scholarly journals Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 5. Connective Tissue Disease Associated Interstitial Lung Disease

2019 ◽  
Vol 82 (4) ◽  
pp. 285 ◽  
Author(s):  
So-My Koo ◽  
Song Yee Kim ◽  
Sun Mi Choi ◽  
Hyun-Kyung Lee ◽  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Diagnosis And Management

scholarly journals Comparison of Characteristics of Connective Tissue Disease-Associated Interstitial Lung Diseases, Undifferentiated Connective Tissue Disease-Associated Interstitial Lung Diseases, and Idiopathic Pulmonary Fibrosis in Chinese Han Population: A Retrospective Study

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Lin Pan ◽  
Yuan Liu ◽  
Rongfei Sun ◽  
Mingyu Fan ◽  
Guixiu Shi
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Western China ◽  
Undifferentiated Connective Tissue Disease

Our study compared the prevalence and characteristics of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD), undifferentiated connective tissue disease-associated interstitial lung disease (UCTD-ILD), or idiopathic pulmonary fibrosis (IPF) between January 2009 and December 2012 in West China Hospital, western China. Patients who met the criteria for ILD were included and were assigned to CTD-ILD, UCTD-ILD, or IPF group when they met the criteria for CTD, UCTD, or IPF, respectively. Clinical characteristics, laboratory tests, and high-resolution CT images were analyzed and compared among three groups. 203 patients were included, and all were Han nationality. CTD-ILD was identified in 31%, UCTD-ILD in 32%, and IPF in 37%. Gender and age differed among groups. Pulmonary symptoms were more common in IPF, while extrapulmonary symptoms were more common in CTD-ILD and UCTD-ILD group. Patients with CTD-ILD had more abnormal antibody tests than those of UCTD-ILD and IPF. Little significance was seen in HRCT images among three groups. A systematic evaluation of symptoms and serologic tests in patients with ILD can identify CTD-ILD, UCTD-ILD, and IPF.

scholarly journals Distribution of interstitial lung diseases in a tertiary care centre of South India

2021 ◽  
Vol 8 (3) ◽  
pp. 420
Author(s):  
Mahesh Babu Vemuri ◽  
Manju Rajaram ◽  
Madhusmita Mohanty Mohapatra ◽  
Mathavaswami Vijayageetha ◽  
V. S. Negi ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Lung Diseases ◽  
South India ◽  
Tertiary Care ◽  
Interstitial Lung Diseases ◽  
Care Centre ◽  
Tertiary Care Centre

Background: The term interstitial lung diseases (ILD) refer to a broad category of lung diseases rather than a specific disease entity. True prevalence of ILD is difficult to estimate and it may vary according to the geography, environment, occupation etc. Aim and objectives were to find out the distribution of interstitial lung disease (ILD) subtypes in a tertiary care centre.  Methods: A descriptive study was conducted between August 2016 and November 2018 in a tertiary care centre in Puducherry, South India. Baseline demographic details, clinical symptoms, signs, radiological findings (chest radiograph and HRCT), pathological findings, and physiological findings were taken into consideration and diagnosis of type of interstitial lung disease was made using multidisciplinary discussion. Statistical analysis was done using SPSS 19.0 version.Results: A total of 150 patients were recruited of which 70.5% were females. Most common ILD subtype in our study was connective tissue disease associated ILD – 97 patients (65%) followed by idiopathic pulmonary fibrosis (IPF)–31 patients (22%). Most common type of CTD ILD observed in our study was progressive systemic sclerosis (46%) followed by mixed connective tissue disease (24%). The most common HRCT finding was NSIP pattern and most commonly observed physiological abnormality was moderate restriction and moderate diffusion impairment.  Conclusions: Connective tissue disease-associated ILD was the most common ILD found in our study amongst south Indian population. This suggests that the distribution of ILD would vary depending on the geographical area and the environmental exposure which was in contrast with the Indian ILD registry.  

Interstitielle Lungenerkrankung bei rheumatischen Erkrankungen – neue Therapieansätze

2020 ◽  
Vol 145 (07) ◽  
pp. 470-473
Author(s):  
Anna Jakubczyc ◽  
Claus Neurohr
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Interstitielle Lungenerkrankung ◽  
Wird Eine ◽  
Antifibrotische Therapie ◽  
Allergische Alveolitis ◽  
Mycophenolat Mofetil

