2,680 results match your criteria Mixed Connective-Tissue Disease


Successful treatment of psoriatic arthritis with apremilast in a mixed connective tissue disease patient.

G Ital Dermatol Venereol 2019 Jan 9. Epub 2019 Jan 9.

Department of Clinical Dermatology, San Gallicano Dermatologic Institute IRCCS, Rome, Italy.

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http://dx.doi.org/10.23736/S0392-0488.18.06042-XDOI Listing
January 2019

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

Ectopic expression of Klotho in fibroblast growth factor 23 (FGF23)-producing tumors that cause tumor-induced rickets/osteomalacia (TIO).

Bone Rep 2019 Jun 31;10:100192. Epub 2018 Dec 31.

Fujii Memorial Institute of Medical Sciences, Tokushima University, Tokushima 770-8503, Japan.

Tumor-induced rickets/osteomalacia (TIO) is a rare paraneoplastic syndrome caused by tumors that ectopically express fibroblast growth factor 23 (FGF23). FGF23 is a bone-derived hormone that regulates serum phosphate concentrations. Patients with TIO develop hypophosphatemic rickets/osteomalacia due to FGF23 excess and suffer from symptoms such as leg deformities, bone pain, skeletal muscle myopathy, and multiple fractures/pseudofractures. Read More

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http://dx.doi.org/10.1016/j.bonr.2018.100192DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6321977PMC
June 2019
1 Read

Connective Tissue Disorders in Childhood: Are They All the Same?

Radiographics 2019 Jan-Feb;39(1):229-250

From the Departments of Radiology (M.N., E.J.I.C., M.R.P.) and Rheumatology (E.I., J.A.), Hospital Sant Joan de Déu, Passeig Sant Joan de Déu 2, 08950 Esplugues de Llobregat, Barcelona, Spain; Department of Medical Imaging, University of Toronto, Toronto, ON, Canada (M.N., O.M.N.); and Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto, ON, Canada (M.N., O.M.N.).

Systemic connective tissue disorders are characterized by the presence of autoantibodies and multiorgan system involvement. Juvenile systemic lupus erythematosus with or without associated antiphospholipid syndrome; juvenile dermatomyositis; sclerodermiform syndromes, including systemic and localized sclerodermas and eosinophilic fasciitis; mixed connective tissue disease; and Sjögren syndrome are the disorders that affect children most frequently. Diagnosis is difficult, because the clinical presentation of patients is diverse, from mild to severe disease. Read More

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http://dx.doi.org/10.1148/rg.2019180078DOI Listing
January 2019
1 Read

Bamboo nodes as evidence of mixed connective tissue disease.

Joint Bone Spine 2018 Dec 28. Epub 2018 Dec 28.

Department of Rheumatology, Ramón y Cajal University Hospital. Ctra. Colmenar Viejo, Km 9,100. PC 28034, Madrid. Spain.

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http://dx.doi.org/10.1016/j.jbspin.2018.12.009DOI Listing
December 2018

Analysis of finger vein variety in patients with various diseases using vein authentication technology.

J Biophotonics 2018 Dec 18:e201800354. Epub 2018 Dec 18.

Department of Dermatology and Allergology, Juntendo University Graduate School of Medicine, Tokyo, Japan.

In finger vein authentication technology, near-infrared rays penetrate the finger and are absorbed by the hemoglobin in blood. The veins appear as dark areas. The finger vein pattern images of patients with various diseases were acquired; a new evaluation method applying image processing technique ("E value") was developed, and it was examined whether the patterns have any characteristics differentiating them from those of healthy volunteers. Read More

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http://dx.doi.org/10.1002/jbio.201800354DOI Listing
December 2018
1 Read

Abnormal Nailfold Capillaroscopy Is Common in Patients with Connective Tissue Disease and Associated with Abnormal Pulmonary Function Tests.

J Rheumatol 2018 Dec 15. Epub 2018 Dec 15.

From the Department of Internal Medicine, Division of Vascular Medicine, and the Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen; Department of Rheumatology, Medical Center Leeuwarden, Leeuwarden, the Netherlands. A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; C.C. Huisman, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; A.M. van Roon, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; D. Zhang, MD, Department of Rheumatology, Medical Center Leeuwarden; A.J. Stel, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; A.J. Smit, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; H. Bootsma, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; D.J. Mulder, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen. Address correspondence to A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University Medical Center Groningen, Huispostcode AA41, Hanzeplein 1, Postbus 30 001, Groningen, the Netherlands. E-mail: Accepted for publication September 11, 2018.

