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Sheetu Singh, Bridget F Collins, Bharat B Sharma, Jyotsna M Joshi, Deepak Talwar, Sandeep Katiyar, Nishtha Singh, Lawrence Ho, Jai Kumar Samaria, Parthasarathi Bhattacharya, Rakesh Gupta, Sudhir Chaudhari, Tejraj Singh, Vijay Moond, Sudhakar Pipavath, Jitesh Ahuja, Ravindran Chetambath, Aloke G Ghoshal, Nirmal K Jain, Hj Gayathri Devi, Surya Kant, Parvaiz Koul, Raja Dhar, Rajesh Swarnakar, Suresh Kumar Sharma, Dhrubajyoti J Roy, Kripesh R Sarmah, Bhavin Jankharia, Rodney Schmidt, Santosh K Katiyar, Arpita Jindal, Daya K Mangal, Virendra Singh, Ganesh Raghu
RATIONALE: Interstitial lung disease (ILD) is a heterogeneous group of acute and chronic inflammatory and fibrotic lung diseases. Existing ILD registries have had variable findings. Little is known about the profile of ILDs in India. OBJECTIVES: Create a prospective Registry with validation of diagnoses by multidisciplinary discussion (MDD) to characterize the clinical profile of new onset-ILD in India. METHODS: Prospective recruitment of adult patients with new onset ILD (27 centers in 19 Indian cities, 3/2012-6/2015) with connective tissue disease (CTD) serologies and high-resolution computed tomography (HRCT) chest...
September 29, 2016: American Journal of Respiratory and Critical Care Medicine
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
Z Jiang, J-H Tao, T Zuo, X-M Li, G-S Wang, X Fang, X-L Xu, X-P Li
OBJECTIVES: To explore the correlation between microRNA (miR)-200c and the severity of interstitial lung disease (ILD) associated with connective tissue diseases (CTDs). METHOD: We recruited 218 patients with CTDs who were evaluated with high-resolution computed tomography (HRCT) and the pulmonary function test (PFT). Peripheral blood mononuclear cells (PBMCs) were acquired from 23 patients with systemic sclerosis (SSc), 29 with dermatomyositis/polymyositis (DM/PM), 30 with primary Sjögren's syndrome (pSS), 47 with rheumatoid arthritis (RA), and 23 normal controls to detect the expression level of miR-200c by quantitative reverse transcription polymerase chain reaction (QRT-PCR)...
June 16, 2016: Scandinavian Journal of Rheumatology
Justin M Oldham, Ayodeji Adegunsoye, Eleanor Valenzi, Cathryn Lee, Leah Witt, Lena Chen, Aliya N Husain, Steven Montner, Jonathan H Chung, Vincent Cottin, Aryeh Fischer, Imre Noth, Rekha Vij, Mary E Strek
Patients with interstitial lung disease (ILD) may have features of connective tissue disease (CTD), but lack findings diagnostic of a specific CTD. A recent European Respiratory Society/American Thoracic Society research statement proposed criteria for patients with interstitial pneumonia with autoimmune features (IPAF).We applied IPAF criteria to patients with idiopathic interstitial pneumonia and undifferentiated CTD-ILD (UCTD). We then characterised the clinical, serological and morphological features of the IPAF cohort, compared outcomes to other ILD cohorts and validated individual IPAF domains using survival as an endpoint...
June 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
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...
2016: BMC Pulmonary Medicine
Charles Sharp, Melanie McCabe, Nick Dodds, Anthony Edey, Lloyd Mayers, Huzaifa Adamali, Ann B Millar, Harsha Gunawardena
OBJECTIVE: CTD-associated interstitial lung disease (ILD) often fails to respond to conventional immunomodulatory agents. There is now considerable interest in the use of rituximab in systemic autoimmune CTD in patients refractory to standard treatments. The aim of this study was to review the experience of North Bristol NHS Trust managing patients with CTD-associated ILD with rituximab and explore possible associations with treatment response. METHODS: We conducted a retrospective analysis of all patients who received rituximab under the Bristol CTD-ILD service, having failed to respond to other immunomodulatory treatments...
