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    Chylous ascites in a patient with an overlap syndrome: a surprising response to rituximab.
    BMJ Case Rep 2017 Oct 20;2017. Epub 2017 Oct 20.
    Leeds Biomedical Research Centre, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
    The authors present a case of a 51-year-old woman with clinical diagnosis of mixed connective tissue disease and overlap systemic lupus erythematosus features, with a 6-month history of progressive painless abdominal distension. On examination, evident signs of ascites were present. Both the abdominal-pelvic ultrasound and CT scan confirmed a large amount of ascites. Read More

    Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity.
    J Investig Med High Impact Case Rep 2017 Oct-Dec;5(4):2324709617734012. Epub 2017 Oct 10.
    Eastern Virginia Medical School, Norfolk, VA, USA.
    Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the renal outcome is usually poor. Read More

    How can we effectively address the paraneoplastic dermatomyositis: Diagnosis, risk factors and treatment options.
    J BUON 2017 Jul-Aug;22(4):1073-1080
    University of Ioannina, School of Health Sciences, Faculty of Medicine, Ioannina, Greece.
    Purpose: Dermatomyositis (DM) represents an auto-immune inflammatory myopathy. In this review, we analyzed the incidence of DM as a clinical manifestation highlighting the peculiar clinical and treatment characteristics of this disease when occurring in the context of different malignancies.

    Methods: A systematic literature review was performed based on database search in PubMed/Medline and included English articles until December 2016. Read More

    Retinal Vasculitis and Choroidopathy in Pediatric-Onset Mixed Connective Tissue Disease.
    J Clin Rheumatol 2017 Oct;23(7):400-401
    Division of Rheumatology Department of Medicine College of Medicine University of Tennessee Health Science Center, Memphis, TN Department of Ophthalmology University of Tennessee Health Science Center, Memphis, TN Division of Rheumatology Department of Medicine College of Medicine University of Tennessee Health Science Center, Memphis, TN Division of Rheumatology Department of Medicine and Department of Pediatrics College of Medicine University of Tennessee Health Science Center, Memphis, TN Department of Otolaryngotology University of Tennessee Health Science Center, Memphis, TN Department of Ophthalmology University of Tennessee Health Science Center, Memphis, TN Division of Rheumatology Department of Medicine College of Medicine University of Tennessee Health Science Center, Memphis, TN Division of Clinical Immunology and Rheumatology, Department of Pediatrics University of Tennessee Health Science Center, Memphis, TN Division of Clinical Immunology and Rheumatology, Department of Pediatrics University of Tennessee Health Science Center, Memphis, TN

    [Mixed connective tissue disease: prevalence and clinical characteristics in African black, study of 7 cases in Gabon and review of the literature].
    Pan Afr Med J 2017 30;27:162. Epub 2017 Jun 30.
    Département de Médecine Interne et Spécialités Médicales, Université des Sciences de la Santé, BP 4009 Libreville Gabon.
    The literature reports that mixed connective tissue disease seems more frequent in the black population and among Asians. This study aims to determine the prevalence of mixed connective tissue disease (MCTD) among connective tissue disorders and all rheumatologic pathologies in a hospital population in Gabon as well as to describe the clinical features of this disease. We conducted a retrospective study by reviewing the medical records of patients treated for mixed connective tissue disease (Kasukawa criteria) and other entities of connective tissue disorders (ACR criteria) in the Division of Rheumatology at the University Hospital in Libreville between January 2010 and December 2015. Read More

    Predictors of Favorable Responses to Immunosuppressive Treatment in Pulmonary Arterial Hypertension Associated With Connective Tissue Disease.
    Circ J 2017 Sep 12. Epub 2017 Sep 12.
    Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine.
    Background: The potential efficacy of immunosuppressive (IS) treatment has been reported in patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD), but its positioning in the treatment algorithm remains uncertain. The aim of this study was to identify predictors of favorable responses to first-line IS treatment.Methods and Results:This single-center retrospective study included 30 patients with PAH accompanied by systemic lupus erythematosus (SLE), mixed CTD (MCTD), or primary Sjögren's syndrome (SS) who received first-line IS treatment alone or in combination with pulmonary vasodilators. Read More