Objective: To assess the presence of a systemic sclerosis (SSc) pattern on nailfold capillary microscopy (NCM) in patients with Raynaud phenomenon (RP) and to explore its association with abnormal pulmonary function tests (PFT).

Methods: NCM patterns were assessed in 759 consecutive patients with RP. Patterns were classified as normal (n = 354), nonspecific (n = 159), or SSc pattern (n = 246). Read More

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http://dx.doi.org/10.3899/jrheum.180615DOI Listing
December 2018

Mixed connective tissue disease in young Saudi patient with recurrent dental abscess: A case report.

Int J Health Sci (Qassim) 2018 Nov-Dec;12(6):65-68

Department of Paediatric, Paediatric Rheumatology Division, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.

Mixed connective tissue disease (MCTD) or overlap syndrome is a rare disease. It has overlapping features of more than one autoimmune disease with high titer of anti-ribonucleoprotein antibodies against U1. We present a 12-year-old Saudi male patient who was presented to the dental clinic complaining from non-healing oral ulcers and multiple periapical abscesses that not responsive to extractions and the use of oral antibiotics, these symptoms were accompanied with persistent fever, headache, muscle weakness, general malaise, and painful bilateral cervical lymphadenopathy. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6257872PMC
December 2018
2 Reads

Immunomodulatory properties of mesenchymal stromal cells: still unresolved "Yin and Yang".

Curr Stem Cell Res Ther 2018 Dec 4. Epub 2018 Dec 4.

Division of Immunology, Transplants and Infectious Diseases, San Raffaele Scientific Institute, Milan. Italy.

Mesenchymal stromal cells (MSC) are mesodermal elements characterized by the ability to differentiate into several types of cells present mainly in connective tissues. They play a key function in tissue homeostasis and repair. Furthermore, they exert a strong effect on both innate and adaptive immune response. Read More

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http://dx.doi.org/10.2174/1574888X14666181205115452DOI Listing
December 2018
5 Reads

A Rare Cause of Dysphagia and Weight Loss.

Case Rep Gastroenterol 2018 Sep-Dec;12(3):640-645. Epub 2018 Nov 1.

Department of Gastroenterology, San Antonio Uniformed Services Health Education Consortium, San Antonio, Texas, USA.

Mixed connective tissue disease (MCTD) is a rare connective tissue disorder with clinical features that overlap with systemic lupus erythematous, systemic sclerosis, and polymyositis. We report the case of a patient who presented with dysphagia, 25-lb weight loss, difficulty opening her mouth, and joint pain. Dysphagia workup showed a normal barium swallow and normal-appearing EGD but esophageal manometry consistent with severe dysmotility. Read More

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https://www.karger.com/Article/FullText/493919
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http://dx.doi.org/10.1159/000493919DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6244109PMC
November 2018
6 Reads

Mixed connective tissue disease complicated by heart failure in Ile-Ife, Nigeria: management challenges in a resource-limited economy.

Int Med Case Rep J 2018 2;11:307-312. Epub 2018 Nov 2.

Department of Cardiology,

Background: Mixed connective tissue disease (MCTD; also known as Sharp's syndrome) is a rare autoimmune inflammatory disorder characterized by high titer of U1 ribonucleoprotein (U1RNP) antibody and clinical and serological overlap of systemic lupus erythematosus, systemic sclerosis, and polymyositis. The diagnosis is based on clinical and serological factors in criteria such as Alarcon-Segovia, Khan, Kusakawa, and Sharps. Cardiac disease can be a complication of connective tissue disease (CTD). Read More

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http://dx.doi.org/10.2147/IMCRJ.S151693DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6219426PMC
November 2018
8 Reads

Acute Thyroid Storm Following Thymectomy: A Surprising Result of Undiagnosed Graves' Disease.

Cureus 2018 Aug 31;10(8):e3239. Epub 2018 Aug 31.

Surgery, University of Texas Medical Branch, Galveston, USA.

Postoperative thyroid storm represents a diagnostic dilemma in patients with overlooked hyperthyroid state undergoing a nonthyroid surgery. We report a 30-year-old female with a history of mixed connective tissue disease who presented with an anterior mediastinal mass and underwent a thoracoscopic resection of the mass. On postoperative day 1, she had an acute change in mental status with fever, tachycardia and hypercapnic respiratory failure requiring intubation and mechanical ventilation. Read More

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http://dx.doi.org/10.7759/cureus.3239DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6209514PMC
August 2018
6 Reads

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

RMD Open 2018 18;4(Suppl 1):e000783. Epub 2018 Oct 18.

Department of Rheumatology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany.

