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https://www.readbyqxmd.com/read/29789305/ro-positive-interstitial-lung-disease-treated-with-cyclophosphamide
#1
Nader Habib Bedwani, Natasha Jefferson, Christopher Marguerie, Jayanta Mukherjee
Interstitial lung disease (ILD) comprises a spectrum of conditions involving inflammation and/or fibrosis of the alveolar wall causing limitation in gaseous exchange. Treatment varies depending on the underlying ILD. We describe the case of a woman presenting with a productive cough who was diagnosed with community-acquired pneumonia. While on the ward she developed type-1 respiratory failure requiring continuous positive airway pressure and intensive care unit admission. Failing to respond to targeted antimicrobials she was investigated by chest high-resolution CT and autoantibody screen to identify non-infective causes of her respiratory signs and symptoms...
May 22, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29788800/relationship-between-ykl-40-and-pulmonary-arterial-hypertension-in-systemic-sclerosis
#2
Tetsuya Furukawa, Kiyoshi Matsui, Masayasu Kitano, Yuichi Yokoyama, Masahiro Sekiguchi, Naoto Azuma, Yasutomo Imai, Seiichi Hirota, Kiyofumi Yamanishi, Hajime Sano
OBJECTIVES: Systemic sclerosis (SSc) is an intractable connective tissue disease that causes skin and organ fibrosis. Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) affect its prognosis. YKL-40 protein impacts inflammation and tissue remodeling. Therefore, we evaluated the utility of YKL-40 blood levels in identifying patients with SSc complicated by PAH, as confirmed by immunohistochemistry (IHC) examination. METHODS: We retrospectively analyzed 78 patients with SSc and performed IHC on 7 normal and 7 SSc skin samples in the Japanese population...
May 23, 2018: Modern Rheumatology
https://www.readbyqxmd.com/read/29777409/hemodynamic-heterogeneity-of-connective-tissue-disease-patients-with-borderline-mean-pulmonary-artery-pressure-and-its-distinctive-characters-from-those-with-normal-pulmonary-artery-pressure-a-retrospective-study
#3
Yusa Asari, Yoshioki Yamasaki, Kosei Tsuchida, Kengo Suzuki, Yoshihiro J Akashi, Takahiro Okazaki, Shoichi Ozaki, Hidehiro Yamada, Kimito Kawahata
To clarify whether patients with connective tissue disease (CTD)-associated borderline mean pulmonary artery pressure (mPAP) have distinctive hemodynamic characteristics from those with normal mPAP and whether pathogenesis is as heterogeneous as manifest pulmonary hypertension (PH). Seventy-five CTD patients who underwent right heart catheterization (RHC) from 2008 through 2016 were retrospectively analyzed. We compared between-group differences in clinical and hemodynamic findings: normal mPAP (n = 35), borderline mPAP (n = 15), and PH (n = 25)...
May 18, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29776440/radiological-pleuroparenchymal-fibroelastosis-associated-to-limited-cutaneous-systemic-sclerosis-a-case-report
#4
D Hassoun, S Dirou, P P Arrigoni, C Durant, M Hamidou, A Néel, C Agard
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...
May 18, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29773465/pulmonary-manifestations-in-systemic-lupus-erythematosus-pleural-involvement-acute-pneumonitis-chronic-interstitial-lung-disease-and-diffuse-alveolar-hemorrhage
#5
Georgina Aguilera-Pickens, Carlos Abud-Mendoza
Systemic lupus erythematosus is the diffuse autoimmune connective tissue disease that most frequently involves pulmonary involvement, affecting 20% of 90% of the patients. The percentage varies depending on the defining criteria (symptoms, pulmonary tests or histopathological studies). At least once during the disease course, 50% of those affected have pleural and/or pulmonary manifestations, which are associated with higher morbidity and mortality. Pulmonary involvement has no correlation with lupus activity biomarkers, and it is necessary to rule out infectious processes in the initial approach...
