2,700 results match your criteria Mixed Connective Tissue Disease


Coexistence of Mixed Connective Tissue Disease and Familial Mediterranean Fever in a Japanese Patient: A Case Report.

Intern Med 2019 Apr 17. Epub 2019 Apr 17.

Department of Rheumatology, Fukushima Medical University School of Medicine, Japan.

We herein report a Japanese patient with familial Mediterranean fever (FMF) who developed the clinical manifestations of mixed connective tissue disease (MCTD) and Sjögren's syndrome. The patient was a 36-year-old woman presenting with a periodic short-duration (2-3 days) fever and pleural pain. An MEFV gene analysis detected a complex allele mutation (P369S/R408Q) in exon 3 of the MEFV gene. Read More

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http://dx.doi.org/10.2169/internalmedicine.2376-18DOI Listing

Overlap myositis, a distinct entity beyond primary inflammatory myositis: A retrospective analysis of a large cohort from the REMICAM registry.

Int J Rheum Dis 2019 Apr 10. Epub 2019 Apr 10.

Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain.

Background: Inflammatory idiopathic myositis (IIM) comprises a heterogeneous group of systemic muscular diseases that can occur together with other connective tissue diseases (CTD), named overlap myositis (OM). The question of whether OM is a distinct entity still remains controversial.

Aim: The present study was conducted to assess the clinical and prognostic differences between patients diagnosed with OM, primary polymyositis (PM) and primary dermatomyositis (DM). Read More

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http://dx.doi.org/10.1111/1756-185X.13559DOI Listing
April 2019
1 Read

High cardiovascular risk in mixed connective tissue disease: evaluation of macrovascular involvement and its predictors by aortic pulse wave velocity.

Clin Exp Rheumatol 2019 Apr 2. Epub 2019 Apr 2.

ACURA Rheumatology Clinics, Bad Kreuznach, and Division of Rheumatology and Clinical Immunology, University Medical Center of the Johannes Gutenberg University Mainz, Germany.

Objectives: Macrovascular involvement and cardiovascular (CV) risk has not been sufficiently studied in mixed connective tissue disease (MCTD). In particular, the gold standard assessment method of aortic stiffness carotid-femoral pulse wave velocity (cfPWV) has never been evaluated in patients with this disease. The aims of the present study were therefore to examine cfPWV in MCTD and to evaluate its associations with MCTD-associated markers and traditional CV risk factors. Read More

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April 2019
4 Reads

Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) demonstrates distinct autoimmune and autoinflammatory disease associations according to the adjuvant subtype: Insights from an analysis of 500 cases.

Clin Immunol 2019 Mar 25;203:1-8. Epub 2019 Mar 25.

Department of Medicine 'B' and Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Laboratory of the Mosaics of Autoimmunity, Saint Petersburg University, Saint Petersburg, Russian Federation. Electronic address:

Background: We investigated the pattern of reported immune diseases in the international ASIA syndrome registry.

Methods: Data from 500 subjects exposed to adjuvants from the ASIA syndrome international registry were analysed.

Results: The patient mean age was 43 ± 17 years and 89% were female. Read More

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http://dx.doi.org/10.1016/j.clim.2019.03.007DOI Listing
March 2019
3 Reads

Palisaded Neutrophilic Granulomatous Dermatitis Leading to Diagnosis of Hodgkin Lymphoma: Report of Rare Case and Literature Review of Paraneoplastic Granulomatous Dermatitides.

Am J Dermatopathol 2019 Apr 1. Epub 2019 Apr 1.

Department of Dermatology, University of Wisconsin, Madison, WI.

Palisaded neutrophilic granulomatous dermatitis (PNGD) presents as skin-colored to erythematous papules symmetrically distributed on extensor extremities. Histologically, interstitial and/or palisaded granulomatous infiltrates are present with neutrophils and leukocytoclasis. Together with interstitial granulomatous dermatitis (IGD), PNGD is considered a reactive granulomatous dermatitis to a systemic trigger including connective tissue disease, infection, medication reaction, and less commonly malignancy. Read More

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http://dx.doi.org/10.1097/DAD.0000000000001411DOI Listing
April 2019
1 Read

Mobility in osteogenesis imperfecta: a multicenter North American study.

Genet Med 2019 Mar 28. Epub 2019 Mar 28.

Orthopaedic Rehabilitation and Engineering Center, Marquette University, Milwaukee, WI, USA.

Purpose: Osteogenesis imperfecta (OI) is a genetic connective tissue disorder that causes bone fragility. Phenotypic severity influences ability to walk, however, little is known about ambulatory characteristics of individuals with OI, especially in more severe forms. The purpose of this work was to characterize mobility in OI using standard clinical assessment tools and determine if patient characteristics could be used to predict mobility outcomes. Read More

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http://dx.doi.org/10.1038/s41436-019-0491-4DOI Listing
March 2019
2 Reads

Anca negative pauci-immune crescentic glomerulonephritis and mixed connective tissue disease: a case study.