Mixed connective tissue disease (MCTD) is a complex overlap disease with features of different autoimmune connective tissue diseases (CTDs) namely systemic sclerosis, poly/dermatomyositis and systemic lupus erythematous in patients with antibodies targeting the U1 small nuclear ribonucleoprotein particle. In this narrative review, we summarise the results of a systematic literature research which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. Since no specific CPGs on MCTD were found, other CPGs developed for other CTDs were taken into consideration in order to discuss what can be applied to MCTD even if designed for other diseases. Read More

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http://rmdopen.bmj.com/lookup/doi/10.1136/rmdopen-2018-00078
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http://dx.doi.org/10.1136/rmdopen-2018-000783DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6203102PMC
October 2018
19 Reads

Assessment of renal function in patients with myositis and treated with subcutaneous immunoglobulin: a series of 24 cases.

Ther Adv Musculoskelet Dis 2018 Oct 7;10(10):201-207. Epub 2018 Oct 7.

Octapharma France, Boulogne-Billancourt, France.

Immunoglobulin (Ig) therapy is used to treat a wide range of immunodeficiencies and autoimmune diseases; While, its clinical benefit has been demonstrated in several studies, Ig therapy is associated with a risk of systemic adverse effects. As such, Onset of renal impairment, including acute renal failure, osmotic nephrosis and renal insufficiency, after immunoglobulin administration is rare, but is one of the most significant concerns related to intravenous Ig use at immunomodulatory doses. However, only few studies have investigated the safety of subcutaneous Ig (SCIg) in relation to these rare conditions. Read More

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http://journals.sagepub.com/doi/10.1177/1759720X18787765
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http://dx.doi.org/10.1177/1759720X18787765DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6178375PMC
October 2018
15 Reads

Clinical and immunological profile in patients with mixed connective tissue disease.

J Pak Med Assoc 2018 Jun;68(6):959-962

Medical Unit-II, Jinnah Postgraduate Medical Centre, Karachi.

Mixed connective tissue disease (MCTD) is a rare disease and presents with varied overlapping symptoms of different connective tissue disorders. Many patients evolve into other connective tissue disorders with the passage of time. The case series included 20 patients with the diagnosis of MCTD, registered at the Rheumatology Clinic of Jinnah Postgraduate Medical Centre (JPMC), Karachi, from June 2010 to May 2015. Read More

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June 2018
1 Read

Neonatal punctate calcifications associated with maternal mixed connective tissue disorder (MCTD).

BMJ Case Rep 2018 Oct 12;2018. Epub 2018 Oct 12.

Neonatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

Chondrodysplasia punctate (CDP) is a rare group of disorders with both genetic and non-genetic underlying aetiologies. The genetic causes associated with CDP include peroxisomal disorders, type two mucolipidosis, type 3 mucopolysaccharidosis, GM1 gangliosidosis and chromosomal disorders. Peroxisomal disorders include deficiency of dihydroxyacetone phosphate acyltransferase, encoded by GNPAT, deficiency of the peroxisomal enzyme alkyl-dihydroxyacetone phosphate synthase, encoded by AGPS and Zellweger syndrome. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2017-22337
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http://dx.doi.org/10.1136/bcr-2017-223373DOI Listing
October 2018
8 Reads

Acroosteolysis in a patient with systemic lupus erythematosus/mixed connective tissue disease.

Rheumatology (Oxford) 2018 Oct 12. Epub 2018 Oct 12.

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

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https://academic.oup.com/rheumatology/advance-article/doi/10
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http://dx.doi.org/10.1093/rheumatology/key315DOI Listing
October 2018
3 Reads

Comparison of articular manifestations of mixed connective tissue disease and systemic lupus erythematosus on clinical examination and musculoskeletal ultrasound.

Lupus 2018 Nov 10;27(13):2086-2092. Epub 2018 Oct 10.

1 Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Background: Polyarthritis is common to both mixed connective tissue disease (MCTD) and systemic lupus erythematosus (SLE). Apart from being erosive and deforming in the former, we speculated that it was more common and the extent of joints involved would be higher in MCTD.

Methods: This was a cross-sectional study that included patients with MCTD aged 18-75 years fulfilling the Kasukawa criteria. Read More

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http://dx.doi.org/10.1177/0961203318804891DOI Listing
November 2018
1 Read
2.200 Impact Factor

The use of ultrasound for assessing interstitial lung involvement in connective tissue diseases.

Clin Exp Rheumatol 2018 Sep-Oct;36 Suppl 114(5):165-170. Epub 2018 Oct 1.

