Search Our Scientific Publications & Authors

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

Rheumatol Int 2018 Oct 21;38(10):1901-1910. Epub 2018 Aug 21.

Department of Rheumatology, University Hospital Nancy, 5 rue du Morvan, 54500, Vandoeuvre les Nancy, France.

To study vertebral fracture (VF) prevalence and the scanographic bone attenuation coefficient of the first lumbar vertebra (SBAC-L1) on computed tomography scans (CT-scans) in systemic sclerosis patients. This monocentric retrospective study included patients followed from 2000 to 2014 and fulfilling ACR/EULAR 2013 criteria for systemic sclerosis and who underwent a thoracic or thoraco-abdomino-pelvic CT-scan during their follow-up. Clinical characteristics for sclerosis and osteoporosis risk factors were collected. Read More

BMJ Case Rep 2018 Jul 30;2018. Epub 2018 Jul 30.

Rheumatology, Johns Hopkins, Baltimore, Maryland, USA.

A 58-year-old man with limited cutaneous systemic sclerosis and myositis overlap complicated by interstitial lung disease presented with several months of progressive dyspnoea and weakness. CT chest revealed extensive pneumomediastinum that was not present on imaging 6 months before this study and appeared to be spontaneous, with no preceding trauma, infection or invasive procedures. Read More

BMC Pulm Med 2018 May 18;18(1):73. Epub 2018 May 18.

Internal Medicine Department, Hôpital Hôtel-Dieu, Centre Hospitalier Universitaire de Nantes, 1 place Alexis Ricordeau, 44093, Nantes, France.

Background: Pleuroparenchymal fibroelastosis (PPFE) is a very rare interstitial lung disease (ILD) characterized by progressive fibrotic lesions of the visceral pleura and the sub-pleural parenchyma, affecting predominantly the upper lobes. PPFE may occur in different contextes like bone marrow or lung transplantations, but also in the context of telomeropathy with mutations of telomerase reverse transcriptase (TERT), telomerase RNA component (TERC) or regulator of telomere elongation helicase 1 (RTEL1) genes. PPFE-like lesions have recently been described in patients with connective tissue disease (CTD)-related ILD. Read More

Curr Rheumatol Rev 2019 ;15(1):79-81

Department of Internal Medicine, Division of Rheumatology, Shiraz University of Medical Sciences, Shiraz, Iran.

Introduction: Gauzoma is an iatrogenic complication which occurs rarely due to surgical team negligence. Depending on the sterility of the retained tissue, it can lead to life threatening surgical complications or may remain asymptomatic for many years and be detected incidentally in imaging studies. It may be mistaken as tumors or aneurysms. Read More

Eur J Radiol 2018 Apr 31;101:50-58. Epub 2018 Jan 31.

Victor Babes University Timisoara Romania, Department V Internal Medicine, Eftimie Murge Str. 2, Romania.

Purpose: To evaluate the role of CT-textural features for monitoring lung involvement in subjects with systemic sclerosis(SSc) undergoing autologous stem cell transplantation(SCT) by comparison with semi-quantitative chest-CT, pulmonary function tests(PFT) and clinical parameters (modified Rodnan skin score[mRSS]).

Methods: In a retrospective single centre analysis, we identified 23 consecutive subjects(11male) with SSc between 07/2005 and 11/2016 that underwent chest CTs before, 6 and 12 months post-SCT. Response to therapy was defined at 6 months after transplantation as stabilisation or improvement in FVC > 10% and DLCO > 10%. Read More

J Thorac Imaging 2018 May;33(3):197-203

Department of Radiology, Mayo Clinic.

Purpose: Differentiating between systemic sclerosis-related interstitial lung disease (SSc-ILD) and idiopathic pulmonary fibrosis (IPF) is important because of the differences in workup, prognosis, and treatment. However, there is much overlap in the appearance of these 2 entities on high-resolution computed tomography. We propose that inflammation and/or fibrosis focally or disproportionately involving the bilateral anterolateral upper lobes and posterosuperior lower lobes ["Four Corners" Sign (FCS)] is specific for SSc-ILD. Read More

Presse Med 2018 Jan 20;47(1):90-92. Epub 2017 Dec 20.

