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Rheumatology (Oxford) 2022 Jun 16. Epub 2022 Jun 16.

Deptartment of Experimental and Clinical Medicine & Division of Rheumatology, Azienda Ospedaliero Universitaria Careggi, Firenze, Italy.

Objectives: Lung vascular and parenchymal changes can be recently assessed quantitatively in thoracic computed tomography (CT) images using automated software tools. We investigated the vessel parameters of patients with Systemic Sclerosis (SSc), quantified by CT imaging, and correlated them with the interstitial lung disease (ILD) features.

Methods: SSc patients undergoing standard of care pulmonary function testing and CT evaluation were retrospectively evaluated. Read More

J Scleroderma Relat Disord 2021 Oct 21;6(3):322-326. Epub 2021 Jun 21.

Department of Rheumatology and clinical Immunology, Sir Ganga Ram Hospital, New Delhi, India.

Ascending aorta aneurysm in scleroderma can be ascribed to its macrovascular involvement which is very less elucidated. We here describe a 56-year-old female with rapidly progressive diffuse cutaneous scleroderma. She presented with skin thickening involving all four limbs, thorax and abdomen. Read More

J Scleroderma Relat Disord 2021 Oct 30;6(3):271-276. Epub 2021 Jun 30.

Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India.

Introduction: There is a paucity of real-world data on mycophenolate mofetil/mycophenolate sodium in systemic sclerosis-related interstitial lung disease.

Aim: To study the efficacy of mycophenolate mofetil/ mycophenolate sodium in systemic sclerosis-related interstitial lung disease.

Methods: In this single-centre study, clinical, laboratory and imaging details of consecutive patients with systemic sclerosis-related interstitial lung disease receiving mycophenolate mofetil/mycophenolate sodium from rheumatology and pulmonology clinics between January 2008 and March 2017 were retrospectively retrieved. Read More

BMC Pulm Med 2022 Jan 25;22(1):44. Epub 2022 Jan 25.

Internal Medicine Department, Nantes Université, CHU Nantes, 1 place Alexis Ricordeau, 44 000, Nantes, France.

Background: Thoracic multidetector computed tomography (MDCT) is essential for the detection of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc). Thoracic MDCT assessment can reveal the presence of thoracic lymphadenopathies (LAP) whose signification remains uncertain. The purpose of the study was to describe the characteristics and to assess the significance of thoracic LAP in patients with diffuse SSc. Read More

Intern Med J 2021 Dec 11. Epub 2021 Dec 11.

Institute for Health Research, University of Notre Dame, Fremantle, Western Australia.

Background: Bronchiectasis has been observed in association with systemic sclerosis (SSc). Theorised aetiology includes aspiration related to oesophageal dysmotility, immunosuppressant medication use and the direct effect of collagen deposition on airway calibre.

Aims: In this study we detail bronchiectasis prevalence in an SSc population who have had a high-resolution computed tomography (HRCT) thorax. Read More

Rheumatology (Oxford) 2022 05;61(6):2221-2234

Faculty of Medicine, University of New South Wales.

Objectives: The nailfold videocapillaroscopy (NVC) has been known to assist with interstitial lung disease (ILD) classification. However, evidence on its diagnostic efficacy is limited, particularly in some connective tissue disease-related interstitial lung diseases (CTD-ILD), and in interstitial pneumonia with autoimmune features (IPAF). This study aimed to address this limitation by conducting a meta-analysis on the efficacy of the NVC in ILD subgroups of CTD-ILD, IPAF and idiopathic pulmonary fibrosis (IPF). Read More

Acta Med Indones 2021 Jul;53(3):254-260

Department of Radiology, Faculty of Medicine, Universitas Padjadjaran, Hasan Sadikin Hospital, Bandung, Indonesia.

Background: Systemic sclerosis (SSc) is a systemic autoimmune disease involving a multisystem. Modified Rodnan's Skin Score (MRSS) is a gold standard for measuring skin fibrosis in SSc. In SSc, lung fibrosis disorders, especially interstitial lung disease (ILD), are the leading cause of mortality and often late in diagnosis. Read More

Clin Rheumatol 2022 Feb 16;41(2):513-521. Epub 2021 Sep 16.

Department of Rheumatology, Kocaeli University Faculty of Medicine, Kocaeli, Turkey.

Objectives: The aim was to investigate the discriminative value of a wide range of quantitative computed tomography (qCT) parameters in systemic sclerosis (SSc) patients with and without pulmonary fibrosis (PF) and their association with pulmonary function tests (PFTs) and visual fibrosis scores (VFS).

