572 results match your criteria Undifferentiated Connective-Tissue Disease


Undifferentiated connective tissue disease: state of the art on clinical practice guidelines.

RMD Open 2018 26;4(Suppl 1):e000786. Epub 2019 Feb 26.

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

The term 'undifferentiated connective tissue disease' (UCTD) is generally used to describe clinical entities characterised by clinical and serological manifestations of systemic autoimmune diseases but not fulfilling the criteria for defined connective tissue diseases (CTDs). In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the ERN ReCONNET project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. No specific CPG on UCTD were found, potential areas of intervention are absence of a consensus definition of UCTD, need for specific monitoring and therapeutic protocols, stratification of UCTD based on the risk of developing a defined CTD and preventive measure for the future development of a more severe condition. Read More

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http://dx.doi.org/10.1136/rmdopen-2018-000786DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6397427PMC
February 2019

Role of lectin pathway complement proteins and genetic variants in organ damage and disease severity of systemic sclerosis: a cross-sectional study.

Arthritis Res Ther 2019 Mar 18;21(1):76. Epub 2019 Mar 18.

Department of Rheumatology, University Hospital Basel, 4031, Basel, Switzerland.

Background: The role of the complement system in the pathogenesis of systemic sclerosis (SSc) is controversial. This study investigated the role of the lectin pathway of complement as a mediator of ischemia/reperfusion injury in SSc.

Methods: This is a prospective observational cross-sectional study of 211 SSc patients and 29 patients with Raynaud's phenomenon in undifferentiated connective tissue disease (UCTD) at risk of developing SSc from two outpatient clinics. Read More

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http://dx.doi.org/10.1186/s13075-019-1859-1DOI Listing

Interstitial Pneumonia with Autoimmune Features.

Ann Am Thorac Soc 2019 Jan 29. Epub 2019 Jan 29.

University of Colorado Denver School of Medicine, 12225, Medicine, Aurora, Colorado, United States ;

"Interstitial pneumonia with autoimmune features" (IPAF) is a research classification proposed by the European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease as an initial step to uniformly define, identify and study patients with interstitial lung disease (ILD) that have features of autoimmunity, yet fall short of a characterizable connective tissue disease. Since its publication in July 2015, there has been substantial interest in IPAF. Centers from around the world have published their findings of retrospectively identified cohorts of patients that fulfill IPAF criteria, suggestions for modification of the criteria have been offered and patients that fulfill IPAF criteria are being included as a subset in the ongoing phase II multicenter unclassifiable ILD treatment trial with pirfenidone (clinicaltrials. Read More

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http://dx.doi.org/10.1513/AnnalsATS.201808-565CMEDOI Listing
January 2019
2 Reads

Mechanic hands: clinical and capillaroscopy manifestations of patients with connective tissue diseases presented with and without mechanic hands.

Clin Rheumatol 2019 Jan 12. Epub 2019 Jan 12.

Division of Rheumatology, Department of Internal Medicine, Shiraz University of Medical Sciences, Shiraz, Fars, Iran.

Objectives: The condition known as 'Mechanic's Hands' is a thickened, hyperkeratotic eruption, which is bilaterally symmetric along the fingers, and often occurs in patients with some connective tissue diseases. Nail fold capillaroscopy is a non-invasive technique for evaluation of connective tissue diseases. We evaluated the prevalence of mechanic hands in patients with connective tissue diseases and compared the clinical manifestations and capillaroscopic changes in the patients with and without mechanic hands. Read More

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http://dx.doi.org/10.1007/s10067-018-04422-zDOI Listing
January 2019
2 Reads

Study of Anti-Malarials in Incomplete Lupus Erythematosus (SMILE): study protocol for a randomized controlled trial.

Trials 2018 Dec 20;19(1):694. Epub 2018 Dec 20.

University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390, USA.

Background: Onset of systemic lupus erythematosus (SLE) is preceded by a preclinical phase characterized by expression of autoantibodies and nonspecific clinical symptoms. Hydroxychloroquine is a treatment for lupus that is widely used based on longstanding experience and a very good safety profile. Existing data suggest that treatment with hydroxychloroquine may postpone the onset of disease. Read More

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http://dx.doi.org/10.1186/s13063-018-3076-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6302430PMC
December 2018
3 Reads

[Systemic lupus erythematosus with acquired hemophilia A: a case report].