    [Manifestations of the connective tissue associated interstitial lung disease under high resolution computed tomography].
    Zhong Nan Da Xue Xue Bao Yi Xue Ban 2017 Aug;42(8):934-939
    Department of Radiology, Xiangya Hospital, Central South University, Changsha 410008, China.
    Objective: To analyze the features of the connective tissue associated interstitial lung disease (CTD-ILD) by high resolution computed tomography (HRCT).
 Methods: A total of 127 patients with CTD-ILD, who were diagnosed by clinic laboratory examination and pathology in Xiangya Hospital of Central South University form September 2013 to September 2015, were enrolled for this study. Their lung features of HRCT imaging were retrospectively analyzed. Read More

    Intravenous immunoglobulins for treatment of connective tissue diseases in dermatology.
    Wien Med Wochenschr 2017 Sep 1. Epub 2017 Sep 1.
    Department of Dermatology and Venereology, Medical University of Sofia, 1st, 1431, Sofia, Bulgaria.
    Background: Connective tissue diseases are a heterogeneous group of autoimmune disorders affecting not only skin, but also various organs and systems. First-line treatment of connective tissue diseases is systemic steroids as monotherapy or combined with immunosuppressive drugs. Since intravenous immunoglobulins (IVIGs) have been found to be effective for various autoimmune dermatoses, their indications have expanded tremendously. Read More

    Facts and controversies in mixed connective tissue disease.
    Med Clin (Barc) 2017 Aug 29. Epub 2017 Aug 29.
    Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España; Instituto de Investigación Biomédica Hospital Gregorio Marañón, Madrid, España; Universidad Complutense de Madrid, Madrid, España.
    Mixed connective tissue disease (MCTD) is a systemic autoimmune rheumatic disease (SARD) characterised by the combination of clinical manifestations of systemic lupus erythematosus (SLE), cutaneous systemic sclerosis (SSc) and polymyositis-dermatomyositis, in the presence of elevated titers of anti-U1-RNP antibodies. Main symptoms of the disease are polyarthritis, hand oedema, Raynaud's phenomenon, sclerodactyly, myositis and oesophageal hypomobility. Although widely discussed, most authors today accept MCTD as an independent entity. Read More

    Imaging the emergence and natural progression of spontaneous autoimmune diabetes.
    Proc Natl Acad Sci U S A 2017 Sep 24;114(37):E7776-E7785. Epub 2017 Aug 24.
    Division of Immunology, Department of Microbiology and Immunobiology, Harvard Medical School, Boston, MA 02115;
    Type 1 diabetes in the nonobese diabetic mouse stems from an infiltration of the pancreatic islets by a mixed population of immunocytes, which results in the impairment and eventual destruction of insulin-producing β-cells. Little is known about the dynamics of lymphocyte movement in the pancreas during disease progression. Using advanced intravital imaging approaches and newly created reporter mice (Flt3-BFP2, Mertk-GFP-DTR, Cd4-tdTomato, Cd8a-tdTomato), we show that the autoimmune process initiates first with a T cell infiltration into the islets, where they have restricted mobility but reside and are activated in apposition to CX3CR1(+) macrophages. Read More

    The Microbiome in Connective Tissue Diseases and Vasculitides: An Updated Narrative Review.
    J Immunol Res 2017 1;2017:6836498. Epub 2017 Aug 1.
    Department of Rheumatology, ASST Fatebenefratelli-Sacco, Milan, Italy.
    Objective: To provide a narrative review of the most recent data concerning the involvement of the microbiome in the pathogenesis of connective tissue diseases (CTDs) and vasculitides.

    Methods: The PubMed database was searched for articles using combinations of words or terms that included systemic lupus erythematosus, systemic sclerosis, autoimmune myositis, Sjögren's syndrome, undifferentiated and mixed CTD, vasculitis, microbiota, microbiome, and dysbiosis. Papers from the reference lists of the articles and book chapters were reviewed, and relevant publications were identified. Read More

    Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension.
    Eur Respir J 2017 Aug 17;50(2). Epub 2017 Aug 17.
    Dept of Respiratory Medicine, Hannover Medical School and German Centre for Lung Research, Hannover, Germany.
    Patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD) have a poor prognosis compared with other aetiologies. The underlying CTD can influence treatment response and outcomes. We characterised the GRIPHON study PAH-CTD subgroup and evaluated response to selexipag. Read More

