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computed tomography scleroderma

Sara R Schoenfeld, Flavia V Castelino
Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are leading causes of morbidity and mortality in systemic sclerosis (SSc). As symptoms are often under-reported in SSc, early screening of ILD and PAH is of paramount importance, and early treatment may be associated with better clinical outcomes. Serologies are particularly helpful in identifying patients at risk for pulmonary involvement. Pulmonary function testing, high-resolution computed tomography of the chest and echocardiography are important tools in the initial screening of these patients...
August 2017: Therapeutic Advances in Respiratory Disease
Misbah Baqir, Ashima Makol, Thomas G Osborn, Brian J Bartholmai, Jay H Ryu
BACKGROUND AND OBJECTIVE: Interstitial lung disease (ILD) remains the number one cause of mortality in scleroderma (SSc). Our goal was to determine the effectiveness of mycophenolate mofetil (MMF) in treating SSc-ILD in a retrospective study. METHODS: A retrospective, computer-assisted search was performed to identify patients with SSc-ILD treated with MMF from 1997 through 2014. We used a novel software tool, Computer-Aided Lung Informatics for Pathology Evaluation and Rating (CALIPER), to quantify parenchymal lung abnormalities on high-resolution computed tomography...
2017: PloS One
Rasha A Abdel-Magied, Shereen R Kamel, Azza Farag Said, Hazem M Ali, Ehab A Abdel Gawad, Mahmoud M Moussa
OBJECTIVE: To assess serum interleukin-6 (IL-6)level in patients with systemic sclerosis (SSc) and its correlations with European Scleroderma Study Group activity score (EUSTAR), Scleroderma Assessment Questionnaire (SAQ), disability index and cardiopulmonary involvement. METHODS: Twenty SSc patients and 10 matched healthy controls were included. Serum IL-6 was measured in patients and controls. Disease activity, status,and disability were assessed.Cardiopulmonary involvement was evaluated by pulmonary function tests (PFTs), six minute walk test, echocardiography, and high resolution computed tomography (HRCT) of chest...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
Anna Lis-Święty, Ligia Brzezińska-Wcisło, Hubert Arasiewicz
INTRODUCTION: Localized scleroderma (LoS) of the face and head is often associated with neurological manifestations and/or imaging abnormalities in the central nervous system (CNS). CASE SERIES: We present an analysis of 20 cases of LoS affecting the face and head. The CNS symptoms and/or abnormalities in high-resolution computed tomography (HRCT) and/or magnetic resonance imaging (MRI) were observed in 12 patients (60%). In addition to the mild and unspecific disorders (e...
October 26, 2016: International Journal of Neuroscience
S Vemulapalli, L Cohen, V Hsu
OBJECTIVES: Left ventricular diastolic dysfunction (LVDD) is more common in systemic sclerosis (SSc) compared to the general population. Focal myocardial ischaemia and fibrosis may be important in its pathogenesis. LVDD is associated with increased mortality and little is known about the risk factors. Advanced SSc lung complications may accompany LVDD. METHOD: We conducted a cross-sectional study of 300 SSc outpatients with and without LVDD, seen between May 2012 and May 2014, and performed univariate and multivariate regression analyses to determine clinical factors associated with LVDD...
September 16, 2016: Scandinavian Journal of Rheumatology
Keisuke Kumada, Nobuo Murakami, Hideshi Okada, Izumi Toyoda, Shinji Ogura, Hiroshi Kondo, Atsuhiro Fukuda
BACKGROUND: A central venous catheter enables the measurement of hemodynamic variations, such as accurate central venous pressure; catheter malposition may induce potentially fatal complications. This case report describes a rare central venous catheter tip malposition in the right internal mammary artery. CASE PRESENTATION: A 56-year-old Japanese woman who presented with severe pneumonia secondary to scleroderma was treated under ventilator support because of acute respiratory failure...
September 13, 2016: Journal of Medical Case Reports
Manabu Hayama, Shingo Sato, Takayuki Shiroyama, Takuji Nishida, Takashi Nishihara, Norio Okamoto
A 68-year-old woman with interstitial lung disease related to dermatomyositis and systemic scleroderma was admitted to our hospital with fever and dyspnoea. Although the fever was reduced after antibiotic therapy, a left pneumothorax suddenly occurred on day 27 after admission. A continuous air leak persisted despite chest drainage with three tubes and repeated pleurodesis. Chest computed tomography (CT) images showed a cavitary lesion with a pinhole in the left upper division, which was suspected to be the affected lesion with the air leak...