Authors:
F Yang Y S Zhou Y Jia

Beijing Da Xue Xue Bao Yi Xue Ban 2018 Dec;50(6):1108-1111

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

Acquired hemophilia A (AHA) is anunusual disease resulting from autoantibodies (inhibitors) against coagulation factor VIII (FVIII) and clinically manifests as bleeding, which sometimes can cause potentially limb-threatening or life-threatening situations. AHA is associated with cancers, auto-immune disorders, infections, dermatologic conditions and certain medications, among which it is commonly secondary to multiple rheumatologic conditions, such as rheumatoid arthritis, systemic lupus erythematosus (SLE), pollymyositis, autoimmune hemolytic anemia and undifferentiated connective tissue disease. In autoimmune diseases, it may be the result of autoantibody producing against FVIII, and some cases of AHA may act as the first manifestation of SLE. Read More

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December 2018
17 Reads

Acquired renal glucosuria in an undifferentiated connective tissue disease patient with a SLC5A2 heterozygous mutation: A case report.

Medicine (Baltimore) 2018 Dec;97(50):e13664

Department of Nephrology, Yueyang hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of TCM, China.

Introduction: Renal glucosuria is a renal tubular disorder caused by genetic conditions, drugs, and poisons. Mutations in the SLC5A2 gene are recently found to be responsible for the inherited renal glucosuria, while undifferentiated connective tissue disease (UCTD) was not considered pathogenic for renal glucosuria. Here, we present a case of acquired renal glucosuria in a UCTD patient. Read More

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http://dx.doi.org/10.1097/MD.0000000000013664DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6319971PMC
December 2018
6 Reads

[Establishment and application of quantitative detection of serum anti-carbamylated protein autoantibodies by microarray chemiluminescence immunoassay].

Xi Bao Yu Fen Zi Mian Yi Xue Za Zhi 2018 Oct;34(10):924-930

Department of Laboratory Diagnosis, Changhai Hospital, Naval Military Medical University, Shanghai 200433, China. *Corresponding authors, E-mail:

Objective To develope a chemiluminescence immunoassay based on microarray protein chip technology for detecting the anti-carbamylated protein (CarP) antibody. We aimed to evaluate the detection performance of this method and to explore its preliminary clinical application value of this index in patients with rheumatoid arthritis (RA). Methods A quantitative detection method for anti-CarP antibody was established to evaluate the precision, minimum detection limit, linear range and specificity of the method. Read More

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October 2018
1 Read

The presence of anti-nuclear antibodies alone is associated with changes in B cell activation and T follicular helper cells similar to those in systemic autoimmune rheumatic disease.

Arthritis Res Ther 2018 Nov 29;20(1):264. Epub 2018 Nov 29.

Krembil Research Institute, University Health Network, 60 Leonard Avenue, 5KD402, Toronto, ON, M5T 2S8, Canada.

Background: Diagnosis of systemic autoimmune rheumatic diseases (SARD) relies on the presence of hallmark anti-nuclear antibodies (ANA), many of which can be detected years before clinical manifestations. However, ANAs are also seen in healthy individuals, most of whom will not develop SARD. Here, we examined a unique cohort of asymptomatic ANA individuals to determine whether they share any of the cellular immunologic features seen in SARD. Read More

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http://dx.doi.org/10.1186/s13075-018-1752-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6263058PMC
November 2018
3 Reads

Dystrophic calcinosis in a patient with overlap syndrome (scleroderma and rheumatoid arthritis) treated by leflunomide: A case report.

Medicine (Baltimore) 2018 Nov;97(47):e13319

Rationale: Dystrophic calcinosis occurs in chronically damaged tissue in patients with complicated autoimmune diseases. The therapeutic options are limited, and the treatment response rate is variable. Here, we describe a rare case of dystrophic calcinosis treated with leflunomide in a patient with overlap syndrome. Read More

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http://dx.doi.org/10.1097/MD.0000000000013319DOI Listing
November 2018
11 Reads

Variant syndromes of primary biliary cholangitis.

