658 results match your criteria Undifferentiated Connective-Tissue Disease


Autoantibodies as biomarkers for interstitial lung disease in idiopathic inflammatory myositis and systemic sclerosis: The case of anti-eIF2B antibodies.

J Transl Autoimmun 2020 31;3:100049. Epub 2020 Mar 31.

Division of Rheumatology and Clinical Immunology, Laboratory of Autoimmunity and Metabolism, Humanitas Clinical and Research Center - IRCCS -, via Manzoni 56, 20089, Rozzano, MI, Italy.

Objectives: Serum autoantibodies are pivotal for the early detection of systemic autoimmune rheumatic diseases such as Systemic Sclerosis (SSc) and Poly/Dermatomyositis (PM/DM), and in some cases are associated with organ complications such as interstitial lung disease (ILD). A paradigmatic example is provided by the autoantibody against the Eukaryotic Initiation Factor 2B (eIF2B) that has been recently detected in SSc.

Methods: Sera from 118 patients with SSc, 8 Poly/Dermatomyositis, 2 overlap SSc/Polymyositis, 4 undifferentiated connective tissue disease-UCTD and 3 healthy controls were tested first by indirect immunofluorescence for anti-nuclear antibodies-ANA pattern. Read More

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http://dx.doi.org/10.1016/j.jtauto.2020.100049DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7388402PMC

Impact of the new 2019 EULAR/ACR classification criteria for Systemic Lupus Erythematosus in a multicenter cohort study of 133 women with undifferentiated connective tissue disease.

Arthritis Care Res (Hoboken) 2020 Jul 23. Epub 2020 Jul 23.

Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, and SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy.

Objective: We aimed to investigate the impact of applying the 2019 EULAR/ACR classification criteria for systemic lupus erythematosus (SLE) in a previously described cohort of women with undifferentiated connective tissue disease (UCTD).

Methods: This study included 133 women with UCTD. At the time of inclusion into the study, none of the patients meet any classification criteria for other defined systemic connective tissue disease. Read More

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http://dx.doi.org/10.1002/acr.24391DOI Listing

Antiphospholipid autoantibody detection is important in all patients with systemic autoimmune diseases.

J Autoimmun 2020 Jul 18:102524. Epub 2020 Jul 18.

Laboratory of Immunology and Immunotherapy, CHRU Morvan, Brest, France; UMR1227, Lymphocytes B et Autoimmunité, Université de Brest, INSERM, CHU de Brest, Brest, France. Electronic address:

Antiphospholipid (aPL) autoantibodies are uncommon in systemic autoimmune diseases (SADs). However, the European PRECISESADS study provides the opportunity to better characterize this rare association. The study was composed of 1818 patients with SADs including 453 with systemic lupus erythematosus (SLE), 359 with rheumatoid arthritis (RA), 385 with systemic sclerosis (SSc), 367 with Sjögren's syndrome (SjS), 94 with mixed connective tissue disease (MCTD), and 160 with undifferentiated connective tissue disease (UCTD). Read More

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http://dx.doi.org/10.1016/j.jaut.2020.102524DOI Listing

IgM antibodies against malondialdehyde and phosphorylcholine in different systemic rheumatic diseases.

Sci Rep 2020 Jul 3;10(1):11010. Epub 2020 Jul 3.

Unit of Immunology and Chronic Disease, Institute of Environmental Medicine, Karolinska Institutet, Stockholm, Sweden.

IgM antibodies against phosphorylcholine (anti-PC) and malondialdehyde (anti-MDA) may have protective properties in cardiovascular and rheumatic diseases. We here compare these antibodies in systemic rheumatic conditions and study their properties. Anti-PC and anti-MDA was measured using ELISA in patients with SLE (374), RA (354), Mixed connective tissue disease (MCTD, 77), Systemic sclerosis (SSc, 331), Sjögren's syndrome (SjS, 324), primary antiphospholipid syndrome (PAPs, 65), undifferentiated connective tissue disease (UCTD, 118) and 515 matched healthy controls (HC). Read More

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http://dx.doi.org/10.1038/s41598-020-66981-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7335044PMC

Alveolar soft-part sarcoma (ASPS) resembles a mesenchymal stromal progenitor: evidence from meta-analysis of transcriptomic data.

Authors:
Luke H Stockwin

PeerJ 2020 19;8:e9394. Epub 2020 Jun 19.

Unaffiliated, Frederick, Maryland.

