at-risk-for-systemic-sclerosis-otherwise-referred-to-as-very-early-early-systemic-sclerosis-a-tdi-study
#11
Michele D'Alto, Antonella Riccardi, Paola Argiento, Ilaria Di Stefano, Emanuele Romeo, Agostino Mattera Iacono, Antonello D'Andrea, Serena Fasano, Alessandro Sanduzzi, Marialuisa Bocchino, Ludovico Docimo, Salvatore Tolone, Maria Giovanna Russo, Gabriele Valentini
Undifferentiated connective tissue disease at risk for systemic sclerosis (UCTD-risk-SSc), otherwise referred to as very early-early SSc, is a condition characterized by Raynaud's phenomenon with serum SSc marker autoantibodies and/or typical capillaroscopic findings and unsatisfying classification criteria for the disease. The aim of the present study was to assess the prevalence of right (RV) or left ventricular (LV) systolic and/or diastolic dysfunction by standard echocardiography and tissue Doppler imaging (TDI)...
May 2018: Clinical and Experimental Medicine
https://www.readbyqxmd.com/read/28937580/diagnosis-of-systemic-inflammatory-diseases-among-patients-admitted-for-acute-pericarditis-with-pericardial-effusion
#12
Maureen Assayag, Rachid Abbas, Noémie Chanson, Anne Perozziello, Gregory Ducrocq, Jean-François Alexandra, Antoine Dossier, Thomas Papo, Karim Sacre
AIMS: Acute pericarditis may be the heralding manifestation of various systemic inflammatory diseases (SIDs). The aim of this study was to identify clinical indicators for SIDs in patients admitted for acute pericarditis with pericardial effusion. METHODS: All consecutive adult patients hospitalized in a Department of Internal Medicine over a 10-year period for acute pericarditis with pericardial effusion were retrospectively reviewed. Patients with cancer and tuberculosis were excluded...
November 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28921728/undifferentiated-connective-tissue-diseases-and-adverse-pregnancy-outcomes-an-undervalued-association
#13
REVIEW
Arsenio Spinillo, Fausta Beneventi, Roberto Caporali, Veronique Ramoni, Carlomaurizio Montecucco
Undifferentiated connective tissue diseases (UCTDs) are a heterogeneous group of disorders characterized by symptoms and signs suggestive of systemic autoimmune rheumatic disease (ARD), but which do not fulfill all the established criteria for definite diagnosis of a condition. Although a third of UCTDs can progress to a definite ARD within months or years, most UCTDs can remain stable for years with minimal disease activity. The annual incidence of UCTD in the general population ranges from 14 to 140 per 100 000 people...
December 2017: American Journal of Reproductive Immunology: AJRI
https://www.readbyqxmd.com/read/28904695/a-case-of-gestational-gigantomastia-in-a-37-years-old-woman-associated-with-elevated-ana-a-casual-linkage
#14
Nicola Zingaretti, Fabrizio De Biasio, Michele Riccio, Nastassia Nardini, Laura Mariuzzi, Pier Camillo Parodi
Hypertrophy of the breast (macromastia and gigantomastia) is a rare medical condition of the breast connective tissues. The etiology of this condition is still not clear; rarely, gigantomastia has been reported to develop in the setting of an autoimmune illness. We reported a case of a 37-years-old woman with undifferentiated connective tissue disease of 2-years duration presented with enlargement of breasts. The breast enlargment started at 5 months of gestation. She successfully underwent reduction mammoplasty with free nipple graft...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28835902/the-microbiome-in-connective-tissue-diseases-and-vasculitides-an-updated-narrative-review
#15
REVIEW
Rossella Talotta, Fabiola Atzeni, Maria Chiara Ditto, Maria Chiara Gerardi, Piercarlo Sarzi-Puttini
OBJECTIVE: To provide a narrative review of the most recent data concerning the involvement of the microbiome in the pathogenesis of connective tissue diseases (CTDs) and vasculitides. METHODS: The PubMed database was searched for articles using combinations of words or terms that included systemic lupus erythematosus, systemic sclerosis, autoimmune myositis, Sjögren's syndrome, undifferentiated and mixed CTD, vasculitis, microbiota, microbiome, and dysbiosis. Papers from the reference lists of the articles and book chapters were reviewed, and relevant publications were identified...
