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connective tissue disease-interstitial lung disease associated

Joanna E Kusmirek, Maria Daniela Martin, Jeffrey P Kanne
Idiopathic interstitial pneumonias are a heterogeneous group of diffuse lung diseases characterized by distinct clinicopathologic entities with the usual interstitial pneumonia (UIP) being the most common. The pattern of UIP can be seen in idiopathic pulmonary fibrosis (IPF) as well as in secondary causes, most commonly in connective tissue diseases. IPF is usually progressive and associated with a very poor prognosis, and newer therapies pose a risk of serious complications; therefore, diagnostic certainty is crucial...
November 2016: Radiologic Clinics of North America
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...
September 24, 2016: Journal of Autoimmunity
Benjamin James Kurth, Vanya Wagler, Michael Keith
In connective tissue disorders, the incidence of pericardial disease and pericardial effusion can be up to 58%, and if untreated, it can lead to cardiac tamponade which can be fatal. Physicians must have a high index of suspicion for this disease as diagnosis can be delayed while evaluating more common causes of tachycardia and hypotension in the immunosuppressed (ie, sepsis). We present a 55-year-old woman with a severe case of dermatomyositis, marked by significant weight loss, a bedridden state and hallmark cutaneous findings...
2016: BMJ Case Reports
Kamonpun Ussavarungsi, Ryan M Kern, Anja C Roden, Jay H Ryu, Eric S Edell
BACKGROUND: Diagnostic evaluation of patients with diffuse parenchymal lung disease (DPLD) is best achieved by a multidisciplinary team correlating clinical, radiologic, and pathologic features. Surgical lung biopsies remain the gold standard for histopathologic diagnosis of idiopathic interstitial pneumonias. Emerging data suggest an increasing role for transbronchial cryobiopsy (TBC) in DPLD evaluation. We describe our experience with TBC in patients with DPLD. METHODS: We retrospectively reviewed medical records of patients with radiographic features of DPLD who underwent TBC at Mayo Clinic, Rochester, Minnesota in June 2013-September 2015...
September 19, 2016: Chest
Yuko Toyoda, Masaki Hanibuchi, Jun Kishi, Hiroshi Kawano, Shun Morizumi, Seidai Sato, Mayo Kondo, Terumi Takikura, Toshifumi Tezuka, Hisatsugu Goto, Yasuhiko Nishioka
Acute exacerbation (AE) of interstitial lung disease is reported to be developed in not only idiopathic pulmonary fibrosis but also connective tissue disease-associated interstitial pneumonia (CTD-IP). As the significance of AE of CTD-IP has not been so widely recognized, its clinical feature is not fully elucidated. In the present study, we investigated the incidence, clinical features and outcome of AE of CTD-IP. We retrospectively reviewed admitted cases in our department with medical record from 2011 to 2015...
2016: Journal of Medical Investigation: JMI
Brandon T Larsen, Maxwell L Smith, Brett M Elicker, Jessica M Fernandez, Guillermo A Arbo-Oze de Morvil, Carlos A C Pereira, Kevin O Leslie
Context .- Idiopathic pulmonary fibrosis (IPF) is a distinctive clinicopathologic entity and the most common form of progressive diffuse lung scarring in older adults. Idiopathic pulmonary fibrosis manifests histopathologically as the usual interstitial pneumonia pattern. The usual interstitial pneumonia pattern is distinguished by geographically and temporally heterogeneous fibrosis that is peripherally accentuated, often with honeycombing and traction bronchiectasis. Idiopathic pulmonary fibrosis is not the only disease that leads to end-stage lung fibrosis, however, and several other entities may also cause advanced fibrosis...
September 15, 2016: Archives of Pathology & Laboratory Medicine
Ilia Atanelishvili, Yuichiro Shirai, Tanjina Akter, Atsushi Noguchi, Kurt T Ash, Suniti Misra, Sibnath Ghatak, Richard M Silver, Galina S Bogatkevich
Pulmonary fibrosis represents the terminal stage of a diverse group of lung diseases including scleroderma associated interstitial lung disease. The molecular mechanisms underlying the pathogenesis of lung fibrosis are not well understood and there is a great need for more effective treatment for this lethal disease. We recently discovered a small fragment of hepatocyte growth factor (HGF) receptor MET as a peptide designated "M10," with strong antifibrotic properties. Furthermore, we showed that aspartic acid at position 1398 of MET is essential for M10 generation...
2016: PloS One
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
Ayodeji Adegunsoye, Mary E Strek
Among the interstitial lung diseases (ILD), idiopathic pulmonary fibrosis (IPF), chronic hypersensitivity pneumonitis and fibrotic connective tissue disease related ILD are associated with a worse prognosis with death occurring both from respiratory failure and serious associated co-morbidities. 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...
August 10, 2016: Chest
Tanmay S Panchabhai, Carol Farver, Kristin B Highland
Lymphocytic interstitial pneumonia (LIP) is a rare lung disease on the spectrum of benign pulmonary lymphoproliferative disorders. LIP is frequently associated with connective tissue diseases or infections. Idiopathic LIP is rare; every attempt must be made to diagnose underlying conditions when LIP is diagnosed. Computed tomography of the chest in patients with LIP may reveal ground-glass opacities, centrilobular and subpleural nodules, and randomly distributed thin-walled cysts. Demonstrating polyclonality with immunohistochemistry is the key to differentiating LIP from lymphoma...