Alveolar soft-part sarcoma (ASPS) is an extremely rare malignancy characterized by the unbalanced translocation der(17)t(X;17)(p11;q25). This translocation generates a fusion protein, ASPL-TFE3, that drives pathogenesis through aberrant transcriptional activity. Although considerable progress has been made in identifying ASPS therapeutic vulnerabilities (e. Read More

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http://dx.doi.org/10.7717/peerj.9394DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7307565PMC

Giant Cell Hepatitis - A Rare Association with Connective Tissue Disease.

Mediterr J Rheumatol 2019 Dec 31;30(4):224-227. Epub 2020 Mar 31.

Rheumatology Department, Luton & Dunstable University Hospital, Luton, United Kingdom.

A 68-year-old gentleman presented to hepatology department with asymptomatic year-long history of stably deranged liver function tests. His peak alkaline phosphatase (ALP), was 828 with alanine transaminase (ALT) of 141. Full liver workup was negative; hence, a liver biopsy was organised, which confirmed giant cell hepatitis (GCH). Read More

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http://dx.doi.org/10.31138/mjr.30.4.224DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7241662PMC
December 2019

Metabolic Disturbances in Urinary and Plasma Samples from Seven Different Systemic Autoimmune Diseases Detected by HPLC-ESI-QTOF-MS.

J Proteome Res 2020 Jun 8. Epub 2020 Jun 8.

Department of Analytical Chemistry, Faculty of Science, University of Granada, Av. Fuentenueva s/n, Granada 18071, Spain.

Systemic autoimmune diseases (SADs) are characterized by dysfunctioning of the immune system, which causes damage in several tissues and organs. Among these pathologies are systemic lupus erythematosus (SLE), systemic sclerosis or scleroderma, Sjögren's syndrome, rheumatoid arthritis, primary antiphospholipid syndrome (PAPS), mixed connective tissue disease (MCTD), 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

Rituximab or cyclosporin in refractory immune thrombocytopenia secondary to connective tissue diseases: a real-world observational retrospective study.

Clin Rheumatol 2020 May 16. Epub 2020 May 16.

Department of Rheumatology, South Campus, Ren Ji Hospital, School of Medicine, Shanghai Jiaotong University, Jiang Yue Road 2000, Shanghai, 201112, China.

Immune thrombocytopenia (ITP) is a common complication of connective tissue diseases (CTD). However, refractory and recurrent cases are frequent, who often need intensive immunotherapy. In the real world to compare the efficacy and safety of two common options, rituximab (RTX) and cyclosporine (CsA), in patients with refractory CTD-ITP, we conducted this retrospective study. Read More

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http://dx.doi.org/10.1007/s10067-020-05152-xDOI Listing

Ocular manifestations in rheumatoid arthritis, connective tissue disease and vasculitis: a systematic review and meta-analysis.

J Rheumatol 2020 May 1. Epub 2020 May 1.

From the Schulich School of Medicine, University of Western Ontario, St. Joseph's Health Care, London, Ontario, Canada; University of Toronto, Toronto, Ontario, Canada; Canadian Rheumatology Association Research studentship, University of Western Ontario. This funding was supported through the Canadian Rheumatology Association Roche summer studentship. There are no conflicts of interest. IRB was not necessary as this was a systematic literature review. Address correspondence to Dr. Janet Pope MD MPH FRCPC, Division of Rheumatology, St. Joseph's Health Care, 268 Grosvenor St. London ON N6A 4V2,

Objective: Rheumatoid Arthritis and other rheumatic diseases may present with ocular manifestations. The purpose of our work was to determine the prevalence and type of eye involvement in rheumatoid arthritis and other connective tissue diseases through a meta-analysis and literature review.

Methods: A systematic review of the literature was performed using Medline, Web of Science, and the Cochrane library from their inceptions until January 7, 2019. Read More

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http://dx.doi.org/10.3899/jrheum.190768DOI Listing

Constrictive (Obliterative) Bronchiolitis as Presenting Manifestation of Connective Tissue Diseases.

J Clin Rheumatol 2020 Aug;26(5):176-180

Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN.

Background: Constrictive (obliterative) bronchiolitis (CB) is an uncommon form of obstructive lung disease that can occur in patients with identifiable causes including connective tissue diseases (CTDs) as a form of lung involvement. We explored whether CB can be the presenting manifestation of CTD.

Methods: We identified 44 patients with cryptogenic CB and examined the presenting clinical, laboratory, and radiologic features, as well as their clinical course. Read More

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http://dx.doi.org/10.1097/RHU.0000000000001387DOI Listing

The clinical phenotype associated with antisynthetase autoantibodies.