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

Interstitial lung disease (ILD) is one of the most serious pulmonary complications associated with connective tissue diseases (CTDs), resulting in significant morbidity and mortality. Recently, lung ultrasound examination has appeared as a useful, feasible, non-invasive, radiation-free imaging technique with high sensitivity and specificity for the diagnosis of CTD-associated ILD. In this review, we discuss the literature concerning lung ultrasound findings (B-lines and pleural irregularities) in systemic sclerosis. Read More

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January 2019
4 Reads

Radiological images of interstitial pneumonia in mixed connective tissue disease compared with scleroderma and polymyositis/dermatomyositis.

Eur J Radiol 2018 Oct 9;107:26-32. Epub 2018 Aug 9.

Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1, Tomioka-Higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address:

Objective: Little has been reported on the radiological and pathological findings of interstitial pneumonia in mixed connective tissue disease (MCTD). There may be possible difference in treatment response and prognosis between the imaging patterns of systemic sclerosis (SSc)-like and polymyositis/dermatomyositis (PM/DM)-like. The purpose of this study was to examine whether the radiological images of interstitial pneumonia in MCTD presented SSc-like or PM/DM-like pattern, and to assess whether the imaging patterns corresponded to clinical and pathological features. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S0720048X183027
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http://dx.doi.org/10.1016/j.ejrad.2018.08.005DOI Listing
October 2018
4 Reads

Phosphaturic mesenchymal tumor and related wound problem.

Medicine (Baltimore) 2018 Oct;97(40):e12507

Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai.

Introduction: Phosphaturic mesenchymal tumor mixed connective tissue type (PMT/MCT) is the most common type (up to 90%) of phosphaturic mesenchymal tumor (PMT), a rare clinicopathologic entity. Besides overproduction of fibroblast growth factor 23 (FGF23), there is a big variation of immunohistochemical characteristic across types of PMT, which makes it difficult to obtain an early diagnosis of PMT/MCT. As a benign tumor, PMT/MCT usually happens in subcutaneous tissues and leads to nonhealing of wound. Read More

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http://Insights.ovid.com/crossref?an=00005792-201810050-0002
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http://dx.doi.org/10.1097/MD.0000000000012507DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6200523PMC
October 2018
12 Reads

Collagen Vascular Diseases: SLE, Dermatomyositis, Scleroderma, and MCTD.

Pediatr Rev 2018 Oct;39(10):501-515

Division of Pediatric Rheumatology, Department of Pediatrics, University of Minnesota and the University of Minnesota Masonic Children's Hospital, Minneapolis, MN.

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http://dx.doi.org/10.1542/pir.2017-0262DOI Listing
October 2018
6 Reads

Rheumatologic Tests: A Primer for Family Physicians.

Authors:
Yousaf Ali

Am Fam Physician 2018 Aug;98(3):164-170

Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Patients with a suspected connective tissue disorder should undergo serologic testing to confirm the diagnosis and, in some cases, to monitor disease activity and predict flares. Patients with suspected systemic lupus erythematosus should be tested for antinuclear antibodies. However, antinuclear antibodies are not specific and may be present in many other connective tissue disorders and nonrheumatologic diseases. Read More

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August 2018
5 Reads
1.820 Impact Factor

Natural history and extracutaneous involvement of congenital morphea: Multicenter retrospective cohort study and literature review.

Pediatr Dermatol 2018 Nov 6;35(6):761-768. Epub 2018 Sep 6.

Section of Dermatology, Division of Paediatric Medicine, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.

Background: Congenital morphea is a form of localized scleroderma that presents at birth. There is limited information on its presentation and progression.

Methods: Patients with congenital morphea were identified from five pediatric dermatology and rheumatology tertiary care centers in Canada, the United States, and Italy from 2001 to 2016. Read More

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http://dx.doi.org/10.1111/pde.13605DOI Listing
November 2018
10 Reads

Acroosteolysis in a patient with systemic lupus erythematosus/mixed connective tissue disease.

Rheumatology (Oxford) 2018 Aug 31. Epub 2018 Aug 31.

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

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http://dx.doi.org/10.1093/rheumatology/key270DOI Listing
August 2018
2 Reads

The Effect of Statin Use on Mortality in Systemic Autoimmune Rheumatic Diseases.

J Rheumatol 2018 Dec 1;45(12):1689-1695. Epub 2018 Sep 1.

From the Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Objective: Systemic autoimmune rheumatic diseases (SARD) are associated with an increased risk of premature cardiovascular disease (CVD) and all-cause mortality. We examined the potential survival benefit of statin use among patients with SARD in a general population setting.

Methods: We conducted an incident user cohort study using a UK general population database. Read More

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http://dx.doi.org/10.3899/jrheum.171389DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6289699PMC
December 2018
31 Reads

Devic's syndrome and mixed connective tissue disease: an unusual association.