Method: Thoracic high-resolution computed tomography (HRCT) images of SSc patients with and without PF were analyzed with Vitrea® Advanced Visualization software. The mean lung attenuation (MLA), skewness, kurtosis, and threshold-based volumes [low-density volume (LDV), medium-density volume (MDV), and high-density volume (HDV)] derived from the attenuation histograms of the right and left lungs were evaluated separately. Read More

Radiology 2021 07;300(1):237-242

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.); Depart ments 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

J Thorac Cardiovasc Surg 2022 06 9;163(6):1979-1986. Epub 2021 Jan 9.

Creighton University School of Medicine-Phoenix Regional Campus, Phoenix, Ariz; Thoracic Surgery and Lung Transplantation Program, Department of Thoracic Disease and Transplantation, Norton Thoracic Institute, St Joseph's Hospital and Medical Center, Phoenix, Ariz.

Objectives: We reported that esophageal peristalsis can improve after lung transplant (LTx), even in patients with pretransplant esophageal aperistalsis. This improvement was associated with better outcomes. We analyzed preoperative factors and sought to predict persistent aperistalsis or motility improvement in patients with pre-LTx esophageal aperistalsis. Read More

Cancers (Basel) 2021 Jan 16;13(2). Epub 2021 Jan 16.

Institute of Pathology, University Medical Centre Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany.

Thymic hyperplasia (TH) with lymphoepithelial sialadenitis (LESA)-like features (LESA-like TH) has been described as a tumor-like, benign proliferation of thymic epithelial cells and lymphoid follicles. We aimed to determine the frequency of lymphoma and autoimmunity in LESA-like TH and performed retrospective analysis of cases with LESA-like TH and/or thymic MALT-lymphoma. Among 36 patients (21 males) with LESA-like TH (age 52 years, 32-80; lesion diameter 7. Read More

Int J Clin Pract 2021 Apr 20;75(4):e13931. Epub 2020 Dec 20.

Department of Rheumatology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey.

Objectives: To investigate lung volume and density in patients with SSc and changes in these parameters because of PF, using a software-aided image quantification method, and compare this with a matched healthy control group.

Methods: Thoracic high-resolution computed tomography (HRCT) images of patients and controls were analysed using Myrian XP Lung 3D software. Right, and left lung densities and volumes were calculated separately by a blinded operator. Read More

Medicine (Baltimore) 2020 Oct;99(43):e22443

Department of Rheumatology, University of Lorraine, CHRU Nancy.

The objectives of this study were to describe the prevalence and characteristics of radiographic lesions of the hands, and calcifications of the spine on computer tomography scans (CT-scans), and to investigate the relationships between radiographic and CT-scan abnormalities and clinical features in a population of patients with systemic sclerosis (SSc).Subjects underwent X-ray examination of the hands, and thoracic or thoraco-abdominal and pelvic CT scan or lumbar CT scan in the year. Structural lesions on hand X ray was scored and spinal calcifications were evaluated in the anterior, intracanal and posterior segments. Read More

Eur Radiol 2021 Apr 6;31(4):1987-1998. Epub 2020 Oct 6.

Institute of Diagnostic and Interventional Radiology, University Hospital Zurich, University of Zurich, Rämistrase 100, 8091, Zurich, Switzerland.

Objective: To retrospectively evaluate if texture-based radiomics features are able to detect interstitial lung disease (ILD) and to distinguish between the different disease stages in patients with systemic sclerosis (SSc) in comparison with mere visual analysis of high-resolution computed tomography (HRCT).

Methods: Sixty patients (46 females, median age 56 years) with SSc who underwent HRCT of the thorax were retrospectively analyzed. Visual analysis was performed by two radiologists for the presence of ILD features. Read More

Cells 2020 09 16;9(9). Epub 2020 Sep 16.

Department of Medical Sciences, Allergy and Clinical Immunology Unit, University of Torino & Mauriziano Hospital, 10128 Turin, Italy.

Background: Systemic sclerosis (SSc) is a connective tissue disorder which key feature is a fibrotic process. The role of Endothelin-1 (ET-1) and T-helper (Th)-1 cells in lung and skin fibrosis is well known, although Th17- and Treg-cells were found to be involved. However, no studies analyzed cytokines expression in gastric-juice of SSc patients. Read More

Acta Biomed 2020 07 13;91(8-S):43-50. Epub 2020 Jul 13.