September 2016: Clinics in Chest Medicine
Tsutomu Hamada, Takuya Samukawa, Tomohiro Kumamoto, Kazuhito Hatanaka, Go Tsukuya, Masuki Yamamoto, Kentaro Machida, Masaki Watanabe, Keiko Mizuno, Ikkou Higashimoto, Yoshikazu Inoue, Hiromasa Inoue
No abstract text is available yet for this article.
2016: BMC Pulmonary Medicine
Ines Maria Grazia Piroddi, Gianluca Ferraioli, Cornelius Barlascini, Corrado Castagneto, Antonello Nicolini
Anti-synthetase syndrome (ASS) is defined as a heterogeneous connective tissue disorder characterized by the association of an interstitial lung disease (ILD) with or without inflammatory myositis with the presence of anti-aminoacyl-tRNA-synthetase antibodies. ILD is one of the major extra-muscular manifestations of polymyositis and dermatomyositis. We report a case of a patient with dyspnea, cough, and intermittent fever as well as ILD associated ASS in the absence of muscular involvement. This patient was admitted to the emergency department with severe respiratory failure requiring non-invasive ventilation...
July 2016: Respiratory Investigation
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
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
Zoe E Betteridge, Felix Woodhead, Hui Lu, Gavin Shaddick, Christopher C Bunn, Christopher P Denton, David J Abraham, Roland M du Bois, Mervyn Lewis, Athol U Wells, Neil J McHugh
Objective Anti-nuclear autoantibodies are known to occur in 85-99% of Systemic Sclerosis (SSc) patients, with each SSc autoantibody correlating with a distinct clinical subset of patients. The objective of this study was to investigate novel SSc autoantibodies in the remaining autoantibody negative patients and establish clinical associations. Methods Serum samples and clinical data were collected from 548 SSc patients. Sera were tested for known SSc autoantibodies by routine serological techniques, with negative samples being further investigated by radiolabelled protein immunoprecipitation (IPP)...
June 6, 2016: Arthritis & Rheumatology
Jeremy Udkoff, Philip R Cohen
Amyopathic dermatomyositis is a rare, idiopathic, connective tissue disease that presents with dermatologic lesions of classic dermatomyositis but lacks the myopathy of this disease. Cutaneous manifestations may include Gottron's sign, heliotrope rash, and characteristic patterns of poikiloderma. There is a substantial risk for developing interstitial lung disease or malignancy in patients with amyopathic dermatomyositis. A literature review of amyopathic dermatomyositis was performed using the PubMed medical database...
October 2016: American Journal of Clinical Dermatology
Shuzo Sato, Makiko Yashiro, Naoki Matsuoka, Manabu Uematsu, Tomoyuki Asano, Hiroko Kobayashi, Hiroshi Watanabe, Hiromasa Ohira
Mixed connective tissue disease (MCTD) is characterized by a combination of clinical features of systemic lupus erythematosus, systemic sclerosis, and polymyositis with elevated antibodies to U1 small nuclear ribonucleoprotein (U1-RNP). MCTD is often accompanied by interstitial lung disease as pulmonary involvement. On the other hand, microscopic polyangiitis (MPA) is a systemic autoimmune disease characterized by the inflammation of small vessels (arterioles, capillaries, and venules) mainly affecting the lung and kidney...
2016: Tohoku Journal of Experimental Medicine
Irene Jarana Aparicio, Joyce S Lee
Interstitial lung disease (ILD) complicating connective tissue disorders, such as scleroderma and rheumatoid arthritis, is associated with significant morbidity and mortality. Progress has been made in our understanding of these collective diseases; however, there are still many unanswered questions. In this review, we describe the current views on epidemiology, clinical presentation, treatment, and prognosis in patients with connective tissue disease (CTD)-associated ILD. We also highlight several areas that remain unresolved and in need of further investigation, including interstitial pneumonia with autoimmune features, histopathologic phenotype, and pharmacologic management...
June 2016: Seminars in Respiratory and Critical Care Medicine
Joseph P Lynch, Richard H Huynh, Michael C Fishbein, Rajan Saggar, John A Belperio, S Sam Weigt
Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic interstitial lung pneumonia associated with the histologic pattern of usual interstitial pneumonia (UIP). Although UIP is a distinct histologic lesion, this histologic pattern is not specific for IPF and can also be found in other diseases (e.g., connective tissue disease and asbestosis). Clinical features of IPF include progressive cough, dyspnea, restrictive ventilatory defect, and progressive fibrosis and destruction of the lung parenchyma...
June 2016: Seminars in Respiratory and Critical Care Medicine
Christine Cramer, Vivi Schlünssen, Elisabeth Bendstrup, Zara Ann Stokholm, Jesper Medom Vestergaard, Morten Frydenberg, Henrik Albert Kolstad
We studied the risk of hypersensitivity pneumonitis and other interstitial lung diseases (ILDs) among pigeon breeders.This is a retrospective follow-up study from 1980 to 2013 of 6920 pigeon breeders identified in the records of the Danish Racing Pigeon Association. They were compared with 276 800 individually matched referents randomly drawn from the Danish population. Hospital based diagnoses of hypersensitivity pneumonitis and other ILDs were identified in the National Patient Registry 1977-2013. Stratified Cox regression analyses estimated the hazard ratios (HR) of hypersensitivity pneumonitis and other ILDs adjusted for occupation, residence and redeemed prescription of medication with ILDs as a possible side-effect...
September 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
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