    Regulatory T cell deficient scurfy mice exhibit a Th2/M2-like inflammatory response in the skin.
    J Dermatol Sci 2017 Sep 13;87(3):285-291. Epub 2017 Jul 13.
    Department of Dermatology, University of Heidelberg, Im Neuenheimer Feld 440, 69120 Heidelberg, Germany. Electronic address:
    Background: Scurfy mice have a functional defect in regulatory T cells (Treg), which leads to lethal multi-organ inflammation. The missing Treg function results in uncontrolled autoimmune cellular and humoral inflammatory responses. We and others have previously shown that during the course of disease scurfy mice develop severe skin inflammation and autoantibodies including anti-nuclear autoantibodies (ANA). Read More

    Protein-losing Gastroenteropathy Related to Mixed Connective Tissue Disease: A Case Report of a Successful Outcome and Literature Review.
    Intern Med 2017 1;56(15):2057-2062. Epub 2017 Aug 1.
    Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Japan.
    We herein report the case of a 44-year-old woman who developed protein-losing gastroenteropathy (PLGE) with hypoalbuminemia as the first manifestation of mixed connective tissue disease (MCTD). Albumin leakage from the stomach and intestinal tract was demonstrated by (99m)Tc-labeled human serum albumin scintigraphy. The patient's response to prednisolone therapy was insufficient; therefore, additional cyclosporin A (CsA) treatment was administered, and clinical remission was achieved. Read More

    Comparisons of neutrophil-, monocyte-, eosinophil-, and basophil- lymphocyte ratios among various systemic autoimmune rheumatic diseases.
    APMIS 2017 Oct 2;125(10):863-871. Epub 2017 Aug 2.
    Department of Laboratory Diagnostics, Changzheng Hospital, Second Military Medical University, Shanghai, China.
    This study was aimed to evaluate levels of neutrophil- (NLR), monocyte- (MLR), eosinophil- (ELR), and basophil-lymphocyte ratio (BLR) and their association with inflammatory markers in systemic autoimmune rheumatic diseases (SARDs). A total of 1139 SARD patients and 170 healthy individuals were enrolled. Clinical and laboratory data were extracted. Read More

    An unusual association between hemophagocytic lymphohistiocytosis, mixed connective tissue disease, and autoimmune hemolytic anemia: A case report.
    Medicine (Baltimore) 2017 Jul;96(28):e7488
    aDepartment of Internal Medicine bDepartment of Pathology, University of Illinois College of Medicine at Peoria, Peoria cDepartment of Gastroenterology, University of Illinois College of Medicine at Chicago, Chicago, IL.
    Rationale: In the adult patient, hemophagocytic lymphohistiocytosis (HLH) is uncommon and frequently difficult to diagnose due to its nonspecific presentation and numerous complications.

    Patient Concerns: Herein, we present the case of a 25-year-old female who initially presented for evaluation of persistent fevers and fatigue. She was found to have splenomegaly, generalized lymphadenopathy, pancytopenia, and acute hepatic failure. Read More

    Autoamputation and Polyneuropathy in Mixed Connective Tissue Disorder: A Case Report.
    Cureus 2017 Jun 5;9(6):e1313. Epub 2017 Jun 5.
    Department of Medicine, Jinnah Postgraduate Medical Center Karachi Pakistan.
    Mixed connective tissue disorder (MCTD) is a multisystem disease with overlapping features of other autoimmune diseases, such as systemic lupus erythematosus (SLE), myositis, rheumatoid arthritis, and scleroderma. MCTD presents with a distinctive antibody in serum known as U1-ribonucleoprotein (RNP). MCTD is quite rare as compared to other connective tissue disorders like SLE, systemic sclerosis, dermatomyositis, and polymyositis. Read More

    Prevalence of anti-NT5C1A antibodies in Japanese patients with autoimmune rheumatic diseases in comparison with other patient cohorts.
    Clin Chim Acta 2017 Sep 4;472:1-4. Epub 2017 Jul 4.
    Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
    Background: Sporadic inclusion body myositis (sIBM) is usually classified as an idiopathic inflammatory myopathies. Although the diagnosis of sIBM is sometimes challenging, recent studies have shown that the autoantibodies against cytosolic 5'-nucleotidase 1A (NT5C1A) are the possible diagnostic biomarker for sIBM. Few reports have shown the frequencies of anti-NT5C1A antibodies in systemic autoimmune rheumatic diseases (SARDs) using large cohorts of SARDs. Read More