Acta Reumatol Port 2018 Apr-Jun;43(2):146-150

Hospital Universitário Getúlio Vargas, Universidade Federal do Amazonas, Manaus, Brasil.

Devic's disease or neuromyelitis optica (NMO) is an autoimmune, inflammatory and demyelinating pathology of the central nervous system that affects the optic nerve and the spinal cord. Diagnosis confirmed by imaging, magnetic resonance (MR) and the presence of the anti-aquaporin 4 antibody (anti-AQP4). We describe two cases of patients with mixed connective tissue disease (MCTD) and Devic's disease, who had anti-AQP4 positive and areas with neuroaxis MR abnormalities, showing this rare association. Read More

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August 2018
5 Reads

Management of multiple neurological complications in mixed connective tissue disease: A case report.

Medicine (Baltimore) 2018 Aug;97(31):e11360

Department of Neurology and Neuroscience Center, The First Hospital of Jilin University.

Rationale: Mixed connective tissue disease (MCTD) refers to an overlapping condition of different autoimmune disorders such as systemic lupus erythematosus, cutaneous systemic sclerosis, rheumatoid arthritis, polymyositis, and dermatomyositis. However, MCTD manifesting as transverse myelitis is extremely rare. Herein, we report a case of MCTD with both central and peripheral nervous system involvement. Read More

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http://dx.doi.org/10.1097/MD.0000000000011360DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6081063PMC
August 2018
2 Reads

Pulmonary Manifestations and Progression of Lung Disease in Juvenile-onset Mixed Connective Tissue Disease.

J Rheumatol 2019 Jan 1;46(1):93-100. Epub 2018 Aug 1.

From the Department of Rheumatology, the Department of Radiology and Nuclear Medicine, and the Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet; Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo; Department of Pediatrics, University Hospital of North Norway; Institute of Clinical Medicine, University of Tromsø, Tromsø; Department of Pediatrics, St. Olavs Hospital; Institute of Laboratory Medicine, Children's and Women's Health, Faculty of Medicine, Norwegian University of Science and Technology, Trondheim, Norway.

Objective: To assess the occurrence and extent of interstitial lung disease (ILD) in patients with juvenile mixed connective tissue disease (JMCTD), compare pulmonary function in patients and matched controls, study associations between ILD and disease-related variables, and examine progression of pulmonary manifestations over time.

Methods: A cohort of 52 patients with JMCTD were examined in a cross-sectional study after a mean 16.2 (SD 10. Read More

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http://dx.doi.org/10.3899/jrheum.180019DOI Listing
January 2019
13 Reads

Less common rheumatologic disorders: Current concepts of skin and systemic manifestations.

Authors:
Taraneh Paravar

Clin Dermatol 2018 Jul - Aug;36(4):525-532. Epub 2018 Apr 10.

Department of Dermatology, University of California-San Diego School of Medicine, San Diego, California, USA. Electronic address:

The cutaneous manifestations of the common rheumatologic disorders, such as systemic lupus erythematosus, dermatomyositis, and systemic sclerosis, are well known. In contrast, the dermatologic findings of less common rheumatologic disorders, including Sjögren syndrome, mixed connective tissue disease, and relapsing polychondritis, are less widely known. The cutaneous manifestations of these connective tissue disorders are reviewed. Read More

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http://dx.doi.org/10.1016/j.clindermatol.2018.04.009DOI Listing
December 2018
2 Reads

Systemic Lupus Erythematosus, Sjögren Syndrome, and Mixed Connective Tissue Disease in Children and Adolescents.

Pediatr Clin North Am 2018 08;65(4):711-737

Division of Rheumatology, Department of Pediatrics, University of Indiana School of Medicine, Riley Hospital for Children at Indiana University Health, 699 Riley Hospital Drive, Riley Research 307, Indianapolis, IN 46202, USA. Electronic address:

Juvenile systemic lupus erythematosus (jSLE), mixed connective tissue disease (jMCTD), and Sjögren syndrome (jSS) are systemic autoimmune and inflammatory disorders with distinct patterns of organ involvement. All are characterized by autoantibody formation, with antinuclear (ANA) and anti-double-stranded DNA common in jSLE, ANA with high-titer ribonucleoprotein antibody in jMCTD, and Sjögren syndrome A and Sjögren syndrome B antibodies + ANA in jSS. Recognition, monitoring, and management for primary care providers are discussed, focusing on the role of primary physicians in recognizing and helping maintain optimal health in children with these potentially life-threatening diseases. Read More

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http://dx.doi.org/10.1016/j.pcl.2018.04.001DOI Listing
August 2018
9 Reads

Ethmoid tumor and oncogenic osteomalacia: Case report and review of the literature.