Best Pract Res Clin Gastroenterol 2018 Jun - Aug;34-35:55-61. Epub 2018 Jun 14.

I. Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Patients with primary biliary cholangitis (PBC) can show biochemical, serological and/or histological features of autoimmune hepatitis (AIH). The term 'AIH-PBC overlap syndrome' has been used frequently for these cases and implies the coexistence of two separate diseases. However, the boundaries between 'classical' PBC, PBC with features of AIH and 'classical' AIH are difficult to define, and therefore the term 'variant syndrome' should be preferred. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15216918183002
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http://dx.doi.org/10.1016/j.bpg.2018.06.003DOI Listing
December 2018
22 Reads

"Three in One" - Polyautoimmunity with Multiple Autoimmune Syndrome.

J Assoc Physicians India 2018 01;66(1):95-7

senior Consultant In Radiodiagnosis, Malviya Nagar Clinic and Sonography Centre, Malviya Nagar, Jaipur, Rajasthan.

Autoimmune disease (AD) may well start off as a single diagnosis and over the years develop into polyautoimmunity and even multiple autoimmune syndromes (MAS) seen in the same patient, as new clinical symptoms and laboratory finding show up in the course of disease. We present a case of MAS who was initially diagnosed to have autoimmune thyroid disease (AITD) - hypothyroidism. She was then evaluated for persistent mild to moderate iron deficiency anemia, unintentional weight loss along with skin rash and diagnosed to have celiac disease and undifferentiated connective tissue disease (uCTD). Read More

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January 2018
6 Reads

Effects of Antioxidant Tempol on Systematic Inflammation and Endothelial Apoptosis in Emphysematous Rats Exposed to Intermittent Hypoxia.

Yonsei Med J 2018 Nov;59(9):1079-1087

Respiratory Department of Tianjin Medical University General Hospital, Tianjin, China.

Purpose: Obstructive sleep apnea and chronic obstructive pulmonary disease are independent risk factors of cardiovascular disease (CVD), and their coexistence is known as overlap syndrome (OS). Endothelial dysfunction is the initial stage of CVD; however, underlying mechanisms linking OS and CVD are not well understood. The aim of this study was to explore whether OS can lead to more severe inflammation and endothelial apoptosis by promoting endothelial dysfunction, and to assess the intervention effects of antioxidant tempol. Read More

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https://synapse.koreamed.org/DOIx.php?id=10.3349/ymj.2018.59
Publisher Site
http://dx.doi.org/10.3349/ymj.2018.59.9.1079DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6192890PMC
November 2018
5 Reads
1.263 Impact Factor

[Prospective comparative study of remote results of surgical treatment of patients with varicothrombophlebitis].

Angiol Sosud Khir 2018;24(3):77-84

Saratov State Medical University named after V.I. Razumovsky under the Ministry of Public Health of the Russian Federation, Saratov, Russia.

The authors examined the effect of the presence of undifferentiated connective tissue dysplasia (UCTD) on the clinical course of lower-limb varicose veins (LLVV) and acute varicothrombophlebitis (AVTP) in patients after the Troyanov-Trendelenburg operation. We carried out a prospective comparative study of the remote results during three years after the Troyanov-Trendelenburg operation in a total of 132 patients suffering from AVTP. The Study Group included 67 patients found to have more than four phenotypic traits of UCTD, thus suggesting the presence of UCTD. Read More

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December 2018
2 Reads

Chronic Fatigue Syndrome: From Chronic Fatigue to More Specific Syndromes.

Eur Neurol 2018 4;80(1-2):73-77. Epub 2018 Oct 4.

Department of Medicine, Brown Medical School, Providence, Rhode Island, USA.

In the last decade, a group of chronic disorders associated with fatigue (CDAF) emerged as the leading cause of chronic fatigue, chronic pain, and functional impairment, all of which have been often labeled in clinical practice as chronic fatigue syndrome (CFS) or fibromyalgia. While these chronic disorders arise from various pathophysiologic mechanisms, a shared autoimmune or immune-mediated etiology could shift the focus from symptomatic treatment of fatigue and pain to targeted immunomodulatory and biological therapy. A clinical paradigm shift is necessary to reevaluate CFS and fibromyalgia diagnoses and its relationship to the CDAF entities, which would ultimately lead to a change in diagnostic and therapeutic algorithm for patients with chronic fatigue and chronic pain. Read More

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https://www.karger.com/Article/FullText/493531
Publisher Site
http://dx.doi.org/10.1159/000493531DOI Listing
January 2019
18 Reads

Non-specific interstitial pneumonia and features of connective tissue disease: What are the consequences of a different point of view?