Hôpital Saint-Antoine, service d'imagerie médicale, 184, rue du faubourg Saint-Antoine, 75012 Paris, France; Université Pierre et Marie Curie, 75005 Paris, France.

Rheumatology (Oxford) 2017 12;56(12):2197-2203

National Pulmonary Hypertension Service, Royal Free London NHS Foundation Trust.

Objectives: SSc-pulmonary arterial hypertension (SSc-PAH) is associated with worse response to therapy and survival when compared with idiopathic PAH. It is suggested that the vasculopathy in SSc may involve postcapillary pulmonary venules resulting in pulmonary veno-occlusive disease (PVOD). This may underlie the lower gas transfer and worse outcome on therapy. Read More

Tohoku J Exp Med 2017 09;243(1):77-83

Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine.

Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of periodic acid-schiff stain-positive lipoproteinaceous materials in the alveolar space due to impaired surfactant clearance by alveolar macrophage. Autoimmune PAP is the most common form of PAP, but rarely accompanies collagen disease or sarcoidosis. We report here a rare case of autoimmune PAP preceded by systemic sclerosis and sarcoidosis. Read More

J Clin Rheumatol 2017 Dec;23(8):411-415

Objectives: The objective of this study was to report the experience with rituximab treatment in a case series of patients with long-standing systemic sclerosis-associated interstitial lung disease (SSc-ILD).

Methods: We reviewed the charts of 197 SSc patients. Fourteen patients who received rituximab for SSc-ILD participated in this analysis. Read More

J Eur Acad Dermatol Venereol 2018 Feb 3;32(2):291-297. Epub 2017 Aug 3.

Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan.

Background: Antimicrobial peptides have attracted much attention as a member of disease-associated molecules in systemic sclerosis (SSc), which is pathologically characterized by immune abnormalities, vasculopathy and tissue fibrosis.

Objective: To investigate the potential contribution of one of the antimicrobial peptide psoriasin to the development of SSc.

Methods: Psoriasin expression in the skin samples and sera derived from SSc patients and its correlation with clinical parameters were analysed. Read More

Am J Respir Crit Care Med 2017 11;196(10):1298-1310

1 Comprehensive Pneumology Center, German Research Center for Environmental Health, Munich, Germany.

Rationale: Analyzing the molecular heterogeneity of different forms of organ fibrosis may reveal common and specific factors and thus identify potential future therapeutic targets.

Objectives: We sought to use proteome-wide profiling of human tissue fibrosis to (1) identify common and specific signatures across end-stage interstitial lung disease (ILD) cases, (2) characterize ILD subgroups in an unbiased fashion, and (3) identify common and specific features of lung and skin fibrosis.

Methods: We collected samples of ILD tissue (n = 45) and healthy donor control samples (n = 10), as well as fibrotic skin lesions from localized scleroderma and uninvolved skin (n = 6). Read More

Intern Med 2017 15;56(12):1549-1552. Epub 2017 Jun 15.

Division of Pulmonary Medicine, St. Luke's International Hospital, Japan.

Small cell lung cancer (SCLC) is a neuroendocrine tumor, and the median age of onset is about 70 years old. A 20-year-old non-smoking man with known systemic sclerosis presented with discomfort in his left chest. Chest X-ray showed a mass shadow in the left upper zone. Read More

Semin Arthritis Rheum 2017 10 1;47(2):228-234. Epub 2017 Apr 1.

Department of Internal Medicine, Federal University of Rio Grande do Sul (UFRGS), Porto Alegre, Brazil; Division of Rheumatology, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil.

Introduction: Interstitial lung disease (ILD) is currently the primary cause of death in systemic sclerosis (SSc). Thoracic high-resolution computed tomography (HRCT) is considered the gold standard for diagnosis. Recent studies have proposed several clinical algorithms to predict the diagnosis and prognosis of SSc-ILD. Read More

Arthritis Res Ther 2017 03 20;19(1):61. Epub 2017 Mar 20.

Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Viale Benedetto XV, n° 6, AOU IRCCS San Martino, 16132, Genova, Italy.