    Rituximab versus cyclophosphamide for the treatment of connective tissue disease-associated interstitial lung disease (RECITAL): study protocol for a randomised controlled trial.
    Trials 2017 Jun 15;18(1):275. Epub 2017 Jun 15.
    NIHR Biomedical Research Unit, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK.
    Background: Interstitial lung disease (ILD) frequently complicates systemic autoimmune disorders resulting in considerable morbidity and mortality. The connective tissue diseases (CTDs) most frequently resulting in ILD include: systemic sclerosis, idiopathic inflammatory myositis (including dermatomyositis, polymyositis and anti-synthetase syndrome) and mixed connective tissue disease. Despite the development, over the last two decades, of a range of biological therapies which have resulted in significant improvements in the treatment of the systemic manifestations of CTD, the management of CTD-associated ILD has changed little. Read More

    Phosphaturic Mesenchymal Tumors: Clinicopathologic, Immunohistochemical and Molecular Analysis of 22 Cases Expanding their Morphologic and Immunophenotypic Spectrum.
    Am J Surg Pathol 2017 Oct;41(10):1371-1380
    *Institute of Pathology ††Department of Hand & Plastic Surgery, University Hospital, Erlangen **Institute of Pathology ‡‡Department of Orthopedic & Traumatology, Section for Tumor Orthopedics, University Hospital, Bonn, Germany †Department of Pathology, Faculty of Medicine in Pilsen, Charles University, Prague ‡Biomedical Center of the Faculty of Medicine in Pilsen, Pilsen, Czech Republic §Department of Pathology, University of Pittsburgh Medical Center, Presbyterian Hospital, Pittsburgh, PA ∥Department of Pathology, National University Health System, Singapore ¶Department of Pathology, 3rd Medical Faculty in Prague, Charles University, Prague, Czech Republic.
    Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm of uncertain histogenesis that has been linked to tumor-induced osteomalacia (TIO) since 1959. The neoplastic cells produce increased amount of FGF23 which results in TIO via uncontrolled renal loss of phosphate (phosphaturia), and consequently diminished bone mineralization. To date, ∼300 cases have been reported. Read More

    Pulmonary-Limited Granulomatosis with Polyangiitis Coexisting with Mixed Connective Tissue Disease.
    Tohoku J Exp Med 2017 06;242(2):109-114
    Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine.
    Granulomatosis with polyangiitis (GPA) is a systemic disease characterized by necrotizing, granulomatous vasculitis of the upper and lower respiratory tracts and glomerulonephritis, and is classified as a classical or limited form. The classical form of GPA demonstrates the involvement of the upper respiratory tract, sinuses, lungs and kidneys, whereas the limited form is characterized by the lack of the renal involvement with female predominance. On the other hand, mixed connective tissue disease (MCTD) shows the clinical and laboratorial features of systemic lupus erythematosus, systemic sclerosis and polymyositis, along with high titers of anti-ribonucleoprotein antibodies and is characterized by good response to corticosteroid therapy and favorable prognosis. Read More

    Antiribonuclease H2 antibodies are an immune biomarker for systemic lupus erythematosus.
    Autoimmunity 2017 Jun 27;50(4):241-246. Epub 2017 May 27.
    a Department of Rheumatology , Juntendo University School of Medicine , Tokyo , Japan.
    We previously reported that autoantibodies against the proliferating cell nuclear antigen protein (PCNA)-binding protein chromatin assembly factor-1 (CAF-1) are specifically found in patients with systemic lupus erythematosus (SLE). PCNA and its complex constituents elicit autoimmune responses in patients with SLE, suggesting that autoantibody diversification likely occurs owing to epitope spreading. Therefore, we sought to clarify whether patients with SLE exhibit an autoimmune response to Ribonuclease H2 (RNase H2), another PCNA-binding protein that regulates cell division. Read More