Reumatologia 2020 28;58(1):4-8. Epub 2020 Feb 28.

Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdansk, Gdansk, Poland.

Objectives: Specific systemic autoimmune syndrome characterized by inflammatory myopathy, arthritis or arthralgias, interstitial lung disease (ILD), fever, Raynaud's phenomenon, and mechanic's hands is called antisynthetase syndrome (AS). The aim of this study was to assess the clinical spectrum associated with presence of aminoacyl-transfer RNA synthetase autoantibodies (ASA).

Material And Methods: A total of 305 patients with presence of myositis-specific autoantibodies were identified in the database of immunological tests performed in the Clinical Immunology and Transplantology Unit, Medical University of Gdansk between January 2011 and March 2016. Read More

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http://dx.doi.org/10.5114/reum.2020.93505DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7174798PMC
February 2020

State-of-the-art technologies provide new insights linking skin and blood vessel abnormalities in SSc-related disorders.

Microvasc Res 2020 07 19;130:104006. Epub 2020 Apr 19.

Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, United Kingdom of Great Britain and Northern Ireland; Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom of Great Britain and Northern Ireland.

Objective: A key unanswered question in systemic sclerosis (SSc) is how microvascular abnormality and fibrosis inter-relate. Our aim was to use state-of-the-art non-invasive imaging methods to gain new insights into pathophysiology, comparing patients with different subtypes of SSc, including early dcSSc, not only to healthy controls but also to patients with causes of Raynaud's phenomenon not progressing to fibrosis.

Methods: Laser Doppler imaging, nailfold capillaroscopy, spectroscopy, and ultrasound measured (respectively) perfusion, microvascular structure, oxygenation/oxidative stress, and skin thickening in the hands of 265 subjects: 31 patients with primary Raynaud's phenomenon (PRP), 35 with undifferentiated connective tissue disease (UCTD), 93 with limited cutaneous SSc (lcSSc), 46 with diffuse cutaneous SSc (dcSSc, including 27 'early') and 60 healthy controls. Read More

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http://dx.doi.org/10.1016/j.mvr.2020.104006DOI Listing

Advances in stem cell therapy for the treatment of Peyronie's disease.

Intractable Rare Dis Res 2020 Feb;9(1):10-13

Clinic of Dermatology, Dr. Brinkmann, Schult & Samimi-Fard, Gladbeck, Germany.

Peyronie's disease (PD) is a connective tissue disorder of the penis characterized by fibrosis and plaque formation within the tunica albuginea. PD is characterized by painful penile curvature that impairs sexual intercourse. Stem cell therapy is one of the recent non-invasive treatment options for patients with PD and it has promising results. Read More

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http://dx.doi.org/10.5582/irdr.2019.01130DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7062597PMC
February 2020

Comment on: Pregnancy and undifferentiated connective tissue disease: outcome and risk of flare in 100 pregnancies.

Rheumatology (Oxford) 2020 Jun;59(6):1457-1458

Department of Rheumatology, Renji Hospital, Shanghai Jiaotong University, Shanghai, China.

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http://dx.doi.org/10.1093/rheumatology/keaa065DOI Listing

Podocyte infolding glomerulopathy with undifferentiated connective tissue disease: a case report.

Ultrastruct Pathol 2020 Mar 14;44(2):245-248. Epub 2020 Mar 14.

Department of Pathology, School of Basic Medical Science, Shanghai Medical College, Fudan University, Shanghai, China.

Podocyte infolding glomerulopathy (PIG) is a special type of glomerular disease that has been proposed in recent years and has attracted considerable attention. PIG is characterized by the formation of microspheres and microtubules in thickened glomerular basement membrane (GBM) on electron microscopy (EM), which is recognized as podocyte cytoplasmic infolding to the GBM. However, to date, only a few cases of PIG have been reported. Read More

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http://dx.doi.org/10.1080/01913123.2020.1739791DOI Listing

Vitamin D and connective tissue diseases.

Inflamm Res 2020 May 14;69(5):453-462. Epub 2020 Mar 14.

Department of Medical and Surgical Sciences, Rheumatology Clinic, Ospedali Riuniti Foggia, University of Foggia, Viale Pinto 1, 71121, Foggia, Italy.

Objective And Design: Recently, many studies have shown that the biologically active form of vitamin D-1,25(OH) D-is involved in many biological processes, including immune system modulation, and patients affected by various autoimmune diseases, such as connective tissue diseases (CTD), showed low levels of vitamin D. It is not clear if vitamin D deficiency is involved in the pathogenesis of autoimmune diseases or it is a consequence.