Was ist neu? Glukokortikosteroide und Immunsuppressiva In der Therapie der Lungengerüstveränderungen bei Patienten mit Kollagenose (connective tissue disease-associated interstitial lung disease, CTD-ILD) werden Kortikosteroide, Immunsuppressiva und auch Biologika angewendet. Im Vergleich zur Scleroderma-Lung-Study (SLS) I und II, die die Effektivität der Immunsuppressiva Cyclophosphamid (CYC) und Mycophenolat-Mofetil (MMF) bei pulmonaler Beteiligung der Sklerodermie (SSc-ILD) belegen, ist die Datenlage bezüglich der Therapie der anderen CTD-ILD unbefriedigend. Biologika In der Gruppe der Biologika nimmt Rituximab (RTX) an Bedeutung zu. Die aktuell erwarteten Studienergebnisse werden die Wirksamkeit von Immunsuppressiva (vor allem MMF und CYC) mit RTX bei verschiedenen CTD-ILD vergleichen. Zu den weiteren untersuchten Biologika gehören u. a. der monoklonale Anti-IL6-Rezeptor-Antikörper Tocilizumab bei SSc-ILD bzw. Abatacept (ein Fusionsprotein, das die T-Zell-Antworten inhibiert) bei pulmonaler Beteiligung bei rheumatoider Arthritis (RA-ILD). Autologe Stammzelltransplantation Die autologe Stammzelltransplantation ist eine potente, aber nebenwirkungsbehaftete Therapie bei schwerer Sklerodermie. Antifibrotische Therapie Für die Gruppe der Lungengerüstkrankheiten unterschiedlicher Genese inkl. CTD-ILD mit einem progredienten fibrosierenden Verlauf trotz adäquater Therapie (z. B. chronische exogen-allergische Alveolitis, RA, SSc) wurde der Begriff der fibrosierenden interstitiellen Lungenerkrankungen mit progressivem Phänotyp (progressive fibrosing interstitial lung diseases, PF-ILD) geprägt. In jüngster Zeit erschienen Arbeiten, die einen positiven Effekt der antifibrotischen Medikamente (Nintedanib, Pirfenidon) in einer solchen Situation nachweisen. Aktuell wird eine Reihe weiterer Ergebnisse bezüglich der Effektivität der Antifibrotika bei CTD-ILD erwartet. Die Studien auf diesem Gebiet haben das Potential der Erweiterung der Behandlungsmöglichkeiten bei CTD-ILD.

scholarly journals Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in Rheumatic Disorders: Still a Long Journey

2022 ◽  
Vol 11 (2) ◽  
pp. 410
Author(s):  
Marco Sebastiani ◽  
Caterina Vacchi ◽  
Giulia Cassone ◽  
Andreina Manfredi
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Clinical Features ◽  
Lung Diseases ◽  
Connective Tissue Diseases ◽  
Interstitial Lung Diseases ◽  
Pulmonary Complications ◽  
Autoimmune Rheumatic Diseases

Interstitial lung disease (ILD) is one of the most frequent pulmonary complications of autoimmune rheumatic diseases (ARDs), and it is mainly associated with connective tissue diseases (CTDs) and rheumatoid arthritis (RA) [...]

Idiopathic pulmonary fibrosis

2017 ◽  
Author(s):  
Clare Ross ◽  
Athol Wells
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Heterogeneous Group

Interstitial lung diseases are a complex heterogeneous group which are challenging to diagnose and treat. The diagnosis of idiopathic pulmonary fibrosis, as opposed to connective tissue interstitial lung disease, is important, as treatments and prognoses are very different. This chapter focusses on a case where this diagnosis is challenging. The evidence for the treatment of idiopathic pulmonary fibrosis is reviewed, in light of recent trials and new recommendations.

scholarly journals Antifibrotic Therapies and Progressive Fibrosing Interstitial Lung Disease (PF-ILD): Building on INBUILD

2021 ◽  
Vol 10 (11) ◽  
pp. 2285
Author(s):  
John N. Shumar ◽  
Abhimanyu Chandel ◽  
Christopher S. King
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Treatment Pathway ◽  
Antifibrotic Therapy ◽  
New Treatment ◽  
Fibrotic Lung Diseases

Progressive fibrosing interstitial lung disease (PF-ILD) describes a phenotypic subset of interstitial lung diseases characterized by progressive, intractable lung fibrosis. PF-ILD is separate from, but has radiographic, histopathologic, and clinical similarities to idiopathic pulmonary fibrosis. Two antifibrotic medications, nintedanib and pirfenidone, have been approved for use in patients with idiopathic pulmonary fibrosis. Recently completed randomized controlled trials have demonstrated the clinical efficacy of antifibrotic therapy in patients with PF-ILD. The validation of efficacy of antifibrotic therapy in PF-ILD has changed the treatment landscape for all of the fibrotic lung diseases, providing a new treatment pathway and opening the door for combined antifibrotic and immunosuppressant drug therapy to address both the fibrotic and inflammatory components of ILD characterized by mixed pathophysiologic pathways.

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