Monaldi Arch Chest Dis 2018 09 4;88(3):970. Epub 2018 Sep 4.

University of Milan Bicocca, Dipartimento Cardio-Toraco-Vascolare.

Patients with Interstitial Lung Disease (ILD) without a definitive diagnosis of connective tissue diseases (CTD) were historically described as Undifferentiated Connective Tissue Disease (UCTD-ILD). Recently a new classification, Interstitial Pneumonia with Autoimmune Features (IPAF), has been proposed. Aim of this study was to describe the prevalence, clinical characteristics and prognostic factors of UCTD and IPAF subjects in a cohort of Non-Specific Interstitial Pneumonia (NSIP) patients. Read More

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http://dx.doi.org/10.4081/monaldi.2018.970DOI Listing
September 2018
7 Reads

Uncommon patterns of antinuclear antibodies recognizing mitotic spindle apparatus antigens and clinical associations.

Medicine (Baltimore) 2018 Aug;97(34):e11727

Dinámica IPS, Medellín, Colombia.

Antinuclear antibodies (ANA) are key biomarkers in the evaluation of rheumatic diseases. The prevalence and clinical significance of uncommon or rare patterns, particularly those directed at the mitotic spindle apparatus (MSA), are not well understood. We aimed to investigate the prevalence and clinical significance of anti-MSA patterns in a Colombian population. Read More

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http://dx.doi.org/10.1097/MD.0000000000011727DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6112870PMC
August 2018
9 Reads

Morphologic Aspects of Interstitial Pneumonia With Autoimmune Features.

Arch Pathol Lab Med 2018 Sep;142(9):1080-1089

Departamento de Patologia, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil (Dr do Nascimento); Divisao de Pneumologia, Instituto do Coracao, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil (Dr Baldi); Departamento de Radiologia, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil (Dr Sawamura); and Departamento de Patologia, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil (Dr Dolhnikoff).

Context: - Interstitial lung disease, a common complication observed in several connective tissue diseases, causes significant morbidity and mortality. Similar to individuals with connective tissue diseases, a significant subgroup of patients with clinical and serologic characteristics suggestive of autoimmunity but without confirmed specific connective tissue disease presents with associated interstitial lung disease. These patients have been classified using different controversial nomenclatures, such as undifferentiated connective tissue disease-associated interstitial lung disease, lung-dominant connective tissue disease, and autoimmune featured interstitial lung disease. Read More

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http://www.archivesofpathology.org/doi/10.5858/arpa.2017-052
Publisher Site
http://dx.doi.org/10.5858/arpa.2017-0528-RADOI Listing
September 2018
8 Reads

[Modified method for determining the normal values of the aortic root diameter in the focus of aortic pathology in individuals with congenital connective tissue disorders].

Kardiologiia 2018 SJul;58(S7):11-18

State Budgetary Educational Institution of Higher Professional Education, "Omsk State Medical University" of the RF Ministry of Health Care.

Introduction: Enlargement of the aortic root of occurs in many cases with cardiovascular disease, including congenital connective tissue disorders (CCTD), especially its differentiated and undifferentiated forms (UCCTD). A common deficiency of the generally accepted methods for diagnosing an aortic root dilation may be that persons with CCTD and cardiovascular diseases of potentially having a broader aortic root diameter could be included in the reference groups. The purpose of our study was to develop a modified method for determining the normal aortic root diameter in group of individuals without CCTD and cardiovascular diseases. Read More

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January 2019
3 Reads

Interstitial Pneumonia With Autoimmune Features: An Emerging Challenge at the Intersection of Rheumatology and Pulmonology.

Arthritis Rheumatol 2018 Dec 27;70(12):1901-1913. Epub 2018 Oct 27.

Vanderbilt University, Nashville, Tennessee.

