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    Scleroderma-related interstitial lung disease.
    Respir Med Case Rep 2017 15;22:109-112. Epub 2017 Jul 15.
    Department of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of Louisville Health Sciences Center, Louisville, KY, USA.
    Scleroderma-related interstitial lung disease (SSc-ILD) is a pulmonary fibrosing disorder characterized by systemic inflammation and progressive scarring of the lungs that leads to respiratory failure. Although certain immunosuppressive therapies may slow disease progression, current treatment strategies are not curative; consequently, SSc-ILD continues to be a major cause of morbidity and mortality. We present four cases of SSc-ILD that emphasize the importance of early screening and detection, close follow-up, and aggressive management. We also highlight the need for well-conducted clinical trials designed to identify new and effective treatments.

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    Rev Mal Respir 2007 Oct;24(8):1035-46
    Service de Médecine Interne, Hôpital Cochin, Centre de Référence pour la sclérodermie systémique, Assistance Publique-Hôpitaux de Paris et Université Paris-Descartes, Faculté de Médecine Paris-Descartes, Paris, France.
    Introduction: Interstitial lung diseases (ILD) in systemic sclerosis (SSc) are mainly encountered in patients with diffuse disease although they may occur less frequently in patients with limited cutaneous disease.

    Background: In SSc early detection of ILD should be achieved by high resolution computed tomography and pulmonary function tests, including measurement of DLCO. In total up to 75% of patients with SSc develop ILD but it is progressive in only a minority of patients. Read More
    Interstitial lung disease in systemic sclerosis.
    Autoimmun Rev 2011 Mar 21;10(5):248-55. Epub 2010 Sep 21.
    Université Paris Descartes, Institut Cochin, Inserm U1016, Paris, France.
    Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc) and mainly encountered in patients with diffuse disease and/or anti-topoisomerase 1 antibodies. ILD develops in up to 75% of patients with SSc overall. However, SSc-ILD evolves to end-stage respiratory insufficiency in only a few patients. Read More
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    Arthritis Rheumatol 2015 Dec;67(12):3256-61
    University Hospital Zurich, Zurich, Switzerland.
    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. Read More
    Association of Interferon- and transforming growth factor β-regulated genes and macrophage activation with systemic sclerosis-related progressive lung fibrosis.
    Arthritis Rheumatol 2014 Mar;66(3):714-25
    Boston University School of Medicine, Boston, Massachusetts; University of Sao Paulo School of Medicine, Sao Paulo, Brazil.
    Objective: Systemic sclerosis (SSc)-related interstitial lung disease (ILD) is one of the leading causes of mortality. We undertook this study to analyze the gene expression of lung tissue in a prospective cohort of patients with SSc-related ILD and to compare it with that in control lungs and with 2 prospective clinical parameters in order to understand the molecular pathways implicated in progressive lung disease.

    Methods: Lung tissue was obtained by open lung biopsy in 28 consecutive patients with SSc-related ILD and in 4 controls. Read More