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Connective tissue disease related ILD

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https://www.readbyqxmd.com/read/28275485/serum-kl-6-and-surfactant-protein-d-as-monitoring-and-predictive-markers-of-interstitial-lung-disease-in-patients-with-systemic-sclerosis-and-mixed-connective-tissue-disease
#1
Hideaki Yamakawa, Eri Hagiwara, Hideya Kitamura, Yumie Yamanaka, Satoshi Ikeda, Akimasa Sekine, Tomohisa Baba, Koji Okudela, Tae Iwasawa, Tamiko Takemura, Kazuyoshi Kuwano, Takashi Ogura
BACKGROUND: Interstitial lung disease (ILD) is frequent complication of systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The disease is heterogeneous, and its outcome is unpredictable. Some patients have severe and progressive deterioration of ILD, which is the leading cause of mortality. We aimed to determine whether serum levels of Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) correlate with SSc/MCTD-associated ILD activity. METHODS: We retrospectively analyzed the medical records of 40 patients with SSc/MCTD-associated ILD: 29 patients with SSc and 11 patients with MCTD...
February 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28213592/the-evidence-of-benefits-of-exercise-training-in-interstitial-lung-disease-a-randomised-controlled-trial
#2
Leona M Dowman, Christine F McDonald, Catherine J Hill, Annemarie L Lee, Kathryn Barker, Claire Boote, Ian Glaspole, Nicole S L Goh, Anne M Southcott, Angela T Burge, Rebecca Gillies, Alicia Martin, Anne E Holland
BACKGROUND: Uncertainty exists regarding the clinical relevance of exercise training across the range of interstitial lung diseases (ILDs). OBJECTIVE: To establish the impact of exercise training in patients with ILDs of differing aetiology and severity. METHODS: 142 participants with ILD (61 idiopathic pulmonary fibrosis (IPF), 22 asbestosis, 23 connective tissue disease-related ILD (CTD-ILD) and 36 with other aetiologies) were randomised to either 8 weeks of supervised exercise training or usual care...
February 17, 2017: Thorax
https://www.readbyqxmd.com/read/27979657/undifferentiated-connective-tissue-disease-and-interstitial-lung-disease-trying-to-define-patterns
#3
María Laura Alberti, Francisco Paulin, Heidegger Mateos Toledo, Martín Eduardo Fernández, Fabián Matías Caro, Jorge Rojas-Serrano, Mayra Edith Mejía
OBJECTIVES: To identify clinical or immunological features in patients with undifferentiated connective tissue disease (UCTD) associated interstitial lung disease (ILD), in order to group them and recognize different functional and high resolution computed tomography (HRCT) behavior. METHODS: Retrospective cohort study. Patients meeting Kinder criteria for UCTD were included. We defined the following predictive variables: 'highly specific' connective tissue disease (CTD) manifestations (Raynaud's phenomenon, dry eyes or arthritis), high antinuclear antibody (ANA) titer (above 1: 320), and 'specific' ANA staining patterns (centromere, cytoplasmic and nucleolar patterns)...
December 12, 2016: Reumatología Clinica
https://www.readbyqxmd.com/read/27977621/lung-cancer-development-in-patients-with-connective-tissue-disease-related-interstitial-lung-disease-a-retrospective-observational-study
#4
Yasunori Enomoto, Naoki Inui, Katsuhiro Yoshimura, Koji Nishimoto, Kazutaka Mori, Masato Kono, Tomoyuki Fujisawa, Noriyuki Enomoto, Yutaro Nakamura, Toshihide Iwashita, Takafumi Suda
Previous studies have reported that patients with idiopathic pulmonary fibrosis occasionally develop lung cancer (LC). However, in connective tissue disease (CTD)-related interstitial lung disease (ILD), there are few data regarding the LC development. The aim of the present study was to evaluate the clinical significance of LC development in patients with CTD-ILD. A retrospective review of our database of 562 patients with ILD between 2000 and 2014 identified 127 patients diagnosed with CTD-ILD. The overall and cumulative incidences of LC were calculated...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27876024/evaluation-of-computer-based-computer-tomography-stratification-against-outcome-models-in-connective-tissue-disease-related-interstitial-lung-disease-a-patient-outcome-study
#5
Joseph Jacob, Brian J Bartholmai, Srinivasan Rajagopalan, Anne Laure Brun, Ryoko Egashira, Ronald Karwoski, Maria Kokosi, Athol U Wells, David M Hansell
BACKGROUND: To evaluate computer-based computer tomography (CT) analysis (CALIPER) against visual CT scoring and pulmonary function tests (PFTs) when predicting mortality in patients with connective tissue disease-related interstitial lung disease (CTD-ILD). To identify outcome differences between distinct CTD-ILD groups derived following automated stratification of CALIPER variables. METHODS: A total of 203 consecutive patients with assorted CTD-ILDs had CT parenchymal patterns evaluated by CALIPER and visual CT scoring: honeycombing, reticular pattern, ground glass opacities, pulmonary vessel volume, emphysema, and traction bronchiectasis...