J Bras Nefrol 2019 Mar 18. Epub 2019 Mar 18.

Hospital Beatriz Angelo, Loures, Portugal.

One of the most common causes of rapidly progressive glomerulonephritis (RPGN) is pauci-immune crescentic glomerulonephritis (CrGN). In the majority of cases, this condition has a positive serologic marker, the anti-neutrophil cytoplasmic antibodies (ANCAs), but in approximately 10% there are no circulating ANCAs, and this subgroup has been known as the ANCA-negative pauci-immune CrGN. RPGN can be associated with systemic diseases, but there are only few case reports describing the association with mixed connective tissue disease (MCTD). Read More

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http://dx.doi.org/10.1590/2175-8239-JBN-2019-0003DOI Listing
March 2019
1 Read

Mixed connective tissue disease in pregnancy: A case series and systematic literature review.

Obstet Med 2019 Mar 19;12(1):31-37. Epub 2018 Sep 19.

Internal Medicine Division, Department of Medicine, Centre Hospitalier de l'Université de Montréal (CHUM), Montréal, QC, Canada.

Objective: To investigate the impact of medical and obstetric complications associated with mixed connective tissue disease (MCTD) in pregnancy.

Method: We analyzed 68 pregnancies from a systematic literature review and 12 pregnancies affected by MCTD at our centre between 1986 and 2015 for medical and obstetric complications.

Results: During pregnancy 37. Read More

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http://journals.sagepub.com/doi/10.1177/1753495X18793484
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http://dx.doi.org/10.1177/1753495X18793484DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6416686PMC
March 2019
4 Reads

3D evaluation of maxillary morphology in Marfan growing subjects: a controlled clinical study.

Prog Orthod 2019 Mar 18;20(1):12. Epub 2019 Mar 18.

Department of Clinical Sciences and Translational Medicine, University of Rome Tor Vergata, via Montpellier 1, 00133, Rome, Italy.

Background: Marfan syndrome is a rare autosomal dominant inherited disease of the connective tissue associated with various craniofacial abnormalities. Aim of the present study was to assess the variability of palatal shape in a sample of 31 Marfan patients compared to a control group of no syndromic subjects, in two stages of dentition, by using 3D geometric morphometric analysis.

Methods: Thirty one growing subjects with Marfan syndrome were selected and divided into two subgroups: MG1 with mixed dentition (10 M, 6F, mean age 7+/- 0. Read More

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https://progressinorthodontics.springeropen.com/articles/10.
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http://dx.doi.org/10.1186/s40510-019-0264-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6421354PMC
March 2019
3 Reads

Effect on Cardiac Function of Longstanding Juvenile-onset Mixed Connective Tissue Disease: A Controlled Study.

J Rheumatol 2019 Mar 15. Epub 2019 Mar 15.

From the Institute for Experimental Medical Research and K.G. Jebsen Center for Cardiac Research, and the Section of Infectious Diseases, and the Department of Cardiology, Oslo University Hospital - Ullevål; University of Oslo; Department of Rheumatology, Oslo University Hospital - Rikshospitalet; Bjørknes College; Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo, Norway; Department of Translational Medicine, Medical School, University of Pécs, Pécs, Hungary. This study was funded by The Norwegian Rheumatism Association and made possible by the Norwegian ExtraFoundation for Health and Rehabilitation. B.N. Witczak, MD, Institute for Experimental Medical Research and K.G. Jebsen Center for Cardiac Research, Oslo University Hospital - Ullevål, and University of Oslo; S.O. Hetlevik, MD, Department of Rheumatology, Oslo University Hospital - Rikshospitalet; H. Sanner, MD, PhD, Department of Rheumatology, Oslo University Hospital - Rikshospitalet, and Bjørknes College; Z. Barth, MD, Institute for Experimental Medical Research and K.G. Jebsen Center for Cardiac Research, Oslo University Hospital - Ullevål, and University of Oslo, and Bjørknes College, and Department of Translational Medicine, Medical School, University of Pécs; T. Schwartz, MD, PhD, Institute for Experimental Medical Research and K.G. Jebsen Center for Cardiac Research, and Section of Infectious Diseases, Oslo University Hospital - Ullevål, and University of Oslo; B. Flatø, MD, PhD, Professor, Department of Rheumatology, Oslo University Hospital - Rikshospitalet, and Faculty of Medicine, Institute of Clinical Medicine, University of Oslo; V. Lilleby, MD, PhD, Department of Rheumatology, Oslo University Hospital - Rikshospitalet; I. Sjaastad, MD, PhD, Professor, Institute for Experimental Medical Research and K.G. Jebsen Center for Cardiac Research, and Department of Cardiology, Oslo University Hospital - Ullevål, and University of Oslo. Address correspondence to Dr. S.O. Hetlevik, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Postboks 4950 Nydalen, 0424 Oslo, Norway. E-mail: Accepted for publication November 8, 2018.