July 2016: Respirology Case Reports
Maren Doelle, Keith E Linder, Janna Boche, Vidhya Jagannathan, Tosso Leeb, Monika Linek
BACKGROUND: Stiff skin syndrome and systemic or localized scleroderma are cutaneous disorders characterized by dermal fibrosis and present clinically with induration of the skin, with or without joint, internal organ or vascular involvement. OBJECTIVES: To provide clinical, histological and preliminary genetic analysis of two West Highland white terrier siblings presenting with indurated skin resembling stiff skin syndrome in humans. ANIMALS: Two client owned full sibling West Highland white terriers from two different litters...
June 2016: Veterinary Dermatology
Céline Tossier, Clairelyne Dupin, Laurent Plantier, Julie Leger, Thomas Flament, Olivier Favelle, Thierry Lecomte, Patrice Diot, Sylvain Marchand-Adam
Gastro-oesophageal reflux has long been suspected of implication in the genesis and progression of idiopathic pulmonary fibrosis (IPF). We hypothesised that hiatal hernia may be more frequent in IPF than in other interstitial lung disease (ILD), and that hiatal hernia may be associated with more severe clinical characteristics in IPF.We retrospectively compared the prevalence of hiatal hernia on computed tomographic (CT) scans in 79 patients with IPF and 103 patients with other ILD (17 scleroderma, 54 other connective tissue diseases and 32 chronic hypersensitivity pneumonitis)...
September 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
Claire Owen, Gene-Siew Ngian, Kathleen Elford, Owen Moore, Wendy Stevens, Mandana Nikpour, Candice Rabusa, Susanna Proudman, Janet Roddy, Jane Zochling, Catherine Hill, Allan Sturgess, Kathleen Tymms, Peter Youssef, Joanne Sahhar
OBJECTIVES: To report the efficacy and tolerability of mycophenolate mofetil (MMF) and azathioprine (AZA) in the management of systemic sclerosis-associated interstitial lung disease (SSc-ILD). METHODS: Patients in the Australian Scleroderma Cohort Study treated with at least 3 months of MMF or AZA for SSc-ILD confirmed on high resolution computed tomography (HRCT) chest were identified and their pulmonary function tests (PFTs) retrieved. Individuals with available results for T-1 (12 months prior to treatment commencement), T0 (date of treatment commencement) and at least one subsequent time point were included in the drug efficacy analysis...
September 2016: Clinical and Experimental Rheumatology
Carrie Richardson, Rishi Agrawal, Jungwha Lee, Orit Almagor, Ryan Nelson, John Varga, Michael J Cuttica, Jane D Amico Dematte, Rowland W Chang, Monique E Hinchcliff
OBJECTIVE: A patulous esophagus on high-resolution computed tomography (HRCT) of the thorax is frequently observed in patients with systemic sclerosis (SSc). Microaspiration has been purported to play a role in the development and progression of SSc interstitial lung disease (ILD), but studies examining the role of microaspiration in SSc ILD have yielded conflicting results. This study was conducted to determine the association between esophageal diameter and SSc ILD. METHODS: A cross-sectional study of Northwestern Scleroderma Registry patients with available HRCT exams was conducted...
August 2016: Seminars in Arthritis and Rheumatism
Susumu Saigusa, Yasuhiro Inoue, Masaki Ohi, Hiroki Imaoka, Ryo Uratani, Minako Kobayashi, Masato Kusunoki
A 78-year-old woman receiving treatment for limited systemic scleroderma (SSc) underwent high anterior resection and partial liver resections for rectosigmoid colon cancer with multiple liver metastases. A year after surgery, an abdominal computed tomography (CT) demonstrated suspicion for peritoneal dissemination with an increase in ascites, and (18)F-fluorodeoxy glucose-positron emission tomography-CT was suggestive of carcinomatosis. We began to decompress the small intestine and administer octreotide. However, the intestinal obstruction did not improve...
December 2015: Surgical Case Reports
Maria De Santis, Angela Ceribelli, Francesca Cavaciocchi, Chiara Crotti, Marco Massarotti, Laura Belloli, Bianca Marasini, Natasa Isailovic, Elena Generali, Carlo Selmi
PURPOSE: Nailfold videocapillaroscopy (NVC) identifies the microvascular hallmarks of systemic sclerosis (SSc) and vascular endothelial growth factor (VEGF) and may play a pivotal role in the associated vasculopathy. The aim of the present study was to compare NVC alterations with clinical subsets, internal organ involvement, and serum VEGF levels in a cohort of selected SSc cases. METHODS: We studied 44 patients with SSc who were evaluated within 3 months from enrollment by NVC, skin score, severity index, pulmonary function tests, carbon monoxide diffusing capacity (DLCO), echocardiography, pulmonary high-resolution computed tomography (HRCT), gastroesophageal (GE) endoscopy or manometry or X-ray, and serum autoantibodies...