Unit of Diagnostic Imaging, Department of Medical, Surgical and Neuro Sciences and of Radiological Sciences, University of Siena, Azienda Ospedaliero-Universitaria Senese, Siena, Italy.

The anterior chest wall (AWC) non-traumatic pathologies are largely underestimated, and early detection through imaging is becoming increasingly important. This paper aims to review the major non-traumatic ACW pathologies, with a particular interest in imaging features and differential diagnosis. Read More

Semin Arthritis Rheum 2020 10 29;50(5):949-956. Epub 2020 Aug 29.

Department of Internal Medicine and Clinical Immunology, CHU Rennes, University of Rennes 1, Rennes, France; University of Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de recherche en santé, environnement et travail) - UMR_S 1085, F-35000, Rennes, France. Electronic address:

Context: Thoracic lymphadenopathy (LA) has been identified as a key prognostic factor in interstitial lung disease (ILD) of all-cause. Crystalline silica is a risk factor of systemic sclerosis (SSc). The association of a history of crystalline silica exposure with chest high-resolution computed tomography (HRCT) features and thoracic LA are still to be determined in SSc patients. Read More

J Comput Assist Tomogr 2020 Sep/Oct;44(5):667-672

From the Departments of Radiology.

Objective: The aim of the study was to compare the prevalence of diffuse pulmonary ossification (DPO) in idiopathic pulmonary fibrosis (IPF), systemic sclerosis (SSc)-related interstitial lung disease (ILD), and chronic hypersensitivity pneumonitis (HP).

Methods: High-resolution computed tomography (HRCT) from 71 IPF, 67 SSc-ILD, and 75 HP cases were independently evaluated by 2 thoracic radiologists blinded to patient data. Studies were assessed for the presence of DPO, HRCT scanning pattern, stigmata of granulomatous infection, and honeycombing. Read More

Gastroenterol Clin North Am 2020 09 16;49(3):451-466. Epub 2020 Jun 16.

Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine, Temple University, 3401 North Broad Street, 7th Floor Parkinson Pavilion, Philadelphia, PA 19140, USA.

Lung transplantation is a high-risk, but lifesaving, procedure for patients with end-stage lung disease. Although 1-year survival is high, long-term survival is not nearly as high, due mainly to acute and chronic rejection. Bronchiolitis obliterans syndrome is the most common type of chronic rejection and often leads to poor outcomes. Read More

Rheumatology (Oxford) 2021 01;60(1):250-255

Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo.

Objective: To investigate the potential contribution of accessory respiratory muscle atrophy to the decline of forced vital capacity (FVC) in patients with SSc-associated interstitial lung disease (ILD).

Methods: This single-centre, retrospective study enrolled 36 patients with SSc-ILD who underwent serial pulmonary function tests and chest high-resolution CT (HRCT) simultaneously at an interval of 1-3 years. The total extent of ILD and chest wall muscle area at the level of the ninth thoracic vertebra on CT images were evaluated by two independent evaluators blinded to the patient information. Read More

Clin Rheumatol 2020 Nov 8;39(11):3393-3400. Epub 2020 May 8.

Division of Rheumatology, University Hospitals Leuven, Herestraat 49, 3000, Leuven, Belgium.

Objectives: In systemic sclerosis, baseline extent of radiological involvement is an important outcome predictor and baseline absence of radiological involvement suggests a more favourable prognosis. As current predictive models are based on cohorts with variable disease duration, we aim to assess disease dynamics in early disease.

Methods: Patients were included from the prospective longitudinal Belgian Systemic Sclerosis Cohort. Read More

J Rheumatol 2021 02 1;48(2):241-246. Epub 2020 Apr 1.

C. Bruni, MD, G. Lepri, MD, G. Tesei, MD, S. Guiducci, MD, D. Melchiorre, MD, S. Bellando-Randone, MD, M. Matucci-Cerinic, MD, Department of Experimental and Clinical Medicine, University of Florence, and Division of Rheumatology AOUC & Scleroderma Unit, Florence.

Objective: The aim of the present retrospective observational study was to evaluate the change of Renal Resistive Index (RRI) over time (ΔRRI) and under treatment in patients with systemic sclerosis (SSc) as well as to correlate these changes with disease complications.

Methods: Two hundred thirty patients [29 male, median age 57 (IQR 48-67) yrs] were enrolled. At baseline and follow-up (3. Read More

Arthritis Rheumatol 2020 07 27;72(7):1236. Epub 2020 May 27.

University of Western Ontario, London, Ontario, Canada.

Eur Radiol 2020 May 10;30(5):2722-2730. Epub 2020 Feb 10.

Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea.