2017: Journal of Immunology Research
https://www.readbyqxmd.com/read/28792000/-lower-extremity-varicose-veins-in-childhood-and-at-a-young-age-mechanism-of-development-and-specific-features
#16
REVIEW
V V Studennikova, L O Severgina, A N Dzyundzya, I A Korovin
In Russia more than 125,000 patients with various venous diseases, lower extremity varicose veins (LEVV) being predominant, were annually operated on. In recent years, there has been a trend toward younger patients with signs of LEVV. Screening studies have revealed the signs of the disease in 10-15% of high-school children. The high prevalence of LEVV as a whole and its younger onset in recent decades cause more attention to an investigation of the relationship between the development of varicose veins, in childhood and adolescence in particular, and genomic changes...
2017: Arkhiv Patologii
https://www.readbyqxmd.com/read/28770704/undifferentiated-connective-tissue-disease-predictors-of-evolution-into-definite-disease
#17
María García-González, Beatriz Rodríguez-Lozano, Sagrario Bustabad, Ivan Ferraz-Amaro
OBJECTIVES: The natural evolution of undifferentiated connective tissue diseases (UCTD) has not yet been established. The aim of our study was to analyse the clinical outcomes of a cohort of UCTD patients followed in a routine outpatient setting and to establish which clinical, serological or capillaroscopy features are associated with an increased risk of evolution to definite connective tissue disease (CTD). METHODS: Data for this study were collected by a retrospective review of 758 patients referred to our hospital, between 1999 and 2008, with suspected CTD...
September 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28747599/interstitial-pneumonia-with-autoimmune-features
#18
REVIEW
Osamu Nishiyama, Yuji Tohda
Some patients with idiopathic interstitial pneumonias (IIPs) have clinical features that suggest autoimmune process but not fulfill established criteria for connective tissue disease (CTD). Different terms and criteria have been proposed to describe these patients, which includes undifferentiated connective tissue disease (UCTD), lung dominant connective tissue disease (LD-CTD), and autoimmune-featured interstitial lung disease (AIF-ILD). To marshal these ununiform classification criteria, the "European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease" proposed a consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity with the term "interstitial pneumonia with autoimmune features" (IPAF)...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28741088/the-autoimmune-inflammatory-syndrome-induced-by-adjuvants-asia-shoenfeld-s-syndrome-descriptive-analysis-of-300-patients-from-the-international-asia-syndrome-registry
#19
Abdulla Watad, Mariana Quaresma, Nicola Luigi Bragazzi, Ricard Cervera, Jan Willem Cohen Tervaert, Howard Amital, Yehuda Shoenfeld
The autoimmune/inflammatory syndrome induced by adjuvants (ASIA) is a recently identified condition in which the exposure to an adjuvant leads to an aberrant autoimmune response. We aimed to summarize the results obtained from the ASIA syndrome registry up to December 2016, in a descriptive analysis of 300 cases of ASIA syndrome, with a focus on the adjuvants, the clinical manifestations, and the relationship with other autoimmune diseases. A Web-based registry, based on a multicenter international study, collected clinical and laboratory data in a form of a questionnaire applied to patients with ASIA syndrome...
February 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/28663326/clinical-associations-of-the-positive-anti-ro52-without-ro60-autoantibodies-undifferentiated-connective-tissue-diseases
#20
Sai H K Murng, Moira Thomas
AIMS: Autoantibodies targeting Ro52 and Ro60 antigens are historically reported as anti SSA/Ro. In general anti SSA/Ro results are either anti Ro52+Ro60+ or anti Ro52-Ro60+ antibodies. Anti Ro52 without anti Ro60 (Ro52+ Ro60-) antibodies are often not reported routinely. This study intends to review the potential significance of these autoantibodies in the management of connective tissue diseases. METHOD: A retrospective survey of Ro52+Ro60- was carried out as part of the service evaluation of extractable nuclear antigen antibodies (ENA) reporting from the immunology laboratory, the NHS Greater Glasgow and Clyde (GGC), UK...
January 2018: Journal of Clinical Pathology
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