Background: The aim of the study was to detect by skin high-frequency ultrasound (US) possible subclinical skin involvement in patients affected by limited cutaneous systemic sclerosis (lcSSc), in those skin areas apparently not affected by the disease on the basis of a normal modified Rodnan skin score (mRSS). Differences in dermal thickness (DT) in comparison with healthy subjects were investigated.

Methods: Fifty patients with lcSSc (age 62 ± 13 years (mean ± SD), disease duration 5 ± 5 years) and 50 sex-matched and age-matched healthy subjects (age 62 ± 11 years) were enrolled. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2016 Dec 23;33(4):349-354. Epub 2016 Dec 23.

Istanbul Haseki Education and Research Hospital,.

 Introduction and aim: Progressive systemic sclerosis (pSS) is a multisystemic connective tissue disease characterized by fibrosis of the skin and internal organs including lung. The mechanisms that leads to progressive lung fibrosis in scleroderma remain obscure. In this study, we aimed to investigate the correlation between HRCT findings and patients' clinical and functional status and the degree of alveolitis based on the BAL resultsMaterials and methods: 65 patients with pSS were evaluated. Read More

Radiol Clin North Am 2016 Nov 12;54(6):1015-1031. Epub 2016 Aug 12.

Department of Radiology, University of Washington, 1959 Northeast Pacific Street, Seattle, WA 98195, USA.

Connective tissue diseases (CTDs) are a heterogeneous group of conditions characterized by circulating autoantibodies and autoimmune-mediated organ damage. Common CTDs with lung manifestations are rheumatoid arthritis, scleroderma or systemic sclerosis, Sjögren syndrome, polymyositis/dermatomyositis, systemic lupus erythematosis, mixed connective tissue disease, and undifferentiated connective tissue disease. The most common histopathologic patterns of CTD-related interstitial lung disease are nonspecific interstitial pneumonia, usual interstitial pneumonia, organizing pneumonia, and lymphoid interstitial pneumonia. Read More

Circ Cardiovasc Imaging 2016 06;9(6)

Department of Cardiac, Thoracic, and Vascular Sciences, University of Padua, Italy.

Chest 2016 09 24;150(3):631-9. Epub 2016 May 24.

Division of Pulmonary, Allergy and Critical Care Medicine, University of Massachusetts Medical School, Worcester, MA.

Background: Cystic lung disease (CLD) in Sjögren syndrome (SS) is a condition with unclear prognostic implications. Our objectives in this study are to determine its frequency, progression over time, and associated risk factors and complications.

Methods: Eighty-four patients with primary or secondary SS and chest imaging, chest radiograph, or CT scan were retrospectively evaluated for CLD. Read More

Int J Cardiol 2016 Aug 27;217:135-48. Epub 2016 Apr 27.

Department of Medicine, Radiology and Epidemiology Johns Hopkins University, Baltimore, MD, USA.

Targeted therapies in connective tissue diseases (CTDs) have led to improvements of disease-associated outcomes, but life expectancy remains lower compared to general population due to emerging co-morbidities, particularly due to excess cardiovascular risk. Cardiovascular magnetic resonance (CMR) is a noninvasive imaging technique which can provide detailed information about multiple cardiovascular pathologies without using ionizing radiation. CMR is considered the reference standard for quantitative evaluation of left and right ventricular volumes, mass and function, cardiac tissue characterization and assessment of thoracic vessels; it may also be used for the quantitative assessment of myocardial blood flow with high spatial resolution and for the evaluation of the proximal coronary arteries. Read More

Eur Respir J 2016 09 12;48(3):833-42. Epub 2016 May 12.

Service de Pneumologie, CHRU Tours, Tours, France Université François Rabelais, UMR 1100, Tours, France INSERM, Centre d'Etude des Pathologies Respiratoires, UMR 1100/EA6305, Tours, France

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). Read More

Clin Rheumatol 2016 Sep 23;35(9):2339-45. Epub 2016 Apr 23.

Autoimmune Systemic Diseases Unit Department of Internal Medicine, Vall d'Hebron University Hospital, Barcelona, Spain.