Interstitial lung disease (ILD) remains a cause of significant morbidity and mortality in patients with connective tissue disease (CTD)-associated ILD. While some patients meet clear classification criteria for a systemic rheumatic disease, a subset of patients do not meet classification criteria but still benefit from immunosuppressive therapy. In 2015, the American Thoracic Society and European Respiratory Society described classification criteria for interstitial pneumonia with autoimmune features (IPAF) to identify patients with lung-predominant CTD who lack sufficient features of a systemic rheumatic disease to meet classification criteria. Read More

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http://dx.doi.org/10.1002/art.40679DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6261671PMC
December 2018
12 Reads

Development of lupus nephritis in a patient with previous scleroderma renal crisis.

Lupus 2018 Sep 20;27(10):1732-1734. Epub 2018 Jul 20.

1 The Ottawa Hospital, Riverside Campus, Department of Medicine, Division of Rheumatology.

We present a case of scleroderma overlap syndrome with systemic lupus erythematosus (SLE) including complications of both scleroderma renal crisis and lupus nephritis. Our patient was initially diagnosed with undifferentiated connective tissue disease in 1996. A diagnosis of scleroderma was made in 2010 after she developed scleroderma renal crisis. Read More

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http://dx.doi.org/10.1177/0961203318785776DOI Listing
September 2018
18 Reads

Case series: rheumatological manifestations attributed to exposure to Libby Asbestiform Amphiboles.

J Toxicol Environ Health A 2018 21;81(15):734-747. Epub 2018 Jun 21.

e Icahn School of Medicine at Mt Sinai , New York NY.

An increased risk for Systemic Autoimmune Diseases (SAID) has been reported in Libby, Montana, where extensive exposures to fibrous amphiboles occurred due to mining and use of asbestos-laden vermiculite. In addition, positive antinuclear autoantibody tests are associated with exposure to Libby Asbestiform Amphiboles (LAA) in both humans and mice. Among 6603 subjects who underwent health screening at the Center for Asbestos Related Diseases (CARD, Libby MT), 13. Read More

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http://dx.doi.org/10.1080/15287394.2018.1485124DOI Listing
June 2018
14 Reads

Systemic sclerosis-rheumatoid arthritis overlap syndrome complicated with Sweet's syndrome.

Clin Rheumatol 2018 Aug 25;37(8):2281-2284. Epub 2018 May 25.

Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shuai Fu Yuan No.1, Dongcheng District, Beijing, 100730, China.

Herein, we report a case of a 34-year-old woman with systemic sclerosis (SSc)-rheumatoid arthritis (RA) overlap syndrome (OS) complicated with Sweet's syndrome. OS has been defined as entities satisfying classification criteria of at least two connective tissue diseases (CTD) occurring at the same or at different times in the same patient. The CTD include RA, SSc, systemic lupus erythematosus (SLE), polymyositis, and dermatomyositis. Read More

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http://dx.doi.org/10.1007/s10067-018-4150-yDOI Listing
August 2018
4 Reads

Interstitial lung disease in patients with mixed connective tissue disease: pilot study on predictors of lung involvement.

Clin Exp Rheumatol 2018 Jul-Aug;36(4):648-651. Epub 2018 May 8.

Division of Rheumatology, Mayo Clinic, Jacksonville, FL, USA.

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. Read More

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September 2018
9 Reads

Early life origins of the Asthma-COPD Overlap Syndrome?

Respirology 2018 08 8;23(8):731-732. Epub 2018 May 8.

Department of Preventive and Social Medicine, Dunedin School of Medicine, University of Otago, Dunedin, New Zealand.

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http://dx.doi.org/10.1111/resp.13323DOI Listing
August 2018
7 Reads

Mid-borderline leprosy masquerading as an overlap syndrome.

Rheumatology (Oxford) 2018 09;57(9):1686-1688

Department of Dermatology, Dr.Vasantrao Pawar Medical College & Hospital & Research Centre, Nashik, India.

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http://dx.doi.org/10.1093/rheumatology/key125DOI Listing
September 2018
5 Reads

Treatment of adult idiopathic inflammatory myopathies with conventional immunosuppressive drugs : Results of a retrospective study.