Material: We carried out a review of literature to summarize the existing connections between 25-OH vitamin D and CTD. Read More

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http://dx.doi.org/10.1007/s00011-020-01337-xDOI 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

Coupled Plasma Filtration Adsorption for Treatment of Capillary Leak Syndrome Superimposed to Acute Generalized Exanthematous Pustolosis: A Case Report.

Blood Purif 2020 18;49(3):372-378. Epub 2020 Feb 18.

Nephrology and Dialysis Unit, Department of Internal Medicine, ASUITS, Trieste, Italy.

Coupled plasma filtration adsorption (CPFA) is an extracorporeal supportive therapy based on nonspecific adsorption of pro- and anti-inflammatory mediators combined with continuous renal replacement therapy. The main field of CPFA application is septic shock, and there are limited data about its efficacy in the treatment of other acute conditions characterized by a dysregulation in immune homeostasis. Capillary leak syndrome (CLS) defines a life-threatening condition sustained by hypercytokinemia and characterized by abrupt onset of increased capillary permeability leading to severe generalized edema and hypovolemic shock refractory to fluid administration. Read More

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http://dx.doi.org/10.1159/000503770DOI Listing
February 2020

Radiation-induced undifferentiated pleomorphic sarcoma of the breast.

BMJ Case Rep 2020 Feb 10;13(2). Epub 2020 Feb 10.

General Surgery, Wayne State University School of Medicine, Detroit, Michigan, USA

Breast sarcoma is a rare form of malignancy that arises from connective tissue within the breast, comprising less than 5% of all sarcomas. They develop as primary tumours or as secondary following radiation therapy. Diagnosis can be challenging as breast sarcomas are often asymptomatic and resemble benign breast tissue changes. Read More

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http://dx.doi.org/10.1136/bcr-2019-232616DOI Listing
February 2020

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

A case of combined central and peripheral demyelination in Zambia.

Mult Scler Relat Disord 2020 May 14;40:101943. Epub 2020 Jan 14.

Department of Internal Medicine, University of Zambia School of Medicine, Lusaka, Zambia; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. Electronic address:

Overlap syndromes, where otherwise distinct autoimmune processes of the central and peripheral nervous systems are present in the same patient, are uncommon and have not been previously reported in sub-Saharan Africa. We present a case of a 32-year-old man who was found to have both clinically isolated syndrome and chronic inflammatory demyelinating polyneuropathy, highlighting the importance of continued efforts to establish the prevalence of demyelinating disease in the region given the limited treatment options currently available for autoimmune disease. Read More

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http://dx.doi.org/10.1016/j.msard.2020.101943DOI Listing

A multicentre study of 244 pregnancies in undifferentiated connective tissue disease: maternal/fetal outcomes and disease evolution.

Rheumatology (Oxford) 2020 Jan 14. Epub 2020 Jan 14.

Center of Research of Immunopathology and Rare Diseases - Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, and SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy.

Objectives: To investigate fetal/perinatal and maternal outcomes from a large multicentre cohort of women diagnosed with UCTD.

Methods: This multicentre retrospective cohort study describes the outcomes of 224 pregnancies in 133 consecutive women with a diagnosis of UCTD, positive for ANA and aged <45 years old at study inclusion.

Results: Of the 224 pregnancies analysed, 177 (79%) resulted in live births, 45 (20. Read More

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http://dx.doi.org/10.1093/rheumatology/kez620DOI Listing
January 2020

Hydroxychloroquine-induced renal phospholipidosis resembling Fabry disease in undifferentiated connective tissue disease: A case report.

World J Clin Cases 2019 Dec;7(24):4377-4383

Nephrology Department, Tungwah Hospital of Sun Yat-Sen University, Dongguan 523000, Guangdong Province, China.

Background: Fabry disease is a kind of lysosomal storage disease resulting from deficient activity of the lysosomal hydrolase alpha-galactosidase A (GLA). A mutation in the gene leads to a loss of activity of alpha-galactosidase A. Some drugs, such as hydroxychloroquine, can cause pathological changes similar to those usually seen in Fabry disease. Read More

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http://dx.doi.org/10.12998/wjcc.v7.i24.4377DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6940343PMC
December 2019

Mesothelial/monocytic incidental cardiac excrescence in autoimmune disease.

J Card Surg 2020 Mar 30;35(3):679-682. Epub 2019 Dec 30.

