2,878 results match your criteria Mixed Connective Tissue Disease


Role of Osteopontin as a Potential Biomarker of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis and Other Connective Tissue Diseases (CTDs).

Pharmaceuticals (Basel) 2021 Apr 21;14(5). Epub 2021 Apr 21.

Department of Translational Medicine, Università del Piemonte Orientale UPO, 28100 Novara, Italy.

Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue diseases (CTD). Its early diagnosis is essential to start effective treatment. In the present paper, we aimed to evaluate the role of plasma osteopontin (OPN) as a candidate biomarker of PAH in a cohort of CTD patients. Read More

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Case of rare association of peripheral neuropathy with mixed connective tissue disorder.

BMJ Case Rep 2021 Apr 22;14(4). Epub 2021 Apr 22.

Internal Medicine, All India Institute Of Medical Science, Rishikesh, Uttarakhand, India

Chronic inflammatory demyelinating polyneuropathy (CIDP) is probably the best-recognised progressive immune-mediated peripheral neuropathy. It presents with symmetrical, motor predominant peripheral neuropathy that produces both distal and proximal weakness. Here we report a case of a 38-year-old man who presented with chronic additive large and small joint inflammatory polyarthritis, associated with morning stiffness, anasarca associated with frothy urine and progressive episodic, relapsing and remitting, sensorimotor lower motor neuron type quadriparesis without any bladder and bowel involvement. Read More

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Clinico-radiological profile of connective tissue disease related-interstitial lung diseases from a tertiary care centre of India: a cross sectional study.

Monaldi Arch Chest Dis 2021 Apr 2. Epub 2021 Apr 2.

Department of Pulmonary Medicine, All India Institute of Medical Sciences (AIIMS), Jodhpur.

Interstitial lung diseases (ILDs) are a frequently occurring pulmonary manifestation in patients of connective tissue diseases (CTD). Detailed understanding of this subset of lung diseases is vital, hence the study was conducted to analyze the clinico-radiological characteristics of CTD-ILD. The present study was conducted between March 2017 to February 2018 at a tertiary care teaching hospital from western India. Read More

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Thrombotic Microangiopathy Associated with Macrophage Activation Syndrome: A Multinational Study of 23 Patients.

J Pediatr 2021 Apr 7. Epub 2021 Apr 7.

IRCCS Istituto Giannina Gaslini, Genoa, Italy; Università degli Studi di Genova, Genoa, Italy; Sechenov First Moscow State Medical University, Moscow, Russian Federation.

Objective: To describe the clinical characteristics, treatment, and outcomes of a multinational cohort of patients with macrophage activation syndrome (MAS) and thrombotic microangiopathy (TMA).

Study Design: International pediatric rheumatologists were asked to collect retrospectively the data of patients with the co-occurrence of MAS and TMA. Clinical and laboratory features of patients with systemic juvenile idiopathic arthritis (sJIA)-associated MAS and TMA were compared with those of an historical cohort of patients with sJIA and MAS. Read More

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An elevated polyclonal free light chain level reflects a strong interferon signature in patients with systemic autoimmune diseases.

J Transl Autoimmun 2021 2;4:100090. Epub 2021 Mar 2.

Laboratory of Immunology and Immunotherapy, CHRU Morvan, Brest, France.

High amount of polyclonal free light chains (FLC) are reported in systemic autoimmune diseases (SAD) and we took advantage of the PRECISESADS study to better characterize them. Serum FLC levels were explored in 1979 patients with SAD (RA, SLE, SjS, Scl, APS, UCTD, MCTD) and 614 healthy controls. Information regarding clinical parameters, disease activity, medications, autoantibodies (Ab) and the interferon α and/or γ scores were recorded. Read More

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Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial.

Lancet Respir Med 2021 05 30;9(5):476-486. Epub 2021 Mar 30.

Center for Interstitial and Rare Lung Diseases, Justus-Liebig University Giessen, Giessen, Germany; Member of the German Center for Lung Research and Cardiopulmonary Institute, and Agaplesion Lung Clinic Waldhof-Elgershausen, Greifenstein, Germany.

Background: Pirfenidone has been shown to slow disease progression in patients with idiopathic pulmonary fibrosis (IPF). However, there are few treatment options for progressive fibrotic interstitial lung diseases (ILDs)) other than IPF. In view of the pathomechanistic and clinical similarities between IPF and other progressive fibrotic ILDs, we aimed to assess the efficacy and safety of pirfenidone in patients with four non-IPF progressive fibrotic ILDs. Read More

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[Atypical arthritis of the hands : Collagenosis-part 2].