Eur Ann Otorhinolaryngol Head Neck Dis 2018 Oct 17;135(5):365-369. Epub 2018 Jul 17.

Département de cancérologie cervicofaciale, Gustave Roussy Cancer Campus, Université Paris-Saclay, 114, rue Edouard-Vaillant, 94800 Villejuif, France.

Introduction: Oncogenic osteomalacia is a very rare disease usually caused by a phosphaturic mesenchymal tumor, particularly the "mixed connective tissue type", secreting FGF-23 hormone.

Objective: The authors report a case of ethmoid tumor associated with oncogenic osteomalacia and discuss management based on a review of the literature.

Case Summary: A 41-year-old woman with multiple fractures causing major disability was diagnosed with early-onset osteoporosis. Read More

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http://dx.doi.org/10.1016/j.anorl.2018.07.001DOI Listing
October 2018
3 Reads

Prevalence of celiac disease serological markers in a cohort of Italian rheumatological patients.

Gastroenterol Hepatol Bed Bench 2018 ;11(3):244-249

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

Aim: To assess the prevalence of celiac disease (CD) serological markers in a cohort of patients referred to an Italian rheumatological outpatient clinic.

Background: Current guidelines do not suggest CD screening in patients with rheumatological diseases and these subjects are not considered to be at high risk for CD.

Methods: A total of 230 sera of rheumatological patients referred to the Division of Internal Medicine at the Department of Medical and Surgical Sciences between January 2005 and December 2013 were screened for CD by testing IgA antitransglutaminase (TTG IgA), IgG deamidated gliadin peptides (DGP IgG) and IgA antiendomysium (EMA) antibodies. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6040033PMC
January 2018
7 Reads

Prevalence of overlap of antineutrophil cytoplasmic antibody associated vasculitis with systemic autoimmune diseases: an unrecognized example of poliautoimmunity.

Clin Rheumatol 2018 Jul 14. Epub 2018 Jul 14.

Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga No. 15, Col. Sección XVI, Tlalpan, CP 14000, Mexico City, Mexico.

We aimed to estimate the frequency of overlap of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with systemic autoimmune diseases. Retrospective single-center study to identify patients with AAV diagnosis and concomitant autoimmune systemic diseases, simultaneously, before or after the diagnosis of AAV. Sociodemographic characteristics, such as comorbidities; follow-up time; type of AAV; disease duration; relapses; treatment and response; clinical, serological, and histological characteristics; disease activity and damage; prognosis; dialysis requirements, and death were assessed. Read More

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http://dx.doi.org/10.1007/s10067-018-4212-1DOI Listing
July 2018
14 Reads

Prevalence of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease.

Medicine (Baltimore) 2018 Jul;97(28):e11437

Department of Rheumatology and Internal Diseases.

Systemic sclerosis (SSc) and mixed connective tissue disease (MCTD) are 2 conditions in which pulmonary hypertension (PH) can develop.We retrospectively analyzed the probability of PH in case of 83 patients (69 SSc and 14 MCTD). The European Society of Cardiology/European Respiratory Society (ESC/ERS) echocardiographic guidelines of 2015 were used for the evaluation. Read More

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http://dx.doi.org/10.1097/MD.0000000000011437DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6076033PMC
July 2018
7 Reads
5.723 Impact Factor

Systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis: An emerging overlap syndrome with cutaneous manifestations.

JAAD Case Rep 2018 Jun 7;4(5):493-496. Epub 2018 May 7.

The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York.

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http://dx.doi.org/10.1016/j.jdcr.2018.01.021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6031565PMC
June 2018
4 Reads

Identification of hnRNP C1/C2 as an Autoantigen in Patients with Behcet's Disease.

Iran J Immunol 2018 Jun;15(2):133-141

School of Chemistry and Biological Engineering, University of Science and Technology Beijing, Beijing, China.

Background: Ribonucleoproteins particles that form the spliceosomes are among the most frequently targeted molecules of the autoimmune response. In the last few years, autoantibodies against all A/B hnRNP proteins have been found in the sera of patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), and serve as diagnostic markers for several rheumatic diseases. However, the functional role of hnRNP C1/C2 in autoimmune diseases is still not clearly understood. Read More

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http://dx.doi.org/IJIv15i2A6DOI Listing
June 2018
13 Reads
0.710 Impact Factor

Antiviral activity of maraviroc plus mirtazapine in a low-risk HIV-negative patient with progressive multifocal leukoencephalopathy.