November 23, 2016: BMC Medicine
https://www.readbyqxmd.com/read/27749242/effects-of-rituximab-in-connective-tissue-disorders-related-interstitial-lung-disease
#6
MULTICENTER STUDY
Gemma Lepri, Jerome Avouac, Paolo Airò, Francisco Anguita Santos, Silvia Bellando-Randone, Jelena Blagojevic, Francisco Garcia Hernàndez, Jose Antonio Gonzalez Nieto, Serena Guiducci, Suzana Jordan, Vidya Limaye, Britta Maurer, Albert Selva-O'Callaghan, Valeria Riccieri, Oliver Distler, Marco Matucci-Cerinic, Yannick Allanore
OBJECTIVES: Interstitial lung disease (ILD) is a key prognostic factor in connective tissue disorders (CTDs). The aim of our study was to assess the changes in pulmonary functional tests (PFTs) in various CTDs, including anti-synthetase syndrome (SYN), systemic sclerosis (SSc) and mixed connective tissue disorder (MCTD), following the use of rituximab therapy. METHODS: A multicentre retrospective analysis of patients with ILD secondary to SYN (n=15), MCTD (n=6) and SSc (n=23)...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27682649/autoantibody-to-scaffold-attachment-factor-b-safb-a-novel-connective-tissue-disease-related-autoantibody-associated-with-interstitial-lung-disease
#7
Akiko Takeuchi, Takashi Matsushita, Kenzo Kaji, Yoshinobu Okamoto, Masahide Yasui, Masayoshi Hirata, Naoto Oishi, Akira Higashi, Mariko Seishima, Tomoya Asano, Manabu Fujimoto, Masataka Kuwana, Kazuhiko Takehara, Yasuhito Hamaguchi
OBJECTIVE: To identify and characterize a novel connective tissue disease (CTD)-related autoantibody (autoAb) directed against scaffold attachment factor B (SAFB). METHODS: AutoAb specificity was analyzed using RNA and protein-immunoprecipitation assays. Autoimmune targets were affinity purified using patients' sera and subjected to liquid chromatography mass spectrometry. RESULTS: By immunoprecipitation assay, 10 sera reacted with a protein with a molecular weight of approximately 160 kDa...
January 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/27564852/clinical-features-of-idiopathic-interstitial-pneumonia-with-systemic-sclerosis-related-autoantibody-in-comparison-with-interstitial-pneumonia-with-systemic-sclerosis
#8
Hideaki Yamakawa, Eri Hagiwara, Hideya Kitamura, Yumie Yamanaka, Satoshi Ikeda, Akimasa Sekine, Tomohisa Baba, Shinichiro Iso, Koji Okudela, Tae Iwasawa, Tamiko Takemura, Kazuyoshi Kuwano, Takashi Ogura
BACKGROUND: Patients with idiopathic interstitial pneumonias sometimes have a few features of connective tissue disease (CTD) and yet do not fulfil the diagnostic criteria for any specific CTD. OBJECTIVE: This study was conducted to elucidate the characteristics, prognosis, and disease behavior in patients with interstitial lung disease (ILD) associated with systemic sclerosis (SSc)-related autoantibodies. METHODS: We retrospectively analyzed medical records of 72 ILD patients: 40 patients with SSc (SSc-ILD) and 32 patients with SSc-related autoantibody-positive ILD but not with CTD (ScAb-ILD), indicating lung-dominant CTD with SSc-related autoantibody...
2016: PloS One
https://www.readbyqxmd.com/read/27540018/telomere-related-lung-fibrosis-is-diagnostically-heterogeneous-but-uniformly-progressive
#9
Chad A Newton, Kiran Batra, Jose Torrealba, Julia Kozlitina, Craig S Glazer, Carlos Aravena, Keith Meyer, Ganesh Raghu, Harold R Collard, Christine Kim Garcia
Heterozygous mutations in four telomere-related genes have been linked to pulmonary fibrosis, but little is known about similarities or differences of affected individuals.115 patients with mutations in telomerase reverse transcriptase (TERT) (n=75), telomerase RNA component (TERC) (n=7), regulator of telomere elongation helicase 1 (RTEL1) (n=14) and poly(A)-specific ribonuclease (PARN) (n=19) were identified and clinical data were analysed.Approximately one-half (46%) had a multidisciplinary diagnosis of idiopathic pulmonary fibrosis (IPF); others had unclassifiable lung fibrosis (20%), chronic hypersensitivity pneumonitis (12%), pleuroparenchymal fibroelastosis (10%), interstitial pneumonia with autoimmune features (7%), an idiopathic interstitial pneumonia (4%) and connective tissue disease-related interstitial fibrosis (3%)...