Z Rheumatol 2019 Mar;78(2):183-189

HELIOS Klinik Sangerhausen, Am Beinschuh 2a, 06526, Sangerhausen, Germany.

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. Read More

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http://dx.doi.org/10.1007/s00393-018-0471-0DOI Listing
March 2019
4 Reads

Clinical features, risk factors, and outcomes of patients with interstitial pneumonia with autoimmune features: a population-based study.

Clin Rheumatol 2018 Aug 18;37(8):2125-2132. Epub 2018 Apr 18.

Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China.

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. Read More

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http://dx.doi.org/10.1007/s10067-018-4111-5DOI Listing
August 2018
7 Reads

[Current view on the pathogenesis of varicocele and the problem of its recurrence].

Urologiia 2018 Mar(1):150-154

I.M. Sechenov First MSMU, Moscow, Russia.

The clinical signs of varicocele typically emerge during the puberty. Varicocele is found in 15% of men in the general population and 25-35% and 50-80% of males presenting with primary and secondary infertility, respectively. Factors contributing to the development and recurrence of varicocele include the abnormalities of the testicular venous drainage and outflow (varicose veins are more common on the left than on the right), the anatomical features of the veins of the testicular and prostatic venous plexus, the patients constitution, predisposition to constipation or diarrhea, physical activity. Read More

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March 2018
4 Reads

Anti-DFS70 autoantibodies in undifferentiated connective tissue diseases subjects: what's on the horizon?

Rheumatology (Oxford) 2018 Apr 3. Epub 2018 Apr 3.

Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Aviv, Israel.

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. Read More

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http://dx.doi.org/10.1093/rheumatology/key012DOI Listing
April 2018
30 Reads

Patients with primary localized high-grade sarcomas of the digestive tract excluding GIST : a retrospective study from the French sarcoma group.

Acta Gastroenterol Belg 2017 Oct-Dec;80(4):481-486

APHM, Hôpital de la Timone, Department of Medical Oncology, Marseille, France.

Introduction : The natural history of localized high-grade sarcomas of the digestive tract (SDT) excluding GIST has been rarely considered owing to their low incidence and heterogeneity. We describe the histoclinical characteristics of SDT and correlate them with patients' outcomes.

Methods: We retrospectively collected medical files from a European database covering connective tissue tumors listed in Europe for about twenty years. Read More

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May 2018
4 Reads

Primary cutaneous amyloidosis associated with autoimmune hepatitis-primary biliary cirrhosis overlap syndrome and Sjögren syndrome: A case report.

Authors:
Xin Yan Jinglan Jin

Medicine (Baltimore) 2018 Feb;97(8):e0004

Department of Hepatology, The First Hospital of Jilin University, Changchun, Jilin, China.

Rationale: Primary cutaneous amyloidosis (PCA) is a localized skin disorder characterized by the abnormal deposition of amyloid in the extracellular matrix of the dermis. The association between PCA and other diseases, although rare, has been documented for various autoimmune diseases. PCA associated with autoimmune hepatitis-primary biliary cirrhosis (AIH-PBC) overlap syndrome and Sjögren syndrome (SS) has not been previously reported in the literature. Read More

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http://dx.doi.org/10.1097/MD.0000000000010004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5842025PMC
February 2018
32 Reads

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.

Clin Exp Rheumatol 2018 Jul-Aug;36(4):589-594. Epub 2018 Jan 31.

Rheumatology, Maastricht University, School for Public Health and Primary Care, Maastricht University Medical Centre, the Netherlands.

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)). Read More

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September 2018
27 Reads

Primary anetoderma with undifferentiated connective tissue disease.

Dermatol Online J 2017 Dec 15;23(12). Epub 2017 Dec 15.

New York University Health, New York.

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. Read More

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December 2017
15 Reads

Primary Biliary Cholangitis- Autoimmune Hepatitis Overlap Syndrome.

Indian J Pediatr 2018 09 9;85(9):803-805. Epub 2018 Feb 9.

Department of Gastroenterology, Seth G S Medical College and KEM Hospital, Multistorey Building, 11th Floor, Parel, Mumbai, 400012, India.

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http://dx.doi.org/10.1007/s12098-018-2628-xDOI Listing
September 2018
16 Reads
0.920 Impact Factor

Podocyte Infolding Glomerulopathy (PIG) in a Patient With Undifferentiated Connective Tissue Disease: A Case Report.