May 14, 2018: Reumatología Clinica
https://www.readbyqxmd.com/read/29745875/interstitial-lung-disease-in-patients-with-mixed-connective-tissue-disease-pilot-study-on-predictors-of-lung-involvement
#6
Neha Narula, Tathagat Narula, Isabel Mira-Avendano, Benjamin Wang, Andy Abril
OBJECTIVES: Mixed connective tissue disease (MCTD) is an immune-mediated systemic disorder characterised by serum autoantibodies against U1-ribonucleoprotein and diverse multisystemic clinical manifestations. Approximately 50% of patients with MCTD develop a radiologic pattern of interstitial lung disease (ILD). Our single centre, cross-sectional study sought to identify clinical and serologic associations of ILD in patients with MCTD which may serve as predictors of lung disease and prognosis...
May 8, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29720509/state-of-the-art-in-interstitial-pneumonia-with-autoimmune-features-a-systematic-review-on-retrospective-studies-and-suggestions-for-further-advances
#7
REVIEW
Gianluca Sambataro, Domenico Sambataro, Sebastiano Emanuele Torrisi, Ada Vancheri, Mauro Pavone, Roberta Rosso, Matteo Schisano, Claudia Crimi, Francesca Pignataro, Aryeh Fischer, Nicoletta Del Papa, Carlo Vancheri
The term interstitial pneumonia with autoimmune features (IPAF) has been proposed to define patients with interstitial lung disease (ILD) associated with autoimmune signs not classifiable for connective tissue diseases (CTDs). This new definition overcomes previous nomenclatures and provides a uniform structure for prospective studies through specific classification criteria.This work evaluates the characteristics of IPAF patients reported in the literature, to highlight potential limits through a comparative analysis and to suggest better performing classification criteria...
June 30, 2018: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/29712908/the-novel-hsa-mir-12528-regulates-tumourigenesis-and-metastasis-through-hypo-phosphorylation-of-akt-cascade-by-targeting-igf-1r-in-human-lung-cancer
#8
Seong Ho Jeon, Jung Ki Yoo, Chang Min Kim, Eun Su Lim, So Jeong Lee, Ji Min Lee, Seung-Hun Oh, Jin Kyeoung Kim
Lung cancer cases are increasing yearly; however, few novel therapeutic strategies for treating this disease have been developed. Here the dysregulation between microRNAs and oncogenes or tumour-suppressor genes forms a close connection-loop to the development or progression in human lung carcinogenesis. That is, the relationship between microRNAs and carcinogenic mechanism may find the critical clue to improve the treatment efficacy. Accordingly, we identified and characterised a novel microRNA, hsa-miR-12528, in A549 cells...
May 1, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29707304/comparison-of-short-term-outcomes-for-connective-tissue-disease-related-interstitial-lung-disease-and-idiopathic-pulmonary-fibrosis-after-lung-transplantation
#9
Ji Eun Park, Song Yee Kim, Joo Han Song, Young Sam Kim, Joon Chang, Jin Gu Lee, Hyo Chae Paik, Moo Suk Park
Background: Pulmonary involvement is common in connective tissue disease (CTD), and respiratory failure is a major cause of morbidity and mortality in CTD-related interstitial lung disease (CTD-ILD). Lung transplantation is thus important for these patients. However, survival, outcomes, and management of these patients after transplantation have been debated. The aim of this study was to evaluate the outcomes for CTD-ILD compared to those for idiopathic pulmonary fibrosis (IPF) after lung transplantation...
March 2018: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29693298/prognostic-factors-and-disease-behaviour-of-pathologically-proven-fibrotic-non-specific-interstitial-pneumonia
#10
Hideaki Yamakawa, Hideya Kitamura, Tamiko Takemura, Satoshi Ikeda, Akimasa Sekine, Tomohisa Baba, Tae Iwasawa, Eri Hagiwara, Shinji Sato, Takashi Ogura
BACKGROUND AND OBJECTIVE: Non-specific interstitial pneumonia (NSIP) has heterogeneous characteristics in terms of background, disease behaviour and prognosis. This study of fibrotic NSIP (f-NSIP) aimed to elucidate prognosis and disease behaviour from the viewpoint of clinical background and determine whether long-term change of pulmonary function could provide useful prognostic information. METHODS: We analysed the medical records of 157 consecutive patients diagnosed with f-NSIP by surgical lung biopsy...