    Cardiovascular magnetic resonance imaging: clinical implications in the evaluation of connective tissue diseases.
    J Inflamm Res 2017 11;10:55-61. Epub 2017 May 11.
    Onassis Cardiac Surgery Center, Athens, Greece.
    Cardiovascular magnetic resonance imaging is a recently developed noninvasive, nonradiating, operator-independent technique that has been successfully used for the evaluation of congenital heart disease, valvular and pericardial diseases, iron overload, cardiomyopathies, great and coronary vessel diseases, cardiac inflammation, stress-rest myocardial perfusion, and fibrosis. Rheumatoid arthritis and other spondyloarthropathies, systemic lupus erythematosus, inflammatory myopathies, mixed connective tissue diseases (CTDs), systemic sclerosis, vasculitis, and sarcoidosis are among CTDs with serious cardiovascular involvement; this is due to multiple causative factors such as myopericarditis, micro/macrovascular disease, coronary artery disease, myocardial fibrosis, pulmonary hypertension, and finally heart failure. The complicated pathophysiology and the high cardiovascular morbidity and mortality of CTDs demand a versatile, noninvasive, nonradiative diagnostic tool for early cardiovascular diagnosis, risk stratification, and treatment follow-up. Read More

    Cochlear Impairment and Autoimmune Ear Disorder in a Patient with Breast Cancer.
    Audiol Res 2017 Feb 13;7(1):165. Epub 2017 Mar 13.
    Tinnitus Center, European Hospital, Rome.
    The purpose of this study was to consider the possible role of autoimmune diseases and paraneoplastic syndrome in the genesis of tinnitus. The incidence of autoimmune inner ear disease (AIED) is rare, accounting for <1% of all cases of hearing impairment and dizziness. In presence of auditory and vestibular deficit in oncological patients, a paraneoplastic syndrome with cochleovestibulopathy should be considered. Read More

    Evaluation of Autoantibodies in Patients with Primary and Secondary Sjogren's Syndrome.
    Open Rheumatol J 2017 31;11:10-15. Epub 2017 Jan 31.
    Immco Diagnostics / Trinity Biotech, Buffalo, NY, USA; Department of Oral Diagnostic Sciences, SUNY at Buffalo School of Dental Medicine, Buffalo, NY, USA.
    Background: Antibodies to salivary gland protein 1 (SP1), carbonic anhydrase 6 (CA6) and parotid secretory protein (PSP) were discovered in an animal model of Sjogren's syndrome (SS). Their expression was noted in patients with SS, especially those with lower focus scores on lip biopsies and those with early disease lacking antibodies to Ro and La.

    Objective: The current studies evaluated these autoantibodies in patients with long-standing SS expressing high levels of anti-Ro antibodies and in patients with Sjogren's syndrome secondary to systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). Read More

    Progression and mortality of interstitial lung disease in mixed connective tissue disease: a long-term observational nationwide cohort study.
    Rheumatology (Oxford) 2017 Mar 29. Epub 2017 Mar 29.
    Institute of Clinical Medicine, University of Oslo, Norway.
    Objectives.: To assess the prevalence, extent, progression, functional impact and mortality of interstitial lung disease (ILD) in a nationwide unselected MCTD cohort.

    Methods. Read More

    Etiological profile of noncompressive myelopathies in a tertiary care hospital of Northeast India.
    Ann Indian Acad Neurol 2017 Jan-Mar;20(1):41-50
    Department of Neurology, Gauhati Medical College and Hospital, Guwahati, Assam, India.
    Background: The discovery of antibodies against aquaporin-4 and evolving concepts of noncompressive myelopathies in the 21(st) century have made a major impact on the etiological profile of these diseases, with few cases turning out to be idiopathic.

    Objective: To find causes of noncompressive myelopathy in a tertiary care hospital of Northeast India.

    Materials And Methods: An observational study was carried out in the Neurology Department of Gauhati Medical College, Guwahati, from September 2013 to February 2016. Read More

    Presentation of calcinosis cutis universalis in mixed connective tissue disorder: an encounter during hip arthroplasty.
    BMJ Case Rep 2017 Mar 13;2017. Epub 2017 Mar 13.
    Department of Orthopedics, KG Hospital, Coimbatore, Tamil Nadu, India.
    A woman aged 23 years with a diagnosis of mixed connective tissue disorder presented with left groin pain extending over 6 months. Workup revealed avascular necrosis of the femoral head (Grade 3) secondary to systemic lupus erythematosus and chronic steroid intake. An uncemented total hip arthroplasty was considered as the patient was only in the third decade of life. Read More