Authors:
Matthias Bollow

Radiologe 2021 May 1;61(5):470-482. Epub 2021 Apr 1.

Klinik für diagnostische und interventionelle Radiologie, Augusta-Kranken-Anstalt Bochum, Bergstraße 26, 44791, Bochum, Deutschland.

Background: Progressive systemic scleroderma (PSS) and mixed connective tissue disease (MCTD) represent vasculitic autoimmune diseases from the group of collagenoses with manifestations in various organ systems such as the skin, the internal organs and the joints.

Objective: To present the atypical arthritis patterns of the hands in PSS and MCTD that differ from those in rheumatoid arthritis (RA) and psoriatic arthritis (PsA) in the context of clinical and serologic findings and in consideration of the classification of PSS and MCTD.

Materials And Methods: Narrative review based on the current literature on the subject from the radiological and rheumatological point of view. Read More

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Outcomes and Risk Factors of Systolic Pulmonary Artery Pressure Progression in Patients with Systemic Rheumatic Diseases: Follow-up Results from a Korean Registry.

Arch Rheumatol 2020 Dec 27;35(4):558-567. Epub 2020 Apr 27.

Department of Internal Medicine, Division of Rheumatology, Gil Medical Center, Gachon University College of Medicine, Incheon, South Korea.

Objectives: This study aims to investigate the outcomes and risk factors associated with the progression of systolic pulmonary artery pressure (sPAP) in patients with systemic rheumatic diseases.

Patients And Methods: A total of 532 patients (73 males, 459 females; median age 49 years; interquartile range (IQR), 36 to 62 years) registered with the Registry of Pulmonary Hypertension Associated with Rheumatic Diseases were included. Mortality curves were constructed using the Kaplan- Meier method and comparisons were performed using the log-rank test. Read More

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December 2020

Capillaroscopy as a diagnostic tool in the diagnosis of mixed connective tissue disease (MCTD): a case report.

BMC Rheumatol 2021 Mar 19;5(1). Epub 2021 Mar 19.

Department of Internal Medicine, Division of Rheumatology, University of Utah, 30 N 1900 E, Ste 4B200, UT, 84132, Salt Lake City, USA.

Background: The concept of mixed connective tissue disease (MCTD) as a unique connective tissue disease has endured for half a century. Disease onset can be in adulthood (MCTD) or of juvenile onset (jMCTD) and is characterized by overlapping features of systemic lupus erythematosus (SLE), polymyositis or dermatomyositis (PM/DM) and systemic sclerosis (SSc). No universally accepted classification criteria for MCTD exists, however agreed upon overlapping disease features include the presence of high titers of U1 small nuclear ribonucleoprotein particle antibodies (U1snRNP) in the peripheral blood, Raynaud's phenomenon, synovitis, myositis and swollen hands or fingers. Read More

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Renal Involvement and HBV Infection Are Common in Chinese Patients With Cryoglobulinemia.

Front Immunol 2021 25;12:580271. Epub 2021 Feb 25.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Science, Beijing, China.

This study aimed to describe the main characteristics of Chinese patients with cryoglobulinemia, especially the characteristics of patients with different causes of cryoglobulinemia. Eighty inpatients diagnosed with cryoglobulinemia from different wards in Peking Union Medical College Hospital were included in this study. Demographic, clinical, biological, and renal pathological data were collected. Read More

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February 2021

Urinary abnormality in mixed connective tissue disease predicts development of other connective tissue diseases and decrease in renal function.

Mod Rheumatol 2021 Mar 30:1-8. Epub 2021 Mar 30.

Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan.

Objective: To clarify the clinical significance of development of urinary abnormality in mixed connective tissue disease (MCTD).

Methods: Forty-one patients with an initial diagnosis of MCTD, followed at five hospitals between April 1, 2000 and December 31, 2013, were included. The relationship between urinary abnormality and various clinical parameters were retrospectively analyzed. Read More

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Clinico-Pathological Profile and Outcomes of 45 Cases of Plasma Cell Gingivitis.

J Clin Med 2021 Feb 18;10(4). Epub 2021 Feb 18.

Department of Neurosciences, Reproductive and Odontostomatological Sciences, Oral Medicine Unit, University of Naples Federico II, 80131 Naples, Italy.