April 24, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29683732/lncrna-pfal-promotes-lung-fibrosis-through-ctgf-by-competitively-binding-mir-18a
#11
Xuelian Li, Tong Yu, Huitong Shan, Hua Jiang, Jian Sun, Xiaoguang Zhao, Wei Su, Lida Yang, Hongli Shan, Haihai Liang
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic parenchymal lung disease of unknown etiology and lacks an effective intervention. Long noncoding RNAs (lncRNAs) participate in organ fibrosis and various pulmonary diseases, but the role of lncRNAs in lung fibrosis is not fully understood. In the present study, we identified that lncRNA NONMMUT021928, designated as pulmonary fibrosis-associated lncRNA (PFAL), was up-regulated in the lungs of mice with experimental lung fibrosis, and in TGF-β1-induced fibrotic lung fibroblasts...
April 23, 2018: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29667101/clinical-features-risk-factors-and-outcomes-of-patients-with-interstitial-pneumonia-with-autoimmune-features-a-population-based-study
#12
Jinghong Dai, Lei Wang, Xin Yan, Hui Li, Kefeng Zhou, Jian He, Fanqing Meng, Siyi Xu, Geyu Liang, Hourong Cai
To investigate the clinical features, risk factors and outcomes of patients with interstitial pneumonia with autoimmune features (IPAF). A total of 1429 patients with idiopathic interstitial pneumonia (IIP) and undifferentiated connective tissue disease-associated interstitial lung disease (UCTD-ILD) were screened to identify patients who met IPAF criteria. Clinical, serological, and morphological features of patients with IPAF were characterized. Outcomes between patients with IPAF, UCTD-ILD, and IIP who were divided into idiopathic pulmonary fibrosis (IPF) and non-IPF groups were compared using survival as an endpoint...
April 18, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29619932/mortality-and-morbidity-in-patients-with-osteogenesis-imperfecta-in-denmark
#13
Lars Folkestad
Ostegenesis imperfecta (OI) is a hereditary disease of the connective tissue caused by mutations to, mainly, the genes that are involved in the biosynthesis of collagen type 1. Patients are grouped according to clinical severity and mode of inheritance according to Sillence's classification (originally 1979, updated 2014). According to our data, the population prevalence of OI in Denmark was 10.3 per 100,000, with 575 patients registered with an OI diagnosis in the National Patient Register and alive at the end of 2012 out of a total population of 5,602,628 persons...
April 2018: Danish Medical Journal
https://www.readbyqxmd.com/read/29607151/lung-cancer-in-connective-tissue-disease-associated-interstitial-lung-disease-clinical-features-and-impact-on-outcomes
#14
Satoshi Watanabe, Keigo Saeki, Yuko Waseda, Akari Murata, Hazuki Takato, Yukari Ichikawa, Masahide Yasui, Hideharu Kimura, Yasuhito Hamaguchi, Takashi Matsushita, Kazunori Yamada, Mitsuhiro Kawano, Kengo Furuichi, Takashi Wada, Kazuo Kasahara
Backgrounds: Lung cancer (LC) adversely impacts survival in patients with idiopathic pulmonary fibrosis. However, little is known about LC in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). The aim of this study was to evaluate the prevalence of and risk factors for LC in CTD-ILD, and the clinical characteristics and survival of CTD-ILD patients with LC. Methods: We conducted a single-center, retrospective review of patients with CTD-ILD from 2003 to 2016...
February 2018: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29605286/pulmonary-hypertension-in-patients-with-interstitial-lung-disease
#15
REVIEW
Theodoros Karampitsakos, Argyrios Tzouvelekis, Serafeim Chrysikos, Demosthenes Bouros, Iraklis Tsangaris, Wassim H Fares
Interstitial lung diseases (ILDs) comprise a broad and heterogeneous group of more than two hundred diseases with common functional characteristics. Their diagnosis and management require a multidisciplinary approach. This multidisciplinary approach involves the assessment of comorbid conditions including pulmonary hypertension (PH) that exerts a dramatic impact on survival. The current World Health Organization (WHO) classification of PH encompasses many of the interstitial lung diseases into WHO Group 3, while sarcoidosis, Pulmonary Langerhans Cell Histiocytosis and lymphangioleiomyomatosis are placed into WHO Group 5 as diseases with unclear or multifactorial mechanisms...