Objective: To assess cardiac function in patients with juvenile mixed connective tissue disease (JMCTD) compared to matched controls, and to investigate possible associations between cardiac impairment and disease variables and cardiovascular risk factors.

Methods: Fifty JMCTD patients (86% female) examined median 14.9 (6. Read More

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http://dx.doi.org/10.3899/jrheum.180526DOI Listing
March 2019
1 Read

Imaging findings of mixed connective tissue disease in children and adolescents: a case series.

Jpn J Radiol 2019 Mar 14. Epub 2019 Mar 14.

Department of Rheumatology, The Hospital for Sick Children, Department of Medical Imaging, University of Toronto, Toronto, ON, Canada.

Mixed connective tissue disease (MCTD) is a rare disease in children and adolescents which overlaps features of juvenile idiopathic arthritis, polymyositis/dermatomyositis, systemic lupus erythematosus, and systemic sclerosis. We have provided an image-based approach for evaluation of MCTD in children and adolescents, outlying the most frequent imaging findings. This approach would aid imagers and clinicians to consider the diagnosis of this rare entity and be able to make an accurate list of differential diagnosis for complex rheumatologic diseases such as MCTD, thus facilitating the ultimate goal of early diagnosis and optimal management of affected children. Read More

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http://dx.doi.org/10.1007/s11604-019-00824-4DOI Listing
March 2019
1 Read

Prevalence of autoimmune diseases and clinical significance of autoantibody profile: Data from National Institute of Hygiene in Rabat, Morocco.

Hum Immunol 2019 Feb 23. Epub 2019 Feb 23.

Laboratory of Human Pathologies Biology, Department of Biology, Faculty of Sciences, and Genomic Center of Human Pathologies, Faculty of Medicine and Pharmacy, Mohammed V University in Rabat, Morocco.

Aim: The objective of this study was to explore the prevalence of various autoimmune diseases (AIDs) in a large cohort of patients and to characterize the autoantibody profile in the patients with and without AIDs to confirm the diagnosis and to refine the Moroccan databases.

Patients And Method: Retrospective study was conducted in the Laboratory of autoimmunity National Institute of Hygiene (NIH) of Rabat in Morocco. A total of 3182 consecutive Moroccan patients (2183 females and 999 males) whose sera were tested for 14 autoantibody profile between 2010 and 2016. Read More

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http://dx.doi.org/10.1016/j.humimm.2019.02.012DOI Listing
February 2019
5 Reads

Correction of pulmonary hypertension with intensive hemodialysis: A case report.

Hemodial Int 2019 Apr 20;23(2):E49-E52. Epub 2019 Feb 20.

Division of Nephrology, University Health Network, Toronto, Canada.

Pulmonary hypertension (PH) defined as a mean pulmonary arterial pressure ≥25 mmHg is a common complication in hemodialysis occurring in up to 58% of patients. PH is classified according to its etiology. We report in a patient with severe PH of mixed etiology (connective tissue disease and left-sided heart failure) who improved after initiation of intensive home hemodialysis. Read More

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http://dx.doi.org/10.1111/hdi.12691DOI Listing

Preclinical Carotid Atherosclerosis in Patients With Juvenile-Onset Mixed Connective Tissue Disease.

J Stroke Cerebrovasc Dis 2019 May 14;28(5):1295-1301. Epub 2019 Feb 14.

Department of Neurology, Oslo University Hospital, Rikshospitalet, Oslo, Norway. Electronic address:

Background: This study investigated preclinical atherosclerosis in patients with juvenile mixed connective tissue disease (JMCTD), which is a chronic inflammatory disease with a varied phenotype. Mixed connective tissue disease (MCTD) has well known associations with other autoimmune diseases known to have increased risk of cardiovascular disease. However, the cardiovascular risk for patients with the juvenile form remains unclear. Read More

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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2019.01.027DOI Listing
May 2019
2 Reads

Posterior Reversible Encephalopathy Syndrome and Azathioprine.

Eur J Case Rep Intern Med 2019 25;6(1):001032. Epub 2019 Jan 25.

Serviço Medicina III, Hospital Prof. Doutor Fernando Fonseca, Lisbon, Portugal.