This study aims to evaluate the utility of magnetic resonance imaging (MRI) to assess interstitial lung disease (ILD) extent in patients with systemic sclerosis (SSc). Patients with SSc and varying degrees of ILD with a high-resolution computed tomography (HRCT), pulmonary function tests (PFTs), and a chest MRI containing an ultrafast SE sequence performed less than 1 year apart were included in the study. Wells global disease extent and Goh's staging algorithm were used to measure and categorize ILD both for MRI and HRCT. Read More

Semin Arthritis Rheum 2016 08 26;46(1):109-14. Epub 2016 Feb 26.

Institute for Public Health and Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL; Division of Rheumatology, Department of Medicine, Northwestern University Feinberg School of Medicine, 240 E Huron St, Suite M-300, Chicago, IL 60611. Electronic address:

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. Read More

J Dermatol 2016 Sep 15;43(9):1071-4. Epub 2016 Mar 15.

Department of Dermatology, Faculty of Medicine, Oita University, Yufu City, Japan.

Pemetrexed, which is used for the treatment of non-small cell lung carcinoma and malignant mesothelioma, induces cutaneous adverse reactions in approximately 20% of patients. There are also reports of the induction of fibrosing disorders. We describe a case of pemetrexed-induced scleroderma-like conditions in the lower legs of a patient whose pulmonary carcinoma has been relatively well controlled, with prolongation of the dose interval, in spite of the discomfort in both his legs. Read More

Rev Med Interne 2016 Oct 8;37(10):708-713. Epub 2016 Feb 8.

Service de médecine interne, pôle hospitalo-universitaire 3, centre de compétences maladies systémiques et auto-immunes rares, hôpital Hôtel-Dieu, place Alexis-Ricordeau, 44093 Nantes cedex 1, France; Inserm UMR1087, CNRS UMR6291, unité de recherche de l'Institut du thorax, équipe 2 « Signalisation et hypertension artérielle », IRS université de Nantes, 8, quai Moncousu, BP 70721, 44007 Nantes cedex 1, France. Electronic address:

Introduction: Jaccoud's arthropathy (JA) is a chronic and non-erosive deforming arthropathy, usually affecting the hands. JA pathophysiology is poorly known but involves periarticular structures such as tendons and the joint capsule. JA is associated with various conditions including the connective tissue disease, especially systemic lupus erythematosis. Read More

Respiration 2016 6;91(2):115-23. Epub 2016 Jan 6.

Respiratory Failure Unit, Aristotle University of Thessaloniki, Thessaloniki, Greece.

Background: Exercise impairment is a common symptom of systemic sclerosis (SSc), a disorder which is frequently complicated by cardiopulmonary involvement.

Objectives: This study's aims were: (a) to define the prevalence and the potential causes of limited exercise capacity and (b) to study potential differences in clinical, radiological and functional characteristics and blood serology among SSc patients with exercise limitation of different etiology.

Methods: Prospectively collected data on SSc patients who had conducted full lung function testing, blood serology, thorax high-resolution computed tomography, Doppler echocardiogram and a maximal cardiopulmonary exercise testing (CPET) were retrospectively analyzed. Read More

Reumatizam 2016;63(1):14-9

Systemic sclerosis (SSC) is an autoimmune disease associated with the risk of malignancies, especially lung cancer, among which adenocarcinoma and squamous cell carcinoma are the most frequent. A 63-year-old female patient with SSC was hospitalized due to blackouts, poor general condition, and changes in her fingers. Because of subsequent epileptic seizures resulting in weakness of the left side of her body, computerized tomography (CT) of the neurocranium was performed which showed metastatic lesions. Read More

Indian J Chest Dis Allied Sci 2016 Jan-Mar;58(1):7-10

Background: Systemic sclerosis (SSc) is a rare connective tissue disorder of unknown aetiology. Pulmonary involvement contributes substantially to its morbidity and mortality. Treatment of pulmonary disease due to SSc remains unsatisfactory. Read More

Radiology 2016 Apr 18;279(1):297-305. Epub 2015 Nov 18.

From the Department of Thoracic Imaging (EA 2694) (F.P., A.S.B., J.B.F., J.R., M.R.J.), Department of Medical Statistics (EA 2694) (A.D.), Department of Internal Medicine (E.H.), and Department of Pulmonary Function (R.M.), Hôpital Calmette, Université Lille II, Boulevard Jules Leclercq, 59037 Lille, France; and Research and Development Department, Siemens Healthcare, Forchheim, Germany (B.S.).