Department of Cardiac Surgery, University of Verona, Verona, Italy.

Mesothelial/monocytic incidental cardiac excrescence (MICE) is a rare benign finding made of mesothelial cells, histiocytes, and fibrin, usually found during heart valve surgery. The clinical relevance resides in the potential misdiagnosis as metastatic carcinoma or arterial embolism. The pathogenesis remains uncertain, with artifactual and reactive hypotheses. Read More

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http://dx.doi.org/10.1111/jocs.14416DOI Listing
March 2020
0.888 Impact Factor

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

Multicentric reticulohistiocytosis in the course of undifferentiated connective tissue disease.

Postepy Dermatol Alergol 2019 Oct 12;36(5):646-647. Epub 2019 Nov 12.

Department of Rheumatology and Internal Diseases, Poznan University of Medical Sciences, Poznan, Poland.

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http://dx.doi.org/10.5114/ada.2019.83500DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6906957PMC
October 2019

[A report of clinical characteristics of 2 Chinese pedigrees with haploinsufficiency of A20 and literature review].

Zhonghua Er Ke Za Zhi 2019 Dec;57(12):922-927

Department of Pediatrics, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.

To summarize the clinical characteristics of patients with haploinsufficiency of A20 (HA20). The clinical manifestations, laboratory examinations, treatment, outcome and genetic analysis of 4 cases with HA20 hospitalized in Peking Union Medical College Hospital were analysed.Further literature review was done after searching articles in PubMed and Wangfang databases with the key words "HA20" "A20 haploinsufficiency" "TNFAIP3" up to the date of September 2019. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2019.12.006DOI Listing
December 2019

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

Fatigue severity in anti-nuclear antibody-positive individuals does not correlate with pro-inflammatory cytokine levels or predict imminent progression to symptomatic disease.

Arthritis Res Ther 2019 11 4;21(1):223. Epub 2019 Nov 4.

Division of Genetics and Development, Krembil Research Institute, University Health Network, 5KD402, 60 Leonard Avenue, Toronto, ON, M5T 2S8, Canada.

Background: Fatigue is a common symptom of systemic autoimmune rheumatic disease (SARD). Patients with SARD have a protracted pre-clinical phase during which progressive immunologic derangements occur culminating in disease. In this study, we sought to determine when fatigue develops and whether its presence correlates with inflammatory factors or predicts disease progression. Read More

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http://dx.doi.org/10.1186/s13075-019-2013-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6827224PMC
November 2019
1 Read

Interstitial Pneumonia With Autoimmune Features (IPAF).

Front Med (Lausanne) 2019 27;6:209. Epub 2019 Sep 27.

Department of Respiratory Medicine, National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Lyon, France.

A significant proportion of patients with interstitial lung disease (ILD) manifest autoimmune features, but do not fulfill the diagnostic criteria for a definite connective tissue disease (CTD). In 2015, the European Respiratory Society (ERS) and American Thoracic Society (ATS) "Task Force on undifferentiated Forms of connective tissue disease-associated interstitial lung disease" proposed classification criteria for a so-called research category of Interstitial Pneumonia with Autoimmune Features (IPAF). These classification criteria were based on a combination of features from three domains: a clinical domain consisting of extra-thoracic features; a serologic domain with specific autoantibodies; and a morphologic domain with imaging patterns, histopathological findings or multi-compartment involvement. Read More

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http://dx.doi.org/10.3389/fmed.2019.00209DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6798044PMC
September 2019
1 Read

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
1 Read

Economic Analysis of the Use of Anti-DFS70 Antibody Test in Patients with Undifferentiated Systemic Autoimmune Disease Symptoms.

J Rheumatol 2020 Aug 1;47(8):1275-1284. Epub 2019 Nov 1.

From the Department of Autoimmune Diseases, the Department of Immunology, and the Assessment of Innovations and New Technologies Unit, Hospital Clínic, University of Barcelona, Barcelona; Fundació Clínic per la Recerca Biomèdica, Barcelona; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain; the Unit of Immunology, Rheumatology, Allergy and Rare Diseases, San Raffaele Scientific Institute, Milan, Italy.

Objective: In patients with antinuclear antibodies (ANA) and undifferentiated features of systemic autoimmune disease, the coexistence of monospecific anti-dense fine speckled 70 (anti-DFS70) antibodies is associated with a lower risk of progression to overt disease. Therefore, they might help in correctly classifying ANA- positive patients and avoiding unnecessary followup diagnostic procedures. The aim of this study was to analyze the economic effect of the introduction of the anti-DFS70 antibody test in a hospital setting. Read More

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http://dx.doi.org/10.3899/jrheum.190533DOI Listing

Role of hypoxia in skeletal muscle fibrosis: Synergism between hypoxia and TGF-β signaling upregulates CCN2/CTGF expression specifically in muscle fibers.