Plasma cell gingivitis (PCG) is an infrequent inflammatory disease of the gingiva of unknown etiology, characterized by a dense polyclonal proliferation of plasma cells in the connective tissue. The aim of this study was to present a case series of patients affected by PCG, analyzing demographic, clinical, histopathological, and therapeutic data. A group of 36 females and 9 males with a mean age of 60. Read More

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February 2021

Prevalence of anti-dense fine speckled 70 antibodies in healthy individuals and patients with antinuclear antibody-associated autoimmune rheumatic diseases in Japan.

Medicine (Baltimore) 2021 Mar;100(9):e24556

Department of Clinical Laboratory, Kobe University Hospital.

Abstract: Previous studies from various countries have reported anti-dense fine speckled pattern (DFS)70 antibody prevalence but few studies have been from Asia. We investigated the prevalence of anti-DFS70 autoantibodies in a Japanese cohort of healthy individuals (HI) and patients with antinuclear antibody-associated autoimmune rheumatic diseases (AARD).Enzyme-linked immunosorbent assay and indirect immunofluorescence were performed using samples from 250 HI and 276 AARD patients. Read More

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Tc-HSA-DTPA Scintigraphy of Protein-Losing Gastroenteropathy Associated with Mixed Connective Tissue Disease Before and After Immunosuppressive Therapy.

Nucl Med Mol Imaging 2021 Feb 7;55(1):46-47. Epub 2021 Jan 7.

Department of Radiology, Faculty of Medicine, Kagawa University, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa 761-0793 Japan.

We present a female in her sixties with mixed connective tissue disease who underwent Tc-human serum albumin diethylenetriaminepentaacetic acid (Tc-HSA-DTPA) scintigraphy to clarify the cause of generalized edema. Scintigraphy findings directed the diagnosis to protein-losing gastroenteropathy. Various disorders are known to be associated with protein-losing gastroenteropathy; however, mixed connective tissue disease is a rare cause. Read More

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February 2021

Risk of primary graft dysfunction following lung transplantation in selected adults with connective tissue disease-associated interstitial lung disease.

J Heart Lung Transplant 2021 May 23;40(5):351-358. Epub 2021 Jan 23.

Division of Rheumatology, Department of Medicine, Columbia University Vagelos College of Physicians and Surgeons, New York, New York. Electronic address:

Background: Previous studies have reported similarities in long-term outcomes following lung transplantation for connective tissue disease-associated interstitial lung disease (CTD-ILD) and idiopathic pulmonary fibrosis (IPF). However, it is unknown whether CTD-ILD patients are at increased risk of primary graft dysfunction (PGD), delays in extubation, or longer index hospitalizations following transplant compared to IPF patients.

Methods: We performed a multicenter retrospective cohort study of CTD-ILD and IPF patients enrolled in the Lung Transplant Outcomes Group registry who underwent lung transplantation between 2012 and 2018. Read More

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Angiofibroma of the Nasal Cavity in a Dog: Case Report.

Top Companion Anim Med 2021 Feb 23;44:100530. Epub 2021 Feb 23.

Postgraduate Program in Animal Science, School of Life Sciences, Pontifical Catholic University of Parana (PUCPR), Curitiba, PR, Brazil.

Nasopharyngeal angiofibroma is a histologic benign but locally aggressive vascular tumor described in humans. The description of this disease in dogs is limited. A mixed-breed female dog was presented with a history of epistaxis and nasal discharge. Read More

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February 2021

Sepsis Mortality Is high in Patients With Connective Tissue Diseases Admitted to the Intensive Care Unit (ICU).

J Intensive Care Med 2021 Feb 25:885066621996257. Epub 2021 Feb 25.

Rheumatology, Medical Department III-Endocrinology, Nephrology and Rheumatology, 9180University Hospital of Leipzig, Leipzig, Germany.

Objectives: Patients with connective tissue diseases (CTD) such as systemic lupus erythematosus (SLE) have an increased risk for infections. This study investigated the outcome and characteristics of CTD patients under intensive care unit (ICU) treatment for sepsis.

Methods: A single-center retrospective analysis was conducted and reviewed all patients with a CTD diagnosis admitted to the ICU of a university hospital for sepsis between 2006 and 2019. Read More

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February 2021

Case 291.

Radiology 2021 Mar;298(3):707-712

From the Department of Imaging and Interventional Radiology, Prince of Wales Hospital, 30-32 Ngan Shing St, Shatin, New Territories, Hong Kong (S.W.Y.Y.); Departments of Radiology (S.H.Y.L., C.S.Y.L., H.Y.F.W.) and Pathology (W.M.T.), Queen Mary Hospital, Hong Kong.