Purpose: To evaluate the effect of iterative reconstruction on the depiction of systemic sclerosis-related interstitial lung disease (ILD) when the radiation dose is reduced by 60%.

Materials And Methods: This study was based on retrospective interpretation of prospectively acquired data over a 12-month period and approved by the institutional review board. The requirement to obtain informed consent was waived. Read More

Rheumatology (Oxford) 2016 Mar 15;55(3):504-12. Epub 2015 Oct 15.

Department of Cardiology, Leiden University Medical Center, Leiden, The Netherlands,

Objectives: Right ventricular (RV) dysfunction is of great prognostic value in patients with SSc. The aim of the present study was to assess in these patients the relationship between pulmonary fibrosis and elevated pulmonary pressure (PHT) with RV function.

Methods: A total of 102 SSc patients who underwent thoracic CT and transthoracic echocardiography were included. Read More

Rheumatology (Oxford) 2015 Dec 28;54(12):2220. Epub 2015 Aug 28.

Department of Rheumatology and Immunology, Singapore General Hospital, Singapore.

Surg Neurol Int 2015 29;6:126. Epub 2015 Jul 29.

Department of Surgery, Section of Neurosurgery, Providence Hospital and Medical Centers, Michigan State University, East Lansing, United States of America ; Department of Neurosurgery, Michigan Spine and Brain Surgeons PLLC, Providence Hospital and Medical Centers, MI 48075, United States of America.

Background: Patients with chronic postural headaches may suffer from spontaneous intracranial hypotension (SIH). Trauma, degenerative disc spurring and connective tissue disorders are documented risk factors; in most cases there is no inciting event. Despite sophisticated means of evaluating the neuraxis, many cerebrospinal fluid (CSF) leaks are radiographically occult and treatment is focused on thoracic and cervical-thoracic regions. Read More

Scand J Rheumatol 2015 23;44(5):389-98. Epub 2015 Jun 23.

b Department of Medical and Paediatric Sciences , University of Catania , Italy.

Objectives: Systemic sclerosis (SSc) patients in the early stages of pulmonary fibrosis (PF) often have few or no symptoms, normal to borderline pulmonary function tests, and negative chest X-ray (CXR); high-resolution computed tomography (HRCT) is the only reliable means of detecting the early signs of PF. However, thoracic ultrasound (TUS) enables detection of pleural thickening, pleural/subpleural nodules, and other subpleural lung abnormalities across 70% of the subpleural surface. We reassessed concordance between TUS abnormalities and HRCT findings in SSc patients, to see whether TUS pleural line thickness (normally <3. Read More

Rheumatology (Sunnyvale) 2015 Jun 30;5(2). Epub 2015 Jun 30.

Department of Biomathematics, University of California, Los Angeles, USA.

Objective: While systemic sclerosis-related interstitial lung disease (SSc-ILD) trials predominantly use forced vital capacity (FVC) as the primary outcome, combining individual outcomes may lead to a more comprehensive measure of treatment response and minimize the risk of type 1 error. The present analysis aimed to develop a composite outcome measure to assess treatment response in SSc-ILD patients.

Methods: We used data from the Scleroderma Lung Study I (SLS-I) to create the composite outcome measure. Read More

Indian J Chest Dis Allied Sci 2014 Oct-Dec;56(4):231-5

Aim: This study was undertaken to find out the characteristics of clinical, radiological and functional changes affecting the respiratory system in patients with systemic sclerosis (SSc) from eastern India, and the association of these characteristics with pulmonary hypertension.

Methods: This was a cross-sectional, observational study involving 46 patients. Other than the routine tests, anti-nuclear antibody (ANA), spirometry, diffusing capacity of lung for carbon monoxide (DLCO) measurement, chest radiograph, high-resolution computed tomography (HRCT) of thorax, 6-minute walk test and echocardiography were done. Read More

AJR Am J Roentgenol 2015 Feb;204(2):408-22

1 Department of Radiology, University of Washington Medical Center, Seattle, WA.

OBJECTIVE. Juvenile systemic sclerosis is a rare multisystem autoimmune disorder characterized by vasculopathy and multiorgan fibrosis. Cardiopulmonary complications are the leading cause of morbidity and mortality. Read More

Curr Respir Med Rev 2015;11(2):163-174

New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center; New Orleans, LA, USA; Tulane University Lung Center; New Orleans, LA, USA.