Matrix Biol 2020 05 24;87:48-65. Epub 2019 Oct 24.

Centro de Envejecimiento y Regeneración, CARE Chile UC, Santiago, Chile; Departamento de Biología Celular y Molecular, Facultad de Ciencias Biológicas, Pontificia Universidad Católica de Chile, Santiago, Chile. Electronic address:

Several skeletal muscle diseases are characterized by fibrosis, the excessive accumulation of extracellular matrix. Transforming growth factor-β (TGF-β) and connective tissue growth factor (CCN2/CTGF) are two profibrotic factors augmented in fibrotic skeletal muscle, together with signs of reduced vasculature that implies a decrease in oxygen supply. We observed that fibrotic muscles are characterized by the presence of positive nuclei for hypoxia-inducible factor-1α (HIF-1α), a key mediator of the hypoxia response. Read More

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http://dx.doi.org/10.1016/j.matbio.2019.09.003DOI Listing
May 2020
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5.074 Impact Factor

[Venous wall weackness pathogenesis in varicose vein disease].

Khirurgiia (Mosk) 2019 (10):69-74

Sechenov First Moscow State Medical University, Ministry of Health of the Russia, Moscow, Russia.

Varicose veins of lower extremities, varicocele and varicose dilation of pelvic veins including ovaricovaricocele are the most common non-inflammatory diseases of venous system. The same mechanisms determined varicose dilatation regardless localization of the vein. First of all, these are wall weakness and valvular insufficiency combined with hereditary collagen fiber defectiveness in case of undifferentiated connective tissue syndrome. Read More

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http://www.mediasphera.ru/issues/khirurgiya-zhurnal-im-n-i-p
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http://dx.doi.org/10.17116/hirurgia201910169DOI Listing
October 2019
6 Reads

[Clinical value of myositis antibodies in patients with connective tissue disease-associated interstitial lung diseases].

Zhonghua Jie He He Hu Xi Za Zhi 2019 Oct;42(10):765-770

Department of Respiratory Medicine, Drum Tower, Clinical Medical College of Nanjing Medical University, Nanjing 210008, China.

To investigate the clinical significance of detection of myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs) in patients with connective tissue disease-associated interstitial lung diseases (CTD-ILD). Serum samples of 120 patients with CTD-ILD admitted to the Department of Respiratory, Affiliated Drum Tower Hospital of Nanjing University Medical College from December 2016 to April 2018 were collected for analysis. The patients included 45 with polymyositis/dermatomyositis (PM/DM), 36 with Sjogren's syndrome (SS) and 39 with undifferentiated connective tissue disease (UCTD). Read More

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http://dx.doi.org/10.3760/cma.j.issn.1001-0939.2019.10.010DOI Listing
October 2019
2 Reads

Pregnancy and undifferentiated connective tissue disease: outcome and risk of flare in 100 pregnancies.

Rheumatology (Oxford) 2020 Jun;59(6):1335-1339

Rheumatology Unit, Department of Clinical and Experimental Medicine, Pisa.

Objective: UCTD is a systemic autoimmune condition that fails to fulfil the criteria for a definite CTD. Given that there are a lack of studies on links between pregnancy and UCTD, the purpose of this study was to evaluate the risk of disease flares or development of CTD in addition to the risk of adverse pregnancy outcomes in patients with UCTD.

Methods: This is a retrospective study using prospectively collected data for 100 pregnancies in 81 incidences of UCTD treated in a single referral centre. Read More

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http://dx.doi.org/10.1093/rheumatology/kez440DOI Listing
June 2020
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Progranulin autoantibodies in systemic sclerosis and autoimmune connective tissue disorders: A preliminary study.

Immun Inflamm Dis 2019 12 10;7(4):271-275. Epub 2019 Sep 10.

Saarland University Medical School, José Carreras Center for Immuno- and Gene Therapy and Internal Medicine I, Homburg/Saar, Germany.