History A 46-year-old woman with known mixed connective tissue disease with clinical features of scleroderma and polymyositis and who was not on specific medications was referred to our institution to assess for interstitial lung disease due to her predisposing condition. She was a nonsmoker, had no respiratory symptoms, and enjoyed good exercise tolerance. She did not have any cutaneous lesions or renal disease. Read More

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Hospital admissions in inflammatory rheumatic diseases during the peak of COVID-19 pandemic: incidence and role of disease-modifying agents.

Ther Adv Musculoskelet Dis 2021 4;13:1759720X20962692. Epub 2021 Feb 4.

Rheumatology Department and IDISSC, Hospital Clínico San Carlos, Madrid, Spain.

Aims: In this pandemic, it is essential for rheumatologists and patients to know the relationship between COVID-19 and inflammatory rheumatic diseases (IRDs). We wanted to assess the role of targeted synthetic or biologic disease-modifying antirheumatic drugs (ts/bDMARDs) and other variables in the development of moderate-severe COVID-19 disease in IRD.

Methods: An observational longitudinal study was conducted during the epidemic peak in Madrid (1 March to 15 April 2020). Read More

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February 2021

Clinical Presentation and Disease Course of 37 Consecutive Cases of Progressive Multifocal Leukoencephalopathy (PML) at a German Tertiary-Care Hospital: A Retrospective Observational Study.

Front Neurol 2021 4;12:632535. Epub 2021 Feb 4.

Division of Infectious Disease, I. Department of Internal Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Progressive multifocal leukoencephalopathy (PML) caused by JCV is a rare but frequently fatal disease of the central nervous system, usually affecting immunocompromised individuals. Our study aims to expand the data on patient characteristics, diagnosis, clinical course, possible PML-directed treatment, and outcome of patients with PML at a German tertiary-care hospital. In this single-center observational cohort study, 37 consecutive patients with a confirmed diagnosis of PML seen at the University Medical Center Hamburg-Eppendorf from 2013 until 2019 were retrospectively analyzed by chart review with a special focus on demographics, risk factors, and clinical aspects as well as PML-directed treatment and survival. Read More

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February 2021

Nailfold Capillaroscopy in Systemic Diseases: Short Overview for Internal Medicine.

Rom J Intern Med 2021 Jan 29. Epub 2021 Jan 29.

Department of Rheumatology, Colentina Clinical Hospital, Bucharest, Romania.

Nailfold capillaroscopy (NFC) is now one of the main imaging tools in systemic sclerosis and imposed over time as an easy, non-invasive method for the nailfold microvascular bed assessment.In qualitative NFC normal pattern is characterized by homogeneous, parallel fashion arrangement of the last capillaries row as well as by capillaries with hairpin or non-specific variations like tortuous and/ or crossing shape.Nailfold capillaroscopy is strongly recommended for evaluation of all patients with Raynaud phenomenon. Read More

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January 2021

Fulminant myocarditis in a COVID-19 positive patient treated with mechanical circulatory support - a case report.

Eur Heart J Case Rep 2021 Feb 30;5(2):ytaa523. Epub 2020 Dec 30.

Department of Cardiology in Linköping and Department of Health, Medicine and Caring Sciences, Unit of Cardiovascular Sciences, Linköping University Hospital, SE-58185 Linköping, Sweden.

Background: Coronavirus disease 2019 (COVID-19) spreading from Wuhan, Hubei province in China, is an expanding global pandemic with significant morbidity and mortality. Even though respiratory failure is the cardinal form of severe COVID-19, concomitant cardiac involvement is common. Myocarditis is a challenging diagnosis due to heterogeneity of clinical presentation, ranging from mild symptoms to fatal arrhythmia and cardiogenic shock (CS). Read More

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February 2021

Scleroderma Renal Crisis in a Case of Mixed Connective Tissue Disease Treated Successfully with Angiotensin-Converting Enzyme Inhibitors.

Case Rep Nephrol 2021 6;2021:8862405. Epub 2021 Jan 6.

Department of Internal Medicine, Faculty of Medicine, Al-Quds University, Abu Dis, State of Palestine.

Mixed connective tissue disease (MCTD) is a rheumatic disease syndrome with overlapping features of scleroderma, systemic lupus erythematosus, and polymyositis. An extremely rare but serious complication that can occur in MCTD is scleroderma renal crisis (SRC). There have been different approaches to the treatment of SRC associated with MCTD. Read More

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January 2021

miR‑212 promotes renal interstitial fibrosis by inhibiting hypoxia‑inducible factor 1‑α inhibitor.

Mol Med Rep 2021 03 26;23(3). Epub 2021 Jan 26.