    A 42-Year-Old Woman With Anemia, Shock, and Ischemic Stroke After Lung Transplantation.
    Chest 2017 Mar;151(3):e63-e68
    Division of Pulmonary Critical Care and Sleep Medicine, University of Florida, Gainesville, FL. Electronic address:
    Case Presentaion: A 42-year-old woman with mixed connective tissue disease-associated interstitial lung disease underwent bilateral lung transplantation. She had an uneventful surgery and was extubated 3 h later. Induction immunosuppression therapy included methylprednisolone 500 mg intraoperatively, basiliximab (anti-IL-2 monoclonal antibody) on days 0 and 4 after transplantation, and methylprednisolone 125 mg intravenously bid for 2 days following surgery. Read More

    Serum KL-6 and surfactant protein-D as monitoring and predictive markers of interstitial lung disease in patients with systemic sclerosis and mixed connective tissue disease.
    J Thorac Dis 2017 Feb;9(2):362-371
    Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
    Background: Interstitial lung disease (ILD) is frequent complication of systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The disease is heterogeneous, and its outcome is unpredictable. Some patients have severe and progressive deterioration of ILD, which is the leading cause of mortality. Read More

    Evaluation of an automated connective tissue disease screening assay in Korean patients with systemic rheumatic diseases.
    PLoS One 2017 8;12(3):e0173597. Epub 2017 Mar 8.
    Department of Laboratory Medicine, Kosin University College of Medicine, Busan, Republic of Korea.
    This study aimed to evaluate the diagnostic utilities of the automated connective tissues disease screening assay, CTD screen, in patients with systemic rheumatic diseases. A total of 1093 serum samples were assayed using CTD screen and indirect immunofluorescent (IIF) methods. Among them, 162 were diagnosed with systemic rheumatic disease, including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and mixed connective tissue disease (MCT). Read More

    The clinical and pathological characteristics of nephropathies in connective tissue diseases in the Japan Renal Biopsy Registry (J-RBR).
    Clin Exp Nephrol 2017 Dec 2;21(6):1024-1029. Epub 2017 Mar 2.
    Division of Nephrology, Kanazawa Medical University School of Medicine, Uchinada, Japan.
    Background: In connective tissue diseases, a wide variety of glomerular, tubulointerstitial, and vascular lesions of the kidney are observed. Nonetheless, recent information is limited regarding renal lesions in connective tissue diseases, except in systemic lupus erythematosus (SLE).

    Methods: In this study, we used a nationwide database of biopsy-confirmed renal diseases in Japan (J-RBR) (UMIN000000618). Read More

    Successful endoscopic fragmentation of large hardened fecaloma using jumbo forceps.
    World J Gastrointest Endosc 2017 Feb;9(2):91-94
    Yasumasa Matsuo, Hiroshi Yasuda, Hiroyasu Nakano, Miki Hattori, Midori Ozawa, Yoshinori Sato, Yoshiko Ikeda, Shun-Ichiro Ozawa, Masaki Yamashita, Hiroyuki Yamamoto, Fumio Itoh, Division of Gastroenterology and Hepatology, Department of Internal Medicine, St. Marianna University School of Medicine, Kanagawa 216-8511, Japan.
    We present a rare case of fecaloma, 7 cm in size, in the setting of systemic scleroderma. A colonoscopy revealed a giant brown fecaloma occupying the lumen of the colon and a colonic ulcer that was caused by the fecaloma. The surface of the fecaloma was hard, large and slippery, and fragmentation was not possible despite the use of various devices, including standard biopsy forceps, an injection needle, and a snare. Read More

    Mixed Connective Tissue Disease and Epitope Spreading: An Historical Cohort Study.
    J Clin Rheumatol 2017 Apr;23(3):155-159
    From the *Internal Medicine and †Immunology Departments, Vall d'Hebron General Hospital, Universitat Autonoma de Barcelona, Barcelona, Spain.
    Objectives: Mixed connective tissue disease (MCTD) is characterized by the presence of anti-U1-snRNP autoantibodies and a variable set of associated clinical features. Some MCTD patients test positive over time to autoantibodies against Sm, proteins spatially related with U1-snRNP. This situation has been attributed to expanding of the autoimmune response by a phenomenon known as epitope spreading. Read More