December 2016: Auto- Immunity Highlights
Saumen Nandi, Avradip Santra, Loknath Ghoshal, Soumya Kundu
Aspergilloma or mycetoma is a saprophytic fungal infection that colonizes pre-existing excavated lung lesion. However, its association with systemic sclerosis related interstitial lung disease is unusual and scarcely found in literature. We report a middle aged female with long standing systemic sclerosis, who was on immunosuppressive therapy for many years, presented with repeated haemoptysis. Although provisionally pulmonary tuberculosis was suspected, imaging investigations showed presence of bilateral masses inside bullous air spaces along with air-crescent sign suggestive of fungal ball...
December 2015: Journal of Clinical and Diagnostic Research: JCDR
Daisuke Takekoshi, Shiva Arami, Todd J Sheppard, Patricia Cole-Saffold, Jon C Michel, George T Kondos, Dean E Schraufnagel
Systemic sclerosis, or scleroderma, is a collagen vascular disease characterized by hardening of the skin and involvement of internal organs, most commonly the esophagus. The most frequent cause of death in these patients is lung disease. Esophageal dysfunction has been implicated in the pathogenesis of interstitial lung disease. We previously developed a standard for the esophageal diameter on chest computed tomography (CT) and hypothesized that patients with esophageal dilation would be more likely to have interstitial lung disease than those without...
2015: Tohoku Journal of Experimental Medicine
Partha Sarathi Chakraborty, Sellam Karunanithi, Varun Singh Dhull, Kunal Kumar, Madhavi Tripathi
We present the case of a 35-year-old man with calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia variant scleroderma who presented with dysphagia, Raynaud's phenomenon and calf pain. (99m)Tc-methylene diphosphonate bone scintigraphy was performed to identify the extent of the calcification. It revealed extensive dystrophic calcification in the left thigh and bilateral legs which was involving the muscles and was well-delineated on single photon emission computed tomography/computed tomography...
October 2015: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
Yossra A Suliman, Rucsandra Dobrota, Dörte Huscher, Thi D L Nguyen-Kim, Britta Maurer, Suzana Jordan, Rudolf Speich, Thomas Frauenfelder, Oliver Distler
OBJECTIVE: Validated methods for the screening and early diagnosis of systemic sclerosis (SSc; scleroderma)-related interstitial lung disease (ILD) are needed. The aim of this study was to evaluate the performance of pulmonary function tests (PFTs) compared with that of high-resolution computed tomography (HRCT) of the chest for the detection of SSc-related ILD in clinical practice, and to identify predictors of lung involvement that is functionally occult but significant on HRCT. METHODS: Prospectively enrolled patients with SSc were assessed according to the European League Against Rheumatism (EULAR)/EULAR Scleroderma Trial and Research standards...
December 2015: Arthritis & Rheumatology
A V Sosnovskaya, V V Fomin, E N Popova, M V Lebedeva, S V Moiseev, A A Svistunov, N A Mukhin
AIM: To study the role of serum surfactant protein D (SP-D) as a biomarker of lung injury in scleroderma systematica (SDS) in relation to the presence of gastroesophageal reflux (GER). SUBJECTS AND METHODS: Fifty-six patients (mean age 46±14 years) with diffuse and circumscribed SDS were examined and underwent pulmonary functional tests, X-ray and, if lung injury was present, high-resolution computed tomography of the lung, echocardiography, gastroduodenoscopy, and barium X-ray of the esophagus; an enzyme-linked immunosorbent assay was used to determine serum SP-D levels...
2015: Terapevticheskiĭ Arkhiv
Sara R Schoenfeld, Flavia V Castelino
Systemic sclerosis is a heterogeneous disease of unknown etiology with limited effective therapies. It is characterized by autoimmunity, vasculopathy, and fibrosis and is clinically manifested by multiorgan involvement. Interstitial lung disease is a common complication of systemic sclerosis and is associated with significant morbidity and mortality. The diagnosis of interstitial lung disease hinges on careful clinical evaluation and pulmonary function tests and high-resolution computed tomography. Effective therapeutic options are still limited...
May 2015: Rheumatic Diseases Clinics of North America
Michele Bisceglia, Antonio Chiaramonte, Gaetano Panniello, Antonio Tucci, Giulio Fraternali Orcioni, Thomas V Colby
Two cases of diffuse dendriform pulmonary ossification (DPO) are presented, one of the secondary type and the other of the idiopathic type. Case 1 was an adult female patient who underwent thoracic surgery to remove a posterior mediastinal bronchogenic cyst, which was discovered on a computed tomography scan performed after an episode of pneumonia when traction bronchiectasis with interstitial lung disease/fibrosis was also suspected in the lungs. Histologic examination performed on the resected lung tissue revealed numerous scattered small osseous spicules on a background of intense interstitial inflammation and fibrosis, leading to further clinical and laboratory investigations and the final diagnosis of DPO secondary to lung involvement by scleroderma...
January 2015: Advances in Anatomic Pathology
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