Posterior reversible encephalopathy syndrome (PRES) is a rare syndrome that presents with neurological manifestations, often associated with arterial hypertension. Magnetic resonance imaging (MRI) shows bilateral white matter oedema in the posterior vascular territories. Immunosuppression, (pre) eclampsia and autoimmune diseases can be implicated. Read More

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http://dx.doi.org/10.12890/2019_001032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6372047PMC
January 2019
8 Reads

Cryoglobulinemia: An update in 2019.

Joint Bone Spine 2019 Feb 4. Epub 2019 Feb 4.

Sorbonne universités, UPMC Université Paris 06, Inserm, UMR S 959, immunology immunopathology immunotherapy (I3), 75005, Paris, France; Biotherapy (CIC-BTi) and inflammation-immunopathology-biotherapy department (DHU i2B), hôpital Pitié-Salpêtrière, AP-HP, 75651, Paris, France; AP-HP, groupe hospitalier Pitié-Salpêtrière, department of internal medicine and clinical immunology, centre national de référence maladies autoinflammatoires et amylose inflammatoire, centre national de références maladies autoimmunes systémiques rares, 75013, Paris, France. Electronic address:

Cryoglobulinemia is defined as the persistent presence in serum of abnormal immunoglobulins (Igs) that precipitate at low temperatures and dissolve again upon warming. Cryoglobulins may be composed only of a monoclonal Ig (simple type I cryoglobulinemia), of a monoclonal Ig bound to the constant domain of polyclonal Ig heavy chains (mixed type II cryoglobulinemia), or only of polyclonal Igs (mixed type III cryoglobulinemia). The manifestations of type I cryoglobulinemia are often related to intravascular obstruction, whereas those seen in the mixed cryoglobulinemias often originate in true immune complex-mediated vasculitis. Read More

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http://dx.doi.org/10.1016/j.jbspin.2019.01.016DOI Listing
February 2019
16 Reads

Breast involvement in mixed connective tissue disease.

Radiol Case Rep 2019 Apr 17;14(4):430-435. Epub 2019 Jan 17.

Department of Pathology, Mount Sinai Downtown Union Square. 10 Union Square East, NY 10003, New York, USA.

To date, mammographic involvement in mixed connective tissue disease has not been described in the literature to the authors' knowledge. In addition, only 2 case reports have described panniculitis in mixed connective tissue disease. We present a patient with mixed connective tissue disease with coarse sheet-like calcifications on mammography bilaterally, which increased over time corresponding to rise in levels of anti-dsDNA. Read More

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http://dx.doi.org/10.1016/j.radcr.2019.01.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6348285PMC
April 2019
2 Reads

The Nd:YAG laser as first-line treatment for fibrin pupillary-block glaucoma following uncomplicated cataract surgery.

Oxf Med Case Reports 2019 Jan 24;2019(1):omy113. Epub 2019 Jan 24.

Ophthalmology Department, Great Western Hospital, Swindon, UK.

A 43-year-old lady presented with painful, loss of vision in the right eye 2 weeks after cataract surgery. This is on a background of previous central retinal vein occlusion in the right eye and bilateral diabetic retinopathy. Her past medical history included mixed connective tissue disease for which she is on systemic immunosuppressants. Read More

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https://academic.oup.com/omcr/article/doi/10.1093/omcr/omy11
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http://dx.doi.org/10.1093/omcr/omy113DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6345086PMC
January 2019
8 Reads

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
3 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
3 Reads

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
4 Reads

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
11 Reads

Autoimmunity and immunodeficiency at the crossroad: autoimmune disorders as the presenting feature of selective IgM deficiency.

BMJ Case Rep 2019 Jan 3;12(1). Epub 2019 Jan 3.

Department of Experimental Rheumatology, Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK.

Selective immunoglobulin M deficiency (sIgMD) is an immunodeficiency with undefined pathogenesis and commonly presenting with recurrent infections. The European Society for Immunodeficiencies Registry defines sIgMD as a serum IgM level repeatedly below 2 SD of normal with normal levels of serum IgA, IgG and IgG subclasses, normal vaccination responses, absence of T-cell defects and absence of causative external factors. Rarely it can also be associated with autoimmune diseases. Read More

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http://dx.doi.org/10.1136/bcr-2017-223180DOI Listing
January 2019
6 Reads

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
4 Reads

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

J Biophotonics 2019 Apr 6;12(4):e201800354. Epub 2019 Jan 6.

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
April 2019
4 Reads

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
8 Reads

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
3 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
18 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
19 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
17 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
19 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
33 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
30 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
3 Reads

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
17 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
6 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
4 Reads
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
6 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
7 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
27 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
10 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
7 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
17 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
4 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
44 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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February 2019
8 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
5 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
14 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
4 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
10 Reads