    The diagnostic value of the bronchoalveolar lavage in interstitial lung diseases.
    J Negat Results Biomed 2017 Mar 1;16(1). Epub 2017 Mar 1.
    Department of Pathology, Hassan II Teaching Hospital, (B.P. 1835, Atlas) Route de sidi Harazem Fès - Maroc, Fez, Morocco.
    Objective: Bronchoalveolar lavage (BAL) is a diagnostic tool often used during the management of interstitial lung diseases (ILD). However, its diagnostic value in discrimination between entities comprising the very heterogenous group of ILD, is still a controversial issue. The objective of our study is to assess the diagnostic value of BAL in the management of ILD, by comparing the cytological findings in BAL fluid among the different diseases of this group. Read More

    Vasculitic and autoimmune wounds.
    J Vasc Surg Venous Lymphat Disord 2017 Mar 14;5(2):280-292. Epub 2016 Dec 14.
    Division of Rheumatology, The George Washington University, School of Medicine and Health Sciences, Washington, D.C.
    Objective: Chronic wounds are a major cause of morbidity and mortality. Approximately 20% to 23% of nonhealing wounds that are refractory to vascular intervention have other causes, including vasculitis, pyoderma gangrenosum, and other autoimmune diseases. The purpose of this article was to review the literature across medical and surgical specialties with regard to refractory chronic wounds associated with vasculitis and autoimmune diseases and to delineate clinical outcomes of these wounds in response to vascular and other interventions. Read More

    Pulmonary hypertension in connective tissue diseases: an update.
    Int J Rheum Dis 2017 Jan 16;20(1):5-24. Epub 2017 Feb 16.
    Department ofClinical Immunology & Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India.
    Pulmonary hypertension (PH) is a relatively commoner complication of systemic sclerosis (SSc) with estimated prevalence ranging between 8% and 12% as compared to much lower figures in other connective tissue diseases (CTD). It is a major cause of morbidity and mortality in CTDs. PH is classified into five major groups. Read More

    Treatment of Vasodilator-resistant Mixed Connective Tissue Disease-associated Pulmonary Arterial Hypertension with Glucocorticoid and Cyclophosphamide.
    Intern Med 2017 15;56(4):445-448. Epub 2017 Feb 15.
    Division of Rheumatology, Endocrinology and Nephrology, Hokkaido University Graduate School of Medicine, Japan.
    Pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MTCD), in contrast to other types of PAH, may respond to immunosuppressive therapy. Most PAH cases with an immunosuppressant response were in the early stages of the disease (WHO functional class III or less). The present case was a 34-year-old woman with MCTD-associated PAH (WHO functional class IV) who was resistant to a combination of three vasodilators. Read More

    Cardiovascular magnetic resonance imaging pattern at the time of diagnosis of treatment naïve patients with connective tissue diseases.
    Int J Cardiol 2017 Jun 1;236:151-156. Epub 2017 Feb 1.
    Onassis Cardiac Surgery Center, Athens, Greece.
    Background-aim: Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis.

    Patients-methods: CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]. Read More

    Survival and outcomes after lung transplantation for non-scleroderma connective tissue-related interstitial lung disease.
    J Heart Lung Transplant 2017 Jul 31;36(7):763-769. Epub 2016 Dec 31.
    Division of Pulmonary and Critical Care, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania; Division of Rheumatology, Brigham and Women's Hospital, Boston, Massachusetts. Electronic address:
    Background: Patients with non-scleroderma connective tissue-related lung disease (NS-CTLD), including rheumatoid arthritis, idiopathic inflammatory myopathies, Sjögren syndrome, mixed connective tissue disease, and systemic lupus erythematosus, may be at risk for worse outcomes after lung transplantation because of immune dysregulation or extrapulmonary manifestations of their underlying disease. We compared survival, acute and chronic rejection, and extrapulmonary organ dysfunction after transplantation in patients with NS-CTLD and idiopathic pulmonary fibrosis (IPF).