Am J Kidney Dis 2018 Jul 1;72(1):149-153. Epub 2018 Feb 1.

Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India.

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. Read More

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http://dx.doi.org/10.1053/j.ajkd.2017.11.023DOI Listing
July 2018
11 Reads
5.900 Impact Factor

Overlap of interstitial pneumonia with autoimmune features with undifferentiated connective tissue disease and contribution of UIP to mortality.

Respirology 2018 Jun 10;23(6):600-605. Epub 2018 Jan 10.

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

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. Read More

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http://dx.doi.org/10.1111/resp.13254DOI Listing
June 2018
15 Reads

Prevalence of comorbidities in patients with obstructive sleep apnea syndrome, overlap syndrome and obesity hypoventilation syndrome.

Clin Respir J 2018 May 16;12(5):1905-1911. Epub 2018 Jan 16.

Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy.

Introduction: Sleep-disordered breathing causes a burden to the sufferer, the health care system and the society. Most studies have focused on obstructive sleep apnea (OSA); however, the prevalence of comorbidities in patients affected by overlap syndrome (OS) and obesity hypoventilation syndrome (OHS) has not been carefully evaluated.

Study Objectives: The principal aim of this study was to identify the presence of comorbidities in patients suffering from OSA, OS, OHS and the differences in three groups of patients. Read More

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http://dx.doi.org/10.1111/crj.12754DOI Listing
May 2018
17 Reads

[Disseminated tuberculosis in a patient with overlap syndrome, challenges in preventing].

Rev Med Inst Mex Seguro Soc 2017 Sep-Oct;55(5):666-670

Subdirección de Enseñanza e Investigación, Hospital Regional de Alta Especialidad de Oaxaca, San Bartolo Coyotepec, Oaxaca, México

Patients with autoimmune rheumatic diseases are at increased risk for developing infections and these are associated with increased morbidity and mortality from these diseases, especially in patients with systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis and autoimmune inflammatory myopathies. The objective of this paper is to address the challenges in detecting latent tuberculosis in immunosuppressed patients and the initiation of prophylactic treatment because currently there are no well-defined guidelines indicating what action to take for detection and treatment; so far the available scientific evidence is scarce and some methodological shortcomings. Read More

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July 2018
5 Reads

Severe Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome-beyond skin involvement.

Pediatr Dermatol 2018 Jan 21;35(1):e17-e19. Epub 2017 Nov 21.

Centro Hospitalar de Lisboa Central-EPE, Hospital Dona Estefânia, Lisbon, Portugal.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but serious dermatologic diseases with many potential multisystem complications. We describe the case of an 8-year-old girl who developed severe SJS/TEN overlap syndrome (25% of her body surface area was affected) complicated by pancreatitis and bronchiolitis obliterans. These rare complications emphasize the need for careful, intensive monitoring of possible complications and an interdisciplinary team approach to provide optimal treatment and follow-up. Read More

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http://dx.doi.org/10.1111/pde.13328DOI Listing
January 2018
9 Reads

Autoantibodies binding to stathmin-4: new marker for polyneuropathy in primary Sjögren's syndrome.

Immunol Res 2017 12;65(6):1099-1102

Private Practice, Rheumatology, Schönsteinstr. 63, Cologne, Germany.

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http://dx.doi.org/10.1007/s12026-017-8970-7DOI Listing
December 2017
8 Reads

Pulmonary Aspergillus Overlap Syndromes.

Mycopathologia 2018 Apr 24;183(2):431-438. Epub 2017 Oct 24.

Department of Pulmonary Medicine, Shanghai Respiratory Research Institute, Zhongshan Hospital, Fudan University, Shanghai, 200032, China.

Background: Broadly, there are three main categories in pulmonary aspergillosis: chronic forms of aspergillosis; allergic bronchopulmonary aspergillosis; and invasive aspergillosis (IPA). IPA has been further subdivided into angioinvasive and airway-invasive aspergillosis. Aspergillus overlap syndromes is defined as the occurrence of more than one form aspergillus disease in a single individual. Read More

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http://dx.doi.org/10.1007/s11046-017-0212-yDOI Listing
April 2018
5 Reads

Chylous ascites in a patient with an overlap syndrome: a surprising response to rituximab.