Objective: The present study aimed to investigate progranulin autoantibodies in systemic sclerosis and autoimmune connective tissue disorders. Progranulin is a physiologic tumor necrosis factor (TNF) antagonist. Progranulin antibodies decrease progranulin levels. Read More

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http://dx.doi.org/10.1002/iid3.270DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6842819PMC
December 2019
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HLA-DRB1 GENE POLYMORPHISMS IN PEDIATRIC PATIENTS WITH TYPE 1 AUTOIMMUNE HEPATITIS AND TYPE 1 AUTOIMMUNE HEPATITIS OVERLAP SYNDROME WITH AUTOIMMUNE CHOLANGITIS.

Arq Gastroenterol 2019 Aug 13;56(2):146-150. Epub 2019 Aug 13.

Universidade Federal de Minas Gerais, Hospital das Clínicas, Serviço de Gastroenterologia Pediátrica, Belo Horizonte, MG, Brasil.

Background: Autoimmune hepatitis (AIH) is a rare chronic inflammatory liver disease associated with a loss of immunological tolerance to self-antigens. Susceptibility to AIH is partially determined by the presence of genes related to human leukocyte antigen (HLA), mainly allelic variants of DRB1.

Objective: The purpose of this study was to investigate the frequencies of the polymorphisms in HLA-DRB1 gene in children and adolescents with type 1 AIH and type 1 AIH overlap syndrome with autoimmune cholangitis (overlap syndrome, OS) in comparison to healthy sex and age-matched individuals (control group). Read More

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http://dx.doi.org/10.1590/S0004-2803.201900000-29DOI Listing
August 2019
4 Reads

Nailfold capillaroscopy and autoimmune connective tissue diseases in patients from a Portuguese nailfold capillaroscopy clinic.

Rheumatol Int 2020 Feb 26;40(2):295-301. Epub 2019 Aug 26.

Internal Medicine Department 7.2, Hospital Curry Cabral-Centro Hospitalar Universitário Lisboa Central, Rua da Beneficência, 8, 1069-166, Lisbon, Portugal.

Raynaud's phenomenon (RP) is frequent in autoimmune connective tissue diseases (AICTD) and its approach includes nailfold capillaroscopy (NFC), as it is a non-invasive technique that permits direct visualization of the microcirculation. The aim of this study is to analyze and establish clinical correlations between NFC findings and particular aspects of autoimmune disorders. This is a retrospective study. Read More

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http://dx.doi.org/10.1007/s00296-019-04427-0DOI Listing
February 2020
3 Reads

Identification of a Shared Microbiomic and Metabolomic Profile in Systemic Autoimmune Diseases.

J Clin Med 2019 Aug 23;8(9). Epub 2019 Aug 23.

Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, 20122 Milan, Italy.

Dysbiosis has been described in systemic autoimmune diseases (SADs), including systemic lupus erythematosus (SLE), Sjögren's syndrome (SjS), and primary anti-phosholipid syndrome (PAPS), however the biological implications of these associations are often elusive. Stool and plasma samples from 114 subjects, including in SLE ( = 27), SjS ( = 23), PAPs ( = 11) and undifferentiated connective tissue (UCTD, = 26) patients, and geographically-matched healthy controls (HCs, = 27), were collected for microbiome (16s rRNA gene sequencing) and metabolome (high-performance liquid chromatography coupled to mass spectrometry) analysis to identify shared characteristics across diseases. Out of 130 identified microbial genera, a subset of 29 bacteria was able to differentiate study groups (area under receiver operating characteristics (AUROC) = 0. Read More

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http://dx.doi.org/10.3390/jcm8091291DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6780636PMC
August 2019
2 Reads

Drug reaction with eosinophilia and systemic symptoms: Pediatric case series and literature review.

Pediatr Dermatol 2019 Nov 18;36(6):887-892. Epub 2019 Aug 18.

Department of Dermatology, Indiana University School of Medicine, Indianapolis, IN, USA.

Background: Pediatric Drug reaction with eosinophilia and systemic symptoms (DRESS) is an uncommon disease that can be difficult to diagnose. This case series and literature review highlights the clinical features of pediatric DRESS and underscores the differential diagnoses, culprit medications, and need for clinical follow-up to detect associated autoimmune sequelae.

Objective: To describe the clinical and laboratory features of pediatric DRESS, identify associated culprit medications, and discuss the natural history of disease. Read More

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http://dx.doi.org/10.1111/pde.13949DOI Listing
November 2019
5 Reads

Investigation of anti-DFS70 antibody in patients with systemic autoimmune rheumatic diseases.

Clin Rheumatol 2019 Dec 8;38(12):3627-3633. Epub 2019 Aug 8.