The chronic fibrosing idiopathic interstitial pneumonias (IIPs) are a group of heterogeneous pulmonary parenchymal disorders described by radiologic and histological patterns termed usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP). These include idiopathic pulmonary fibrosis (IPF) and those related to connective tissue disease (CTD) and are associated with substantial morbidity and mortality. Beyond the importance of establishing an appropriate diagnosis, designing optimal clinical trials for IIPs has been fraught with difficulties in consistency of clinical endpoints making power analyses, and the establishment of efficacy and interpretation of results across trials challenging. Read More

Thorax 2015 Apr 18;70(4):382-7. Epub 2014 Dec 18.

Sheffield Pulmonary Vascular Disease Unit and Academic Department of Respiratory Medicine, Royal Hallamshire Hospital, Sheffield, UK.

We evaluated the prevalence and prognostic value of CT-pulmonary angiographic (CTPA) measures in 292 treatment naive patients with pulmonary arterial hypertension (PAH). Pulmonary artery calcification (13%) and thrombus (10%) were exclusively seen in PAH-congenital heart disease. Oesophageal dilation (46%) was most frequent in PAH-systemic sclerosis. Read More

Ann Rheum Dis 2016 Feb 1;75(2):374-81. Epub 2014 Dec 1.

Department of Biostatistics, David Geffen School of Medicine at UCLA, Los Angeles, California, USA.

Objectives: Extent of systemic sclerosis (SSc)-related interstitial lung disease (ILD) assessed from thoracic high-resolution CT (HRCT) predicts disease course, mortality and treatment response. While quantitative HRCT analyses of extent of lung fibrosis (QLFib) or total interstitial lung disease (QILD) are more sensitive and reproducible than visual HRCT assessments of SSc-ILD, these analyses are not widely available. This study evaluates the relationship between clinical disease parameters and QLFib and QILD scores to identify potential surrogate measures of radiographic extent of ILD. Read More

Respir Care 2015 Feb 11;60(2):259-68. Epub 2014 Nov 11.

St Vincent's University Hospital, Dublin University College Dublin, Dublin, Republic of Ireland.

Background: Emphysema and fibrosis, typically the idiopathic pulmonary fibrosis (IPF) form of usual interstitial pneumonia (UIP), can co-exist as combined pulmonary fibrosis emphysema (CPFE). It is unknown whether there is a pathobiologic basis for CPFE beyond the coexistence of fibrosis and emphysema. The aim of this study was to ascertain radiologic differences in severity of fibrosis and emphysema in smokers with IPF versus other forms of UIP. Read More

Curr Probl Diagn Radiol 2015 Jan-Feb;44(1):47-59. Epub 2014 Aug 15.

Department of Radiology, Hospital de Santa Marta, Central Lisbon Hospital Centre, Lisbon, Portugal.

Connective tissue diseases (CTDs) comprise several immunologic systemic disorders, each of which associated with a particular set of clinical manifestations and autoimmune profile. CTDs may cause numerous thoracic abnormalities, which vary in frequency and pattern according to the underlying disorder. The CTDs that most commonly involve the respiratory system are progressive systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, Sjögren syndrome, polymyositis, dermatomyositis, and mixed connective tissue disease. Read More

Clin Rheumatol 2016 May 4;35(5):1403-9. Epub 2014 Jun 4.

Department of Surgical Pathology, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan.

Dystrophic calcinosis in soft tissue occurs in damaged or devitalized tissues in the presence of normal calcium and phosphorous metabolism. It is often noted in subcutaneous tissues in patients with collagen vascular diseases and may involve a relatively localized area or be widespread. A 74-year-old Japanese woman with an overlap of rheumatoid arthritis, Sjögren's syndrome, and systemic sclerosis developed a huge tumor-like mass at the atlanto-axial vertebral joint region that caused severe cervical pain and difficulty in activities of daily living. Read More

Clin Radiol 2014 Jul 10;69(7):758-64. Epub 2014 May 10.

Department of Clinical Radiology, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan.

