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IgG4-related pulmonary disease

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https://www.readbyqxmd.com/read/29768335/igg4-related-disease-of-pulmonary-artery-causing-pulmonary-hypertension
#1
Hui Deng, Sheng Zhao, Yunlong Yue, Yong Liu, Yali Xu, Jin Qian, Xiaorong Ma, Peiliang Gao, Xiaoyan Yao, Xin Jiang, Xiqi Xu, Zhicheng Jing, Yong Wang, Lei Pan, Xinying Xue
IgG4-related disease (IgG4-RD) is recognized as an immune-mediated condition with pathology features of lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis, accompanied with or without elevated serum IgG4 concentrations. However, few of pulmonary artery IgG4-RD causing pulmonary hypertension (PH) was reported.The medical records of 3 patients with pulmonary artery IgG4-RD inducing PH were analyzed retrospectively.Imaging findings demonstrated that the lesions of 3 patients located in pulmonary artery, which were initially diagnosed as pulmonary thrombus or malignant tumor...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29526046/transbronchial-lung-biopsy-for-the-diagnosis-of-igg4-related-lung-disease
#2
Kyoko Otani, Dai Inoue, Tomoo Itoh, Yoh Zen
AIMS: The diagnosis of IgG4-related disease (IgG4-RD) requires a multidisciplinary approach, in which histology plays an important role. Although a diagnosis was previously established by the use of surgically resected specimens, there is increasing clinical demand to diagnose this systemic condition by the use of biopsies. The aim of the present study was to elucidate how useful transbronchial lung biopsies (TBLBs) are for this diagnostic purpose. METHODS AND RESULTS: The study cohort consisted of 20 consecutive patients diagnosed with IgG4-RD in other organs who underwent TBLB for potential pulmonary involvement...
March 11, 2018: Histopathology
https://www.readbyqxmd.com/read/29434730/clinical-and-pathological-characteristics-of-igg4-related-interstitial-lung-disease
#3
Xiaoting Lv, Feng Gao, Qicai Liu, Sheng Zhang, Zhihua Huang, Yongping Zhu, Haiyang Zong, Quwen Li, Sanyan Li
IgG4-related interstitial lung disease (IgG4-RILD), which is characterized by increased IgG4 levels, IgG4+ plasma cell infiltration and irregular whorled fibrosis, is a recently described lung disorder that belongs to the group of systemic fibroinflammatory IgG4-related diseases (IgG4-RD). The aim of the present study was to improve the current knowledge regarding the specific clinical and histopathological characteristics of IgG4-RILD and to investigate its underlying immune mechanism in vivo . A total of 7 patients newly diagnosed with IgG4-RILD were enrolled in the present study (4 men and 3 women; mean age, 57 years; range, 29-71 years)...
February 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29362197/immunoglobulin-g4-related-disease-mimicking-an-anterior-mediastinal-tumor
#4
Seung Ri Kang, Hyeong Ryul Kim, Soo Jeong Nam, Seokchan Hong
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated collection of disorders that were once thought to be unrelated but are increasingly being recognized as a single entity. The tumor-like swelling of the involved organ, a storiform pattern of fibrosis, and a serologic elevation of IgG4 can lead to the diagnosis of IgG4-RD. The usual organs affected are the pancreas, biliary tract, and salivary and lacrimal glands. There have been few reports of intrathoracic cases, which usually have pulmonary lesions...
February 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29245357/igg4-related-lung-disease-presenting-as-interstitial-lung-disease-with-bronchiolitis-a-case-report
#5
Chiu-Fan Chen, Kuo-An Chu, Yen-Chiang Tseng, Chang-Che Wu, Ruay-Sheng Lai
RATIONALE: IgG4-related disease is a rare and novel disease entity that tends to involve multiple organs. The pulmonary manifestation of this disease is highly variable and may mimic lung cancer, pneumonia, interstitial lung disease (ILD), sarcoidosis, and so forth. Small airway disease is rarely reported in IgG4-related lung disease (IgG4-RLD). In the current study, we describe a rare case of IgG4-RLD with patterns of ILD and bronchiolitis. PATIENT CONCERN: A 43-year-old man had chronic cough and dyspnea on exertion for 4 years...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29229107/clinical-characterization-of-52-patients-with-immunoglobulin-g4-related-disease-in-a-single-tertiary-center-in-japan-special-reference-to-lung-disease-in-thoracic-high-resolution-computed-tomography
#6
Takeshi Saraya, Kosuke Ohkuma, Masachika Fujiwara, Chika Miyaoka, Shoko Wada, Takayasu Watanabe, Sunao Mikura, Manami Inoue, Miku Oda, Mitsuru Sada, Yukari Ogawa, Kojiro Honda, Masaki Tamura, Takuma Yokoyama, Daisuke Kurai, Haruyuki Ishii, Hajime Takizawa
BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a rare multi-organ disorder. Physicians rarely encounter patients with IgG4-RD and its range of symptoms. METHODS: To elucidate the clinical characterization of IgG4-RD, along with the clinical significance of lung involvement, we retrospectively reviewed the medical records of patients who satisfied the comprehensive diagnostic criteria for IgG4-RD. RESULTS: We identified 52 patients with IgG4-RD...
November 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/29139157/seronegative-granulomatosis-with-polyangiitis-presenting-with-multiple-cranial-nerve-palsies
#7
Eunhye Lee, Jynkyun Park, Seung Hong Choi, Sung-Hye Park
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti-neutrophil cytoplasmic antibody (ANCA)-negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50-year-old man whose first manifestation was multiple neurologic defects without respiratory or renal symptoms...
November 14, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29046807/pulmonary-schistosomiasis-mimicking-igg4-related-lung-disease
#8
Timothy Baird, Caroline L Cooper, Richard Wong, Naomi Runnegar, Gregory Keir
IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterized by tumefactive lesions in various organ systems, including the lungs. Clinical and radiological manifestations of IgG4-RD are relatively non-specific, and we report a case highlighting the importance of histopathological confirmation in cases of suspected IgG4-related lung disease. A 44-year-old male with significantly elevated serum IgG4 levels, patchy consolidation on thoracic CT imaging, and cough was referred with suspected IgG4-related lung disease...
January 2018: Respirology Case Reports
https://www.readbyqxmd.com/read/28950319/igg4-related-disease-of-the-lung-a-rare-differential-diagnosis-to-lung-cancer-