Infez Med 2018 Jun;26(2):160-163

Unit of Infectious Diseases, Department of Medical Sciences, University of Torino, Italy.

A case of progressive multifocal leukoencephalopathy (PML) is described in an HIV-negative patient with mixed connective-tissue disease (MCTD) on a minimally immunosuppressive treatment with hydroxychloroquine. The patient presented with right-sided weakness, episodes of disorientation and loss of short-term memory and of vision in her right eye. PML was diagnosed by JCV DNA on cerebrospinal fluid and radiological criteria. Read More

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June 2018
22 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
3 Reads

Lymphocytic interstitial pneumonia in a patient with mixed connective tissue disease - A case report.

Respir Med Case Rep 2018 5;25:12-17. Epub 2018 Jun 5.

Division of Chest Medicine, Department of Internal Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.

Lymphocytic interstitial pneumonia (LIP) is an uncommon interstitial lung disease that is characterized by an interstitial infiltrate of lymphoplasmacytic cells. While idiopathic LIP appears to be extremely rare, most reported cases of LIP have been associated with coexisting immune derangements, particularly autoimmune diseases such as Sjögren's syndrome. In this report, we describe the presentation of LIP in a patient with underlying mixed connective tissue disease. Read More

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http://dx.doi.org/10.1016/j.rmcr.2018.06.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5991914PMC

Antinuclear antibodies and cancer: A literature review.

Crit Rev Oncol Hematol 2018 Jul 26;127:42-49. Epub 2018 May 26.

Infanta Sofía University Hospital, SS de los Reyes, Madrid, Spain. Electronic address:

Antinuclear antibodies (ANAs) are a spectrum of autoantibodies targeted to various nuclear and cytoplasmic components of the cells. They are very useful as serological markers for different autoimmune disease, like systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), scleroderma, polymyositis, or mixed connective tissue disease. In these years, an increasing attention has been focussed in the relationship between tumours and autoimmunity. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10408428173054
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http://dx.doi.org/10.1016/j.critrevonc.2018.05.002DOI Listing
July 2018
5 Reads

HRCT findings of collagen vascular disease-related interstitial pneumonia (CVD-IP): a comparative study among individual underlying diseases.

Clin Radiol 2018 Sep 30;73(9):833.e1-833.e10. Epub 2018 May 30.

Division of Pathology, Fujisawa City Hospital, 2-6-1 Fujisawa, Fujisawa, Kanagawa 251-8550, Japan.

Aim: To identify characteristic high-resolution computed tomography (CT) findings for individual collagen vascular disease (CVD)-related interstitial pneumonias (IPs).

Materials And Methods: The HRCT findings of 187 patients with CVD, including 55 patients with rheumatoid arthritis (RA), 50 with systemic sclerosis (SSc), 46 with polymyositis/dermatomyositis (PM/DM), 15 with mixed connective tissue disease, 11 with primary Sjögren's syndrome, and 10 with systemic lupus erythematosus, were evaluated. Lung parenchymal abnormalities were compared among CVDs using χ test, Kruskal-Wallis test, and multiple logistic regression analysis. Read More

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http://dx.doi.org/10.1016/j.crad.2018.04.017DOI Listing
September 2018
36 Reads

Evaluation of an Automated Screening Assay, Compared to Indirect Immunofluorescence, an Extractable Nuclear Antigen Assay, and a Line Immunoassay in a Large Cohort of Asian Patients with Antinuclear Antibody-Associated Rheumatoid Diseases: A Multicenter Retrospective Study.

J Immunol Res 2018 2;2018:9094217. Epub 2018 May 2.

Department of Laboratory Medicine, Severance Hospital, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul 03722, Republic of Korea.

We assessed the diagnostic utility of the connective tissue disease (CTD) screen as an automated screening test, in comparison with the indirect immunofluorescence (IIF), EliA extractable nuclear antigen (ENA), and line immunoassay (LIA) for patients with antinuclear antibody- (ANA-) associated rheumatoid disease (AARD). A total of 1115 serum samples from two university hospitals were assayed using these four autoantibody-based methods. The AARD group consisted of patients with systemic lupus erythematosus (SLE), systemic sclerosis (SSc), Sjögren's syndrome (SS), and mixed connective tissue disease (MCTD). Read More

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https://www.hindawi.com/journals/jir/2018/9094217/
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http://dx.doi.org/10.1155/2018/9094217DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5954951PMC
October 2018
16 Reads

Superior sagittal sinus thrombosis in a case of Mixed Connective Tissue Disease.