BMJ Case Rep 2017 Oct 20;2017. Epub 2017 Oct 20.

Leeds Biomedical Research Centre, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.

The authors present a case of a 51-year-old woman with clinical diagnosis of mixed connective tissue disease and overlap systemic lupus erythematosus features, with a 6-month history of progressive painless abdominal distension. On examination, evident signs of ascites were present. Both the abdominal-pelvic ultrasound and CT scan confirmed a large amount of ascites. Read More

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http://dx.doi.org/10.1136/bcr-2017-222339DOI Listing
October 2017
11 Reads

Cardiac involvement in undifferentiated connective tissue disease at risk for systemic sclerosis (otherwise referred to as very early-early systemic sclerosis): a TDI study.

Clin Exp Med 2018 May 9;18(2):237-243. Epub 2017 Oct 9.

Rheumatology Unit, Department of Internal and Experimental Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy.

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). Thirty patients with UCTD-risk-SSc (28 female, mean age 47 ± 13 years, range 21-70) and 30 age- and sex-matched controls underwent cardiac assessment by standard echocardiography and TDI. Read More

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http://dx.doi.org/10.1007/s10238-017-0477-yDOI Listing
May 2018
24 Reads

Diagnosis of systemic inflammatory diseases among patients admitted for acute pericarditis with pericardial effusion.

J Cardiovasc Med (Hagerstown) 2017 Nov;18(11):875-880

aDépartement de Médecine Interne bDépartement d'Epidémiologie et Recherche Clinique cDépartement d'Information Médicale dDépartement de Cardiologie, Hôpital Bichat, Université Paris Diderot, PRES Sorbonne Paris Cité, Assistance Publique Hôpitaux de Paris eINSERM U1149 fDépartement Hospitalo-Universitaire FIRE (Fibrosis, Inflammation and Remodelling in Renal and Respiratory Diseases), Paris, France.

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. Read More

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http://dx.doi.org/10.2459/JCM.0000000000000576DOI Listing
November 2017
19 Reads

Undifferentiated connective tissue diseases and adverse pregnancy outcomes. An undervalued association?

Am J Reprod Immunol 2017 Dec 16;78(6). Epub 2017 Sep 16.

Division of Rheumatology, University of Pavia, IRCCS Fondazione Policlinico San Matteo, Pavia, Italy.

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. Read More

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http://doi.wiley.com/10.1111/aji.12762
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http://dx.doi.org/10.1111/aji.12762DOI Listing
December 2017
11 Reads

A case of gestational gigantomastia in a 37-years-old woman associated with elevated ANA: a casual linkage?

Pan Afr Med J 2017 4;27:167. Epub 2017 Jul 4.

Department of Plastic and Reconstructive Surgery, Breast Unit, University of Udine, Italy.

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. Read More

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http://www.panafrican-med-journal.com/content/article/27/167
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http://dx.doi.org/10.11604/pamj.2017.27.167.11281DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5579424PMC
September 2017
44 Reads

The Microbiome in Connective Tissue Diseases and Vasculitides: An Updated Narrative Review.

J Immunol Res 2017 1;2017:6836498. Epub 2017 Aug 1.

Department of Rheumatology, ASST Fatebenefratelli-Sacco, Milan, Italy.

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. Read More

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http://dx.doi.org/10.1155/2017/6836498DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5556609PMC
May 2018
15 Reads

European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes.

J Eur Acad Dermatol Venereol 2017 Sep 9;31(9):1401-1424. Epub 2017 Aug 9.

Department of Dermatology and Venereology, University Hospital of Cologne, Cologne, Germany.

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. Read More

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http://dx.doi.org/10.1111/jdv.14458DOI Listing
September 2017
41 Reads

[Lower extremity varicose veins in childhood and at a young age: Mechanism of development and specific features].

Arkh Patol 2017;79(4):56-60

I.M. Sechenov First Moscow State Medical University, Ministry of Health of the Russian Federation, Moscow, Russia.

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. Read More

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http://dx.doi.org/10.17116/patol201779456-60DOI Listing
April 2018
11 Reads