July 2016: Rheumatology
Shan Jiang, Zhi Wang, Han Ouyang, Zhichun Liu, Lingyun Li, Yongbing Shi
INTRODUCTION: Connective tissues diseases (CTDs) are a heterogeneous group of disorders that share certain clinical characteristics and disturbed immunoregulation. Interstitial lung diseases (ILDs), also known as diffuse parenchymal lung diseases, are among the most serious pulmonary complications associated with CTDs. Interleukin 9 (IL-9), IL-4 and interferon γ (IFN-γ) - cytokines with important roles in autoimmune disease - were studied in CTD patients and CTD-ILD patients. MATERIAL AND METHODS: Sixty-one hospitalized untreated CTD patients were recruited, and 20 healthy volunteers were enrolled as controls...
February 1, 2016: Archives of Medical Science: AMS
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...
2016: BMJ: British Medical Journal
C Zhu, Y B Zhao, L F Kong, Z H Li, J Kang
OBJECTIVE: To detect the levels of KL-6 in the serum and the bronchoalveolar lavage fluid (BALF) of patients with different type of diffuse interstitial lung diseases(DILD), and to analyze its correlation with pulmonary function, pulmonary HRCT scores and other parameters. METHOD: Seventy-three patients with DILD were enrolled, including 34 patients with idiopathic pulmonary fibrosis (IPF), 10 patients with stage Ⅰ sarcoidosis(SAR Ⅰ), 15 patients with hypersensitivity pneumonia (HP), 14 patients with connective tissue diseases (CTD)...
February 2016: Chinese Journal of Tuberculosis and Respiratory Diseases
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]
Sandra Chartrand, Jeffrey J Swigris, Lina Peykova, Aryeh Fischer
OBJECTIVE: To describe our experience with rituximab (RTX) as treatment for a diverse spectrum of chronic connective tissue disease-associated interstitial lung disease (CTD-ILD). METHODS: Twenty-four subjects with CTD-ILD were included. All had pulmonary function testing before and after their first RTX infusion. Each subject was evaluated in a multidisciplinary autoimmune and ILD outpatient clinic. Data were extracted by retrospective review of complete medical records...
2015: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
Gwan Gyu Song, Sang-Cheol Bae, Young Ho Lee
OBJECTIVES: The purpose of this study was to evaluate and compare the diagnostic performance of lung ultrasound (US) in respect to high-resolution computed tomography (HRCT) findings in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). METHODS: We searched the Pubmed, Embase, and Cochrane Library databases, and performed a meta-analysis on the diagnostic accuracy of lung US according to B-lines (comet tail sign) and on the correlation coefficients between lung US scores and HRCT Warrick scores in CTD-ILD patients...
January 2016: Clinical and Experimental Rheumatology
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
Ekin Oktay Oguz, Orhan Kucuksahin, Murat Turgay, Mustafa Turgut Yildizgoren, Askin Ates, Nalan Demir, Ozlem Ozdemir Kumbasar, Gulay Kinikli, Nursen Duzgun
It was aimed to evaluate KL-6 glycoprotein levels to determine if it may be a diagnostic marker for the connective tissue diseases (CTDs) predicting CTD-related interstitial lung diseases (ILDs) (CTD-ILD) development and to examine if there was a difference between patients and healthy controls. The study included 113 patients with CTD (45 CTD without lung involvement, 68 CTD-ILD) and 45 healthy control subjects. KL-6 glycoprotein levels were analyzed with ELISA in patients and the control group. The relationship between KL-6 glycoprotein levels and CTD-ILD was assessed...
March 2016: Clinical Rheumatology
Joyce S Lee, Aryeh Fischer
The management of connective tissue disease-associated interstitial lung disease (CTD-ILD) is complex and this arena offers many challenges to the practicing clinician. Unfortunately, treatment strategies and recommendations are often based on experience rather than evidence, and there are few effective therapeutic options. Pharmacologic intervention with immunosuppression is usually the mainstay of therapy and is reserved for those with clinically significant and/or progressive ILD. There is a desperate need for controlled trials across the spectrum of CTD-ILD and a number of potentially promising novel therapies warrant further study...