Objectives: To investigate CT imaging features associated with poor clinical outcome after corticosteroid treatment in patients diagnosed with cryptogenic organizing pneumonia (COP) and connective tissue disease-related organizing pneumonia (CTD-OP) and to assess the difference in CT findings and treatment responses between COP and CTD-OP.

Methods: Chest CT images from 166 patients (COP, 131; CTD-OP, 35) with pathologically proven organizing pneumonia were reviewed by two thoracic radiologists. The type, distribution pattern, and extent of parenchymal abnormalities, along with other associated imaging features, were assessed for each patient. Read More

Rheumatology (Oxford) 2020 Sep;59(9):2523-2533

Rheumathology Division, Fondazione Policlinico Universitario A. Gemelli - IRCCS, Rome.

Objective: To outline the clinical, histological and prognostic features of systemic sclerosis (SSc) endomyocardial biopsy-proven myocarditis with respect to those of diverse endomyocardial biopsy-proven virus-negative myocarditis (VNM).

Methods: We retrospectively analysed data from three cohorts of endomyocardial biopsy-proven myocarditis: SSc-related VNM (SSc-VNM); isolated VNM (i-VNM); and VNM related to other systemic autoimmune diseases (a-VNM). The degree of myocardial fibrosis was expressed as relative percentage and fibrotic score (0-3). Read More

Clin Rheumatol 2020 May 22;39(5):1687-1691. Epub 2020 Jan 22.

Cardiology Department, Hospital Garcia de Orta EPE, Avenida Torrado da Silva, 2805-267, Almada, Portugal.

Pulmonary hypertension is a serious complication of systemic sclerosis and remains one of the leading causes of mortality. Pulmonary veno-occlusive disease (PVOD), recently reclassified as pulmonary arterial hypertension (PAH) with overt features of venous/capillaries involvement, is a subgroup of group 1 pulmonary hypertension, which has been rarely reported in systemic sclerosis patients. It is symptomatically indistinguishable from idiopathic pulmonary arterial hypertension and should be suspected in those with manifestations of pulmonary arterial hypertension who have evidence of pulmonary venous congestion in the absence of left-sided heart disease. Read More

Eur J Intern Med 2019 Dec 17;70:43-49. Epub 2019 Sep 17.

Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Via Delle Oblate 4, 50134 Florence, Italy; Department of Geriatric Medicine, Division of Rheumatology, Azienda Ospedaliera Universitaria Careggi, Florence, Italy.

Background: Renal Resistive Index (RRI), reflects changes in both renal vascular and tubular-interstitial compartments and in systemic vascular compliance related to age and comorbidities.

Objectives: a) To investigate determinants of RRI in SSc population, b) its association with SSc-related features and c) to test its prognostic impact on organ specific worsening or death.

Methods: 380 SSc patients ≥18 years were enrolled after giving informed consent. Read More

ERJ Open Res 2019 Feb 11;5(1). Epub 2019 Feb 11.

Division of Pulmonary Medicine, Dept of Medicine, National Jewish Health, Denver, CO, USA.

http://ow.ly/vPvc30neJZV. Read More

Eur Respir J 2019 01 24;53(1). Epub 2019 Jan 24.

Dept of Pulmonary Circulation and Cardidology, Medical Center for Postgraduate Education, ECZ-Otwock, Otwock, Poland.

A revised diagnostic algorithm provides guidelines for the diagnosis of patients with suspected pulmonary hypertension, both prior to and following referral to expert centres, and includes recommendations for expedited referral of high-risk or complicated patients and patients with confounding comorbidities. New recommendations for screening high-risk groups are given, and current diagnostic tools and emerging diagnostic technologies are reviewed. Read More

Semin Arthritis Rheum 2019 08 13;49(1):98-104. Epub 2018 Oct 13.

Department of Internal Medicine, Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies Systémiques Autoimmunes Rares d'Ile de France, DHU Authors (Autoimmune and Hormonal Diseases), Université Paris Descartes, Assistance Publique-Hôpitaux de Paris (AP-HP), 27, rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14, France. Electronic address:

Background: The syndrome of combined pulmonary fibrosis and emphysema (CPFE) primarily due to tobacco smoking has been reported in connective tissue disease, but little is known about its characteristics in systemic sclerosis (SSc).

Methods: In this retrospective multi-center case-control study, we identified 36 SSc patients with CPFE, and compared them with 72 SSc controls with interstitial lung disease (ILD) without emphysema.

Results: Rate of CPFE in SSc patients with CT scan was 3. Read More