Aim: To verify whether quantitative analysis of the extent of ground-glass opacity (GGO) on high-resolution computed tomography (HRCT) could show a stronger correlation with the therapeutic response of interstitial pneumonia (IP) associated with systemic sclerosis (SSc) compared with qualitative analysis.

Materials And Methods: Seventeen patients with IP associated with SSc received autologous peripheral blood stem cell transplantation (auto-PBSCT) and were followed up using HRCT and pulmonary function tests. Two thoracic radiologists assessed the extent of GGO on HRCT using a workstation. Read More

Neuromuscul Disord 2014 May 1;24(5):425-30. Epub 2014 Feb 1.

Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA.

Inflammatory myopathy is rare in localized scleroderma. We report 2 new cases of regional inflammatory myopathy associated with localized scleroderma and review 10 reported cases of localized scleroderma associated with an inflammatory myopathy with regional muscle involvement, more often in the upper extremities. Serum creatine kinase was mildly elevated or normal. Read More

Med Ultrason 2014 Mar;16(1):27-31

Brigham and Women's Hospital, Harvard-MIT Division of Health Sciences and Technology, Harvard Medical School, USA, Email:

Aims: Pulmonary fibrosis is the main cause of mortality in patients with Systemic Scleroderma (SSc). This study was performed to investigate the utility of modified trans-thoracic ultrasound (TTUS) scoring system according to the comet tail sign (B-line artifacts) and to compare it with high-resolution computed tomography (HRCT) findings in patients with SSc and pulmonary involvement.

Patients And Method: Seventy subjects with SSc diagnosed according to the American College of Rheumatology criteria were enrolled. Read More

J Comput Assist Tomogr 2014 Mar-Apr;38(2):163-8

From the *Division of Rheumatology, Department of Medicine, †Department of Radiology and Radiologic Sciences, and ‡Division of Biostatistics and Epidemiology, Department of Medicine, Medical University of South Carolina, Charleston, SC.

Objective: This study aimed to determine the relationship between main pulmonary artery diameter (MPAD) and pulmonary hypertension (PH) in scleroderma patients with and without interstitial lung disease.

Methods: We retrospectively reviewed 48 subjects with scleroderma who underwent a chest computed tomography (CT) and right heart catheterization with 6 months of each other. Patients were divided into 2 groups based on the absence or presence of interstitial lung disease on chest CT. Read More

Clin Exp Rheumatol 2014 Nov-Dec;32(6 Suppl 86):S-21-7. Epub 2013 Dec 2.

Department of Internal Medicine, University of Michigan Scleroderma Program, Ann Arbor, MI, USA.

Objectives: The aim of this study was to utilise the Quality Enhancement Research Initiative in Systemic Sclerosis (QuERI-SSc) to measure and reduce a perceived gap in the diagnosis of pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc).

Methods: Rheumatologists enrolled patients with SSc (aged ≥ 18 years) and provided data on a panel of diagnostic tests over 3 years. Pulmonary function testing, echocardiography, 6-minute walk distance, N-terminal pro-brain natriuretic peptide assays, high-resolution computed tomography of the lungs, and ventilation/perfusion scan plus right heart catheterisation (RHC; when appropriate) were emphasised. Read More

Acta Radiol 2014 Nov 19;55(9):1050-5. Epub 2013 Nov 19.

Department of Pathology, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai City, Japan.

Background: Collagen vascular disease-associated interstitial lung disease (CVD-ILD) must be differentiated from idiopathic pulmonary fibrosis (IPF) since prognosis and treatment strategies differ between these two conditions. However, differentiating between CVD-ILD and IPF is often difficult.

Purpose: To examine the utility of expiratory high-resolution computed tomography (HRCT) for differentiating between CVD-ILD and IPF. Read More

Lung 2013 Oct 8;191(5):483-9. Epub 2013 Aug 8.

First Department of Propedeutic and Internal Medicine, Athens University Medical School, Athens, Greece,

Objective: Cyclophosphamide is considered the treatment of choice for interstitial lung disease (ILD) secondary to systemic sclerosis (SSc), albeit having a minimal effect. Although controlled evidence does not exist, mycophenolate is used increasingly in clinical practice as an alternative. We aimed to compare the long-term efficacy of these drugs. Read More