2016: Expert Review of Clinical Immunology
Shikha Mittoo, Sid Frankel, Daphne LeSage, Vibeke Strand, Ami A Shah, Lisa Christopher-Stine, Sonye Danoff, Laura K Hummers, Jeffery J Swigris, Dörte Huscher, Angela M Christensen, Sophia L Cenac, Jen K Erbil, Sancia Ferguson, Ignacio Garcia-Valladares, Harmanjot K Grewal, Ana-Maria Orbai, Katherine Clegg Smith, Maithy Tran, Clifton O Bingham, Flavia V Castelino, Aryeh Fischer, Lesley Ann Saketkoo
OBJECTIVE: The impact and natural history of connective tissue disease related interstitial lung disease (CTD-ILD) are poorly understood; and have not been previously described from the patient's perspective. This investigation sought insight into CTD-ILD from the patients' perspective to add to our knowledge of CTD-ILD, identify disease-specific areas of unmet need and gather potentially meaningful information towards development of disease-specific patient-reported outcome measures (PROMs)...
June 2015: Current Respiratory Medicine Reviews
Yang Hu, Liu-Sheng Wang, Ya-Ru Wei, Shan-Shan Du, Yu-Kui Du, Xian He, Nan Li, Ying Zhou, Qiu-Hong Li, Yi-Liang Su, Fen Zhang, Li Shen, Dong Weng, Kevin K Brown, Hui-Ping Li
BACKGROUND: Because the prevalence of connective tissue disease (CTD)-associated interstitial lung disease (ILD; CTD-ILD) in China is unknown, we wanted to analyze the clinical characteristics of this disease in Chinese patients. METHODS: The medical records of patients who received a diagnosis of ILD and treated in Shanghai Pulmonary Hospital from January 1999 to January 2013 were reviewed. Based on the records, patients who also received a diagnosis of CTD were identified, and their records of follow-up examinations for a minimum of 12 months until the end of December 2013 were reviewed...
January 2016: Chest
Tsutomu Hamada, Takuya Samukawa, Tomohiro Kumamoto, Kazuhito Hatanaka, Go Tsukuya, Masuki Yamamoto, Kentaro Machida, Masaki Watanabe, Keiko Mizuno, Ikkou Higashimoto, Yoshikazu Inoue, Hiromasa Inoue
BACKGROUND: Interstitial lung diseases (ILDs) are common in patients with connective tissue diseases (CTDs). Although the diagnosis of an underlying CTD in ILD (CTD-ILD) affects both prognosis and treatment, it is sometimes difficult to distinguish CTD-ILD from chronic fibrosing interstitial pneumonia (CFIP). B cell-activating factor belonging to the tumour necrosis factor family (BAFF) plays a crucial role in B cell development, survival, and antibody production. METHODS: We examined serum levels of BAFF, surfactant protein D (SP-D), and Krebs von den Lungen-6 (KL-6) in 33 patients with CTD-ILD, 16 patients with undifferentiated CTD-ILD, 19 patients with CFIP, and 26 healthy volunteers...
2015: BMC Pulmonary Medicine
Aryeh Fischer, Sandra Chartrand
The intersection of the connective tissue diseases (CTD) and the interstitial lung diseases (ILD) is complex. Although often considered as a single entity, "CTD-ILD" actually reflects a heterogeneous spectrum of diverse CTDs and a variety of patterns of interstitial pneumonia. The evaluation of patients with CTD that develop ILD, or the assessment for underlying CTD in those presenting with presumed "idiopathic" ILD can be challenging and these evaluations can be optimized by effective multidisciplinary collaboration...
2015: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
Aryeh Fischer, Katerina M Antoniou, Kevin K Brown, Jacques Cadranel, Tamera J Corte, Roland M du Bois, Joyce S Lee, Kevin O Leslie, David A Lynch, Eric L Matteson, Marta Mosca, Imre Noth, Luca Richeldi, Mary E Strek, Jeffrey J Swigris, Athol U Wells, Sterling G West, Harold R Collard, Vincent Cottin
Many patients with an idiopathic interstitial pneumonia (IIP) have clinical features that suggest an underlying autoimmune process but do not meet established criteria for a connective tissue disease (CTD). Researchers have proposed differing criteria and terms to describe these patients, and lack of consensus over nomenclature and classification limits the ability to conduct prospective studies of a uniform cohort.The "European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease" was formed to create consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity...
October 2015: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
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