    Methods: This was a retrospective cohort study of patients with NS-CTLD and IPF who were listed in the Scientific Registry of Transplant Recipients and underwent lung transplantation from May 5, 2005, to March 1, 2016. Read More

    Hypercalcemia due to Primary Hepatic Lymphoma.
    Case Rep Hematol 2016 25;2016:1876901. Epub 2016 Dec 25.
    Department of Hematology and Oncology, University of Massachusetts Medical School, Worcester, MA, USA.
    A 65-year-old female with a history of mixed connective tissue disease and pulmonary fibrosis on azathioprine, hydroxychloroquine, and prednisone (osteoporosis on teriparatide) presented with a 1-month history of hypercalcemia. After discontinuation of teriparatide, the patient's hypercalcemia persisted. Further evaluation revealed primary hepatic lymphoma as the source of her hypercalcemia. Read More

    Successful treatment of type B insulin resistance with mixed connective tissue disease by pulse glucocorticoids and cyclophosphamide.
    J Diabetes Investig 2017 Jul 25;8(4):626-628. Epub 2017 Apr 25.
    Departments of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
    Type B insulin resistance syndrome is a very rare condition caused by autoantibodies against the insulin receptor. We report the successful treatment of a patient with refractory type B insulin resistance with pulse glucocorticoids and cyclophosphamide. The medical record of a patient with type B insulin resistance was reviewed. Read More

    Mixed Connective Tissue Disorder Complicated by Polymyositis, Sjogren's Syndrome, Pleural Effusion and Pericarditis.
    Cureus 2016 Dec 1;8(12):e906. Epub 2016 Dec 1.
    Department of Internal Medicine, Carthage Area Hospital, New York, USA.
    We report a case of a 24-year-old female with a history of asthma and gastroesophageal reflux disease (GERD). She presented to the emergency room with severe chest pain, chest tightness, and shortness of breath following an upper respiratory tract infection. The patient reported that she had a cough and runny nose one week prior to this presentation, followed by a sudden sharp pain in the center of the chest 8/10 in intensity on the visual analog scale and pleuritic in nature, which aggravated by deep breathing and lying down flat. Read More

    Intravenous Immunoglobulin for Mixed Connective Tissue Disease Presenting With Bilateral Trigeminal Neuropathy.
    Am J Ther 2016 Dec 26. Epub 2016 Dec 26.
    1Division of Rheumatology, Yale University, New Haven, CT 2Division of Rheumatology, University of Nebraska Medical Center, Omaha, NE 3Department of Neurological Sciences, University of Nebraska Medical Center, Omaha, NE.

    Physical and cultural determinants of postpartum pelvic floor support and symptoms following vaginal delivery: a protocol for a mixed-methods prospective cohort study.
    BMJ Open 2017 Jan 10;7(1):e014252. Epub 2017 Jan 10.
    Department of Obstetrics and Gynecology, School of Medicine, University of Utah, Salt Lake City, Utah, USA.
    Introduction: Pelvic floor disorders (PFDs), including pelvic organ prolapse (POP), stress and urgency urinary incontinence, and faecal incontinence, are common and arise from loss of pelvic support. Although severe disease often does not occur until women become older, pregnancy and childbirth are major risk factors for PFDs, especially POP. We understand little about modifiable factors that impact pelvic floor function recovery after vaginal birth. Read More

    Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial.
    Ann Rheum Dis 2017 Jul 30;76(7):1219-1227. Epub 2016 Dec 30.
    University of California at San Diego, La Jolla, California, USA.
    Background: Patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH), in particular systemic sclerosis (SSc), had an attenuated response compared with idiopathic PAH in most trials. Thus, there is uncertainty regarding the benefit of PAH-targeted therapy in some forms of CTD-PAH.

    Objective: To explore the safety and efficacy of initial combination therapy with ambrisentan and tadalafil versus ambrisentan or tadalafil monotherapy in patients with CTD-PAH and SSc-PAH enrolled in the AMBITION trial. Read More

    Adhesive arachnoiditis in mixed connective tissue disease: a rare neurological manifestation.
    BMJ Case Rep 2016 Dec 16;2016. Epub 2016 Dec 16.
    Rheumatology Department, University Hospital Limerick, Limerick, Ireland.
    The overall incidence of neurological manifestations is relatively low among patients with mixed connective tissue disease (MCTD). We recently encountered a case of autoimmune adhesive arachnoiditis in a young woman with 7 years history of MCTD who presented with severe back pain and myeloradiculopathic symptoms of lower limbs. To the best of our knowledge, adhesive arachnoiditis in an MCTD patient has never been previously reported. Read More

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