BMJ Case Rep 2018 May 30;2018. Epub 2018 May 30.

Department of General Medicine, Jaslok Hospital and Research Centre, Mumbai, Maharashtra, India.

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http://dx.doi.org/10.1136/bcr-2018-225078DOI Listing
May 2018
10 Reads

Bamboo Nodes of Vocal Folds: A Description of 10 Cases and Review of the Literature.

Folia Phoniatr Logop 2018 30;70(1):1-7. Epub 2018 May 30.

Department of Otorhinolaryngology, Faculty of Medicine, University of Geneva Hospitals, Geneva, Switzerland.

Objective: Bamboo nodes are vocal fold lesions, mostly associated with autoimmune diseases.

Patients And Methods: This is a retrospective clinical study including 10 patients with bamboo nodes. Data were collected regarding associated autoimmune disorder and type of treatment. Read More

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http://dx.doi.org/10.1159/000487925DOI Listing
May 2018
9 Reads

The impact of anti-U1-RNP positivity: systemic lupus erythematosus versus mixed connective tissue disease.

Rheumatol Int 2018 07 23;38(7):1169-1178. Epub 2018 May 23.

Internal Medicine Department, Carol Davila University of Medicine and Pharmacy, Dionisie Lupu Street 37, 020022, Bucharest S2, Romania.

Anti-U1-RNP positivity remains mandatory for the mixed connective tissue disease (MCTD) diagnosis, reason for which anti-U1-RNP occurrence in patients with lupus clinical features might determine diagnostic issues. Thus, the prevalence of 25-30% for anti-RNP was reported in John Hopkins and LUMINA lupus cohorts and also 13% prevalence for the anti-U1-RNP in Euro-Lupus cohort. Presence of anti-U1-RNP antibodies in patients fulfilling SLE criteria (but not the MCTD ones) was associated with manifestations such as Raynaud phenomenon, musculoskeletal and lung impairment or nail fold capillaroscopy changes, some clinical features frequently encountered in MCTD patients and only rarely described in lupus population. Read More

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http://dx.doi.org/10.1007/s00296-018-4059-4DOI Listing
July 2018
5 Reads

Dermoscopic patterns of filiform papillae of the tongue in patients with and without connective tissue autoimmune diseases.

Int J Dermatol 2018 Aug 18;57(8):938-942. Epub 2018 May 18.

Department of Stomatology, Institute of Biomedical Sciences, Autonomous University of Ciudad Juárez, Ciudad Juárez, Chihuahua, Mexico.

Background: Connective tissue autoimmune diseases (CTADs) constitute a group of conditions, including rheumatoid arthritis; systemic lupus erythematosus; mixed connective tissue disease; calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome; scleroderma; dermatomyositis; and Sjögren syndrome. There are few studies on the alterations in filiform papillae in CTAD. Thus, the objective of this work was to determine whether there are changes in the macroscopic and dermoscopic patterns of filiform papillae. Read More

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http://dx.doi.org/10.1111/ijd.14038DOI Listing
August 2018
11 Reads

Bentall’s Procedure in Pediatric Mixed Connective Tissue Disease Syndrome: Management of Pediatric Aortic Aneurysm - A Brief Review.

J Heart Valve Dis 2017 09;26(5):610-612

MIOT Hospitals, Manapakkam, Chennai, India.

Mixed connective tissue disease (MCTD) syndrome in children may lead to large aortic aneurysms, which in turn pose a difficult surgical problem. Valve-sparing root replacement is not always a viable option as the disease process invariably affects the aortic valve leaflets. Among pediatric patients, the Ross procedure is contraindicated on account of weakness of the pulmonary root, while Bentall surgery is the 'gold standard' treatment of aortic aneurysm, with reproducible and excellent long-term results. Read More

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September 2017
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
7 Reads

Anti-High-Density Lipoprotein Antibodies and Antioxidant Dysfunction in Immune-Driven Diseases.

Front Med (Lausanne) 2018 23;5:114. Epub 2018 Apr 23.

Area of Immunology, Department of Functional Biology, University of Oviedo, Oviedo, Spain.

Introduction: Impaired high-density lipoprotein (HDL) levels and antioxidant functionality of HDL, mainly attributed to a decreased paraoxonase-1 (PON1) functionality, have been described in autoimmune conditions. In this setting, a role for humoral response in cardiovascular disease is emerging. This study evaluates the role of immunoglobulin G (IgG) antibodies against HDL and disease-related autoantibodies on HDL dysfunction in immune-driven diseases. Read More

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http://dx.doi.org/10.3389/fmed.2018.00114DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5925573PMC
April 2018
4 Reads