Department of Medical Microbiology, Izmir Katip Çelebi University Medical Faculty, Izmir, Turkey.

Introduction: Dense fine-speckled 70 (DFS70) antibody is defined as an antinuclear antibody (ANA) pattern in indirect immunofluorescence (IIF). The presence of anti-DFS70 antibody has been shown as a potential marker for the exclusion of systemic autoimmune rheumatic diseases (SARD) (without any other SARD-associated autoantibodies). We aimed to investigate the frequency of anti-DFS70 antibodies in patients with SARD and in the blood bank donors (BD). Read More

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http://link.springer.com/10.1007/s10067-019-04730-y
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http://dx.doi.org/10.1007/s10067-019-04730-yDOI Listing
December 2019
11 Reads

Interstitial Pneumonia with Autoimmune Features.

Authors:
Aryeh Fischer

Clin Chest Med 2019 09;40(3):609-616

Divisions of Rheumatology, Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Medical Campus, University of Colorado School of Medicine, 12631 East 17th Avenue, Academic Office Building One, Aurora, CO 80045, USA. Electronic address:

The European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease put forth the research classification interstitial pneumonia with autoimmune features as a step toward uniformly describing these patients. Diverse nomenclature and classification schemes had been proposed to characterize them. This classification has provided uniform nomenclature and criteria, fostering interdisciplinary engagement and research. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S02725231193003
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http://dx.doi.org/10.1016/j.ccm.2019.05.007DOI Listing
September 2019
4 Reads

Rhabdomyolysis-induced acute kidney injury in a patient with undifferentiated connective tissue disease: A case report and literature review rhabdomyolysis-induced AKI in a patient with UCTD.

Medicine (Baltimore) 2019 Jul;98(30):e16492

Division of Nephrology, Kidney Research Institute, West China Hospital of Sichuan University, Chengdu, China.

Rationale: Acute kidney injury (AKI) accounts for 8% to 16% of hospital admissions and can quadruple hospital mortality, placing a serious burden on the health economy. Acute kidney injury (AKI) is mainly caused by dehydration, shock, infection, sepsis, heart disease, or as a side-effect of nephrotoxic drugs. About 10% to 60% of patients with rhabdomyolysis develop AKI, and 10% of AKI is attributable to rhabdomyolysis. Read More

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http://dx.doi.org/10.1097/MD.0000000000016492DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6709088PMC
July 2019
6 Reads

An atypical presentation of subacute cutaneous lupus erythematous.

Rheumatology (Oxford) 2019 12;58(12):2142

Department of Rheumatology, Royal Lancaster Infirmary, University Hospitals of Morecambe Bay NHS Foundation Trust, Lancaster, UK.

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http://dx.doi.org/10.1093/rheumatology/kez144DOI Listing
December 2019
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Only monospecific anti-DFS70 antibodies aid in the exclusion of antinuclear antibody associated rheumatic diseases: an Italian experience.

Clin Chem Lab Med 2019 Oct;57(11):1764-1769

SOS Laboratorio Immunologia e Allegologia Ospedale S. Giovanni di Dio Firenze, Florence, Italy.

Background The dense fine speckled (DFS) is one of the most common patterns that can be observed as a result of the anti-nuclear antibodies (ANA) test on HEp-2 cells and is mostly caused by antibodies to DFS70 as the main antigenic target. As was recently demonstrated, isolated anti-DFS70 positivity can be used as an aid in the exclusion of ANA associated rheumatic diseases (AARD) due to the opportunity to better interpret unexplained positive IIF ANA results. Methods Our study included 333 subjects with AARD, 51 undifferentiated connective tissue disease (UCTD) patients, 235 disease controls and 149 healthy blood donors from an Italian cohort. Read More

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http://dx.doi.org/10.1515/cclm-2019-0454DOI Listing
October 2019
4 Reads

Anti-centrosome antibodies: Prevalence and disease association in Chinese population.

Scand J Immunol 2019 Oct 23;90(4):e12803. Epub 2019 Jul 23.

Department of Laboratory Medicine, National Clinical Research Center for Geriatrics, West China Hospital, Sichuan University, Chengdu, China.

Anti-centrosome antibodies are rare findings with undefined clinical significance in clinical research. We aimed at investigating the prevalence and clinical significance of anti-centrosome antibodies in Chinese population. Testing results of total of 281,230 ANA-positive sera were retrospectively obtained from West China Hospital Sichuan University in China between 2008 and 2017. Read More

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http://dx.doi.org/10.1111/sji.12803DOI Listing
October 2019
11 Reads