March 29, 2018: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29605201/distinctive-characteristics-and-prognostic-significance-of-interstitial-pneumonia-with-autoimmune-features-in-patients-with-chronic-fibrosing-interstitial-pneumonia
#16
Katsuhiro Yoshimura, Masato Kono, Yasunori Enomoto, Koji Nishimoto, Yoshiyuki Oyama, Hideki Yasui, Hironao Hozumi, Masato Karayama, Yuzo Suzuki, Kazuki Furuhashi, Noriyuki Enomoto, Tomoyuki Fujisawa, Yutaro Nakamura, Naoki Inui, Hiromitsu Sumikawa, Takeshi Johkoh, Thomas V Colby, Haruhiko Sugimura, Takafumi Suda
BACKGROUND: Interstitial lung diseases are heterogeneous, and patients with chronic fibrosing interstitial pneumonia (CFIP) often have clinical, serologic, and morphologic features suggestive but not diagnostic of connective tissue disease. Recently, the concept of interstitial pneumonia with autoimmune features (IPAF) has been proposed as a platform for such patients. However, the prognostic role of IPAF, including the cumulative incidence of acute exacerbations (AEs), is not fully clear...
April 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29601564/-nintedanib-in-the-treatment-of-fibrosing-interstital-lung-diseases
#17
REVIEW
Katarzyna Lewandowska
Nintedanib is an intracellular tyrosine kinase inhibitor approved in a treatment of idiopathic pulmonary fibrosis. It reduces the annual rate of forced vital capacity decline by approximately 50%, that results in slowing of disease progression. The drug also reduces the incidence of acute exacerbations of idiopathic pulmonary fibrosis. The efficacy of nintedanib was the same in different groups of patients, irrespectively to disease status, age, gender and race. The most frequent side effects were gastrointestinal, i...
March 27, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29599600/an-enigmatic-clinical-presentation-of-plasma-cell-granuloma-of-the-oral-cavity
#18
Pravesh Kumar Jhingta, Kavita Mardi, Deepak Sharma, Vinay Kumar Bhardwaj, Ashu Bhardwaj, Nitin Saroch, Nishant Negi
Plasma cell granuloma is a rare benign lesion characterized by the infiltration of plasma cells; primarily occurring in the lungs. It is also seen to occur in the brain, kidney stomach, heart, and so on but its intraoral occurrence is a rarity. This case report represents one of the uncommon locations in the oral cavity affected by plasma cell granuloma, its clinical and histological features, and establishes the differential diagnosis with other malignant or benign disease entities and planning the treatment accordingly...
January 2018: Contemporary Clinical Dentistry
https://www.readbyqxmd.com/read/29595279/-systemic-sclerosis-in-2017
#19
Tomáš Soukup, Tomáš Veleta
Systemic sclerosis is classed as a diffuse (systemic) disease of connective tissue. It is a heterogeneous disease significantly shortening life expectancy. Its etiology is unknown. Pathogenetic interplay is assumed to involve a triad of pathological autoimmune inflammation, vasculopathy and fibrosis. Clinical manifestations can be classed based on the preponderant pathogenetic process. Vasculopathy is manifested by secondary Raynauds phenomenon with abnormal findings on the nailfold capillaroscopy, skin telangiectasias, gastric antral vascular ectasia, life threatening scleroderma renal crisis, digital ulcerations and prognostically severe pulmonary arterial hypertension...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29595014/-invasive-diagnostic-techniques-for-diffuse-interstitial-pneumopathies
#20
B Duysinx, J Guiot, I Pellegrini, R Louis, J L Corhay, V Heinen
Interstitial lung diseases represent a very heterogeneous group of diseases mainly affecting connective lung tissue even if alveolar space may sometimes be involved. The identification of their etiology is the key stage in their management. It requires the integration of anamnestic, clinical, biological, radiological data and, sometimes relies on, cytology or histology. In this review, we assess the contribution and feasibility of the different invasive techniques used for interstitial lung disease diagnosis...
March 2018: Revue Médicale de Liège
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