December 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/27521738/therapeutic-approach-to-adult-fibrotic-lung-diseases
#10
REVIEW
Ayodeji Adegunsoye, Mary E Strek
Among the interstitial lung diseases (ILDs), idiopathic pulmonary fibrosis (IPF), chronic hypersensitivity pneumonitis, and fibrotic connective tissue disease-related ILD are associated with a worse prognosis, with death occurring as a result of both respiratory failure and serious associated comorbidities. The recent development and approval of the antifibrotic agents nintedanib and pirfenidone, both of which reduced the rate of decline in lung function in patients with IPF in clinical trials, offer hope that it may be possible to alter the increased mortality associated with IPF...
December 2016: Chest
https://www.readbyqxmd.com/read/27421219/mixed-connective-tissue-disease
#11
REVIEW
Ragnar Gunnarsson, Siri Opsahl Hetlevik, Vibke Lilleby, Øyvind Molberg
The concept of mixed connective tissue disease (MCTD) as a separate connective tissue disease (CTD) has persisted for more than four decades. High titers of antibodies targeting the U1 small nuclear ribonucleoprotein particle (U1 snRNP) in peripheral blood are a sine qua non for the diagnosis of MCTD, in addition to distinct clinical features including Raynaud's phenomenon (RP), "puffy hands," arthritis, myositis, pleuritis, pericarditis, interstitial lung disease (ILD), and pulmonary hypertension (PH). Recently, population-based epidemiology data from Norway estimated the point prevalence of adult-onset MCTD to be 3...
February 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27421215/connective-tissue-disease-related-interstitial-lung-disease
#12
REVIEW
M Kristen Demoruelle, Shikha Mittoo, Joshua J Solomon
Interstitial lung disease (ILD) is commonly present in patients with an underlying connective tissue disease (CTD), particularly those with systemic sclerosis, rheumatoid arthritis, and inflammatory myositis. The clinical spectrum can range from asymptomatic findings on imaging to respiratory failure and death. Distinguishing features in the clinical, radiographic, and histopathologic characteristics of CTD-ILD subsets can predict prognosis and treatment response. Treatment often consists of combinations of immunosuppressive medications, but there is a paucity of guidance in the literature to help clinicians determine appropriate screening and management of CTD-ILD...
February 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27193471/myositis-specific-and-myositis-associated-autoantibodies-in-indian-patients-with-inflammatory-myositis
#13
Puja Srivastava, Sanjay Dwivedi, Ramnath Misra
We aimed to study the prevalence and clinical associations of myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs) in a large cohort of Indian patients with idiopathic inflammatory myositis (IIM). Clinical details and serum samples were collected from patients with IIM (satisfying Bohan and Peter Criteria, 1975) and CTD-associated myositis. Sera were analysed for antibodies against SRP, Mi2, Jo1, PL7, PL12, EJ, OJ, Ro52, Ku, Pm-Scl 75 and PM-Scl 100, using immunoblot assay. The cohort comprised 124 patients with IIM (M:F = 1:3...
July 2016: Rheumatology International
https://www.readbyqxmd.com/read/27094018/mean-pulmonary-arterial-pressure-as-a-prognostic-indicator-in-connective-tissue-disease-associated-with-interstitial-lung-disease-a-retrospective-cohort-study
#14
Kota Takahashi, Hiroyuki Taniguchi, Masahiko Ando, Koji Sakamoto, Yasuhiro Kondoh, Naohiro Watanabe, Tomoki Kimura, Kensuke Kataoka, Atsushi Suzuki, Satoru Ito, Yoshinori Hasegawa
BACKGROUND: Pulmonary hypertension (PH) can develop in connective tissue disease associated interstitial lung disease (CTD-ILD), and contributes to increased morbidity and mortality. However, except for systemic sclerosis and mixed connective tissue disease, the impact of mean pulmonary arterial pressure (MPAP) on survival in CTD-ILD has not been sufficiently elucidated. We hypothesized that pulmonary arterial pressure may be a prognostic factor in CTD-ILDs regardless of the kind of CTD...