Department of Renal Medicine, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, Fujian 362000, P.R. China.

Renal interstitial fibrosis is one of the common causes, and a major pathological basis for the development of various types of chronic progressive renal to end‑stage renal diseases. Therefore, it is important to clarify the underlying mechanisms of disease progression in order to develop effective strategies for the treatment and prevention of these pathologies. The aim of the present study was to investigate the association between microRNA (miR)‑212 expression and the development of renal interstitial fibrosis, as well as analyzing the role of miR‑212 in the disease. Read More

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Transcatheter Aortic Valve Implantation in Patients with Connective Tissue Disease.

Acta Cardiol Sin 2021 Jan;37(1):38-46

Department of Cardiology, Medicana International Ankara Hospital, Ankara, Turkey.

Background: There is still no consensus on the treatment of patients with connective tissue disease (CTD) with severe symptomatic aortic stenosis (AS). The aim of this study was to evaluate the feasibility and safety of transcatheter aortic valve implantation (TAVI) in patients with CTD.

Methods: Five hundred and fifty consecutive symptomatic severe AS patients who underwent TAVI between 2011 and 2019 were included in this retrospective study, of whom 14 had CTD. Read More

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January 2021

Membranous Nephropathy: Core Curriculum 2021.

Am J Kidney Dis 2021 03 21;77(3):440-453. Epub 2021 Jan 21.

Section of Nephrology, Department of Medicine, Boston Medical Center, Boston, MA; Section of Nephrology, Department of Medicine, Boston University School of Medicine, Boston, MA. Electronic address:

The understanding and management of membranous nephropathy, a common cause of nephrotic syndrome that is more frequently encountered in adults than in children, has rapidly evolved over the past decade. Identification of target antigens has allowed for more precise molecular diagnoses, and the ability to monitor circulating autoantibodies has added a new vantage point in terms of disease monitoring and decisions about immunosuppression. Although immunosuppression with alkylating agents combined with corticosteroids, or with calcineurin inhibitor-based regimens, has been the historical mainstay of treatment, observational and now randomized controlled trials with the B-cell-depleting agent rituximab have moved this agent to the forefront of therapy for primary membranous nephropathy. Read More

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Family History of Pulmonary Fibrosis Predicts Worse Survival in Patients With Interstitial Lung Disease.

Chest 2021 May 21;159(5):1913-1921. Epub 2021 Jan 21.

Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX.

Background: A number of genetic markers linked to familial pulmonary fibrosis predict differential survival in interstitial lung disease (ILD) patients. Although genetic testing is not performed routinely for ILD, family history commonly is obtained and may inform outcome risk.

Research Question: Does survival vary between patients with and without self-reported familial pulmonary fibrosis?

Methods: Family history was acquired systematically for consecutive ILD patients who consented to clinical registry enrollment at the University of Texas Southwestern and the University of California at Davis. Read More

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Malignancy in giant cell tumor of bone: analysis of an open-label phase 2 study of denosumab.

BMC Cancer 2021 Jan 22;21(1):89. Epub 2021 Jan 22.

Chemotherapy Unit, IRCCS Istituto Ortopedico Rizzoli, Department of Experimental, Diagnostic and Specialty Medicine (DIMES), Bologna University, 40136, Bologna, Italy.

Background: Giant cell tumor of bone (GCTB) is a rare osteoclastogenic stromal tumor. GCTB can rarely undergo malignant transformation. This post hoc analysis evaluated and classified malignancies in patients with GCTB who received denosumab. Read More

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January 2021

Why is a paediatric respiratory specialist integral to the paediatric rheumatology clinic?

Breathe (Sheff) 2020 Dec;16(4):200212

Dept of Paediatric Respiratory Medicine, Nottingham Children's Hospital, Nottingham University Hospitals, Nottingham, UK.

Systemic connective tissue diseases (CTDs) are characterised by the presence of autoantibodies and multiorgan involvement. Although CTDs are rare in children, they are associated with pulmonary complications, which have a high morbidity and mortality rate. The exact pathophysiology remains unclear. Read More

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December 2020

Clinical perspective and practices on pleural effusions in chronic systemic inflammatory diseases.

Breathe (Sheff) 2020 Dec;16(4):200203

Oxford Centre for Respiratory Medicine, Churchill Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.

Systemic inflammatory diseases are a heterogeneous family of autoimmune chronic inflammatory disorders that affect multiple systems within the human body. Connective tissue disease (CTD) is a large group within this family characterised by immune-mediated inflammation of the connective tissue. This group of disorders are often associated with pleural manifestations. Read More

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December 2020