April 19, 2016: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/27027811/management-of-connective-tissue-diseases-associated-interstitial-lung-disease-a-review-of-the-published-literature
#15
REVIEW
Beth Wallace, Dharshan Vummidi, Dinesh Khanna
PURPOSE OF REVIEW: Interstitial lung disease (ILD), though a common and often a severe manifestation of many connective tissue diseases (CTD), is challenging to manage because of its variable presentation and the relative lack of guidelines to assist the clinician. In this review, we discuss the approach to diagnosis, treatment, and monitoring patients with CTD-associated ILD, with a focus on systemic sclerosis (SSc), rheumatoid arthritis (RA), and idiopathic inflammatory myopathy (IIM)...
May 2016: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/26933302/antisynthetase-syndrome-an-under-recognized-cause-of-interstitial-lung-disease
#16
Venkata Nagarjuna Maturu, Arjun Lakshman, Amanjit Bal, Varun Dhir, Aman Sharma, Mandeep Garg, Biman Saikia, Ritesh Agarwal
BACKGROUND: Antisynthetase syndrome (AS) is an uncommon and under-recognised connective tissue disease characterized by the presence of antibodies to anti-aminoacyl t-RNA synthetase along with features of interstitial lung disease (ILD), myositis and arthritis. The aim of the current study is to describe our experience with management of AS. MATERIALS AND METHODS: This was a 2-year (2013-2014) retrospective analysis of patients diagnosed with anti-Jo-1-related AS...
January 2016: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/26921132/the-use-of-auto-antibody-testing-in-the-evaluation-of-interstitial-lung-disease-ild-a-practical-approach-for-the-pulmonologist
#17
REVIEW
Thomas Bahmer, Micaela Romagnoli, Francesco Girelli, Martin Claussen, Klaus F Rabe
Interstitial lung diseases (ILD), also defined as diffuse parenchymal lung diseases (DPLD) include a heterogeneous group of pulmonary disorders. They may be caused by an underlying connective tissue disease (CTD), Rheumatoid Arthritis (RA) or ANCA-associated Vasculitis (AAV). Pulmonary manifestations of these conditions may also precede systemic onset and therefore, pulmonologists may be confronted with diagnosing a systemic rheumatic disease. For the discrimination of CTD-related ILD and idiopathic interstitial pneumonia (IIP), serological testing is recommended...
April 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/26912511/management-of-interstitial-lung-disease-associated-with-connective-tissue-disease
#18
REVIEW
Stephen C Mathai, Sonye K Danoff
The lung is a common site of complications of systemic connective tissue disease (CTD), and lung involvement can present in several ways. Interstitial lung disease (ILD) and pulmonary hypertension are the most common lung manifestations in CTD. Although it is generally thought that interstitial lung disease develops later on in CTD it is often the initial presentation ("lung dominant" CTD). ILD can be present in most types of CTD, including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, polymyositis or dermatomyositis, Sjögren's syndrome, and mixed connective tissue disease...
February 24, 2016: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/26849923/-randomized-control-multi-center-clinical-study-of-mycophenolate-mofetil-and-cyclophosphamide-in-the-treatment-of-connective-tissue-disease-related-interstitial-lung-disease
#19
RANDOMIZED CONTROLLED TRIAL
Guangfeng Zhang, Ting Xu, Hongwei Zhang, Shanhui Ye, Qingwen Wang, Lijun Zhang, Yunxia Lei, Riqiang Luo, Xiao Zhang
OBJECTIVE: To investigate the efficacy and safety of mycophenolate mofetil (MMF) in the treatment of connective tissue disease-related interstitial lung disease (CTD-ILD). METHODS: A total of 60 patients with CTD-ILD, confirmed by high resolution computer tomography (HRCT), were enrolled from five clinical centers from July 2010 to July 2014. In addition to the basic glucocorticoid treatment, patients received intravenous cyclophosphamide (Group A) or oral MMF (Group B) for one year...
December 1, 2015: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/26762710/benefit-of-adjunctive-tacrolimus-in-connective-tissue-disease-interstitial-lung-disease
#20
Leah J Witt, Carley Demchuk, James J Curran, Mary E Strek
We evaluated the safety and effectiveness of adjunctive tacrolimus therapy with conventional immunosuppression in patients with severe connective tissue disease-related interstitial lung disease (CTD-ILD). We included patients from our interstitial lung disease (ILD) registry with CTD-ILD, in whom tacrolimus was added to corticosteroids and an additional immunosuppressive agent. Demographic data, clinical features, lung function, radiographic images, and pathologic findings were reviewed. Effectiveness was assessed by comparing pulmonary function tests (PFTs) closest to tacrolimus initiation to PFTs approximately 6-12 months later...
February 2016: Pulmonary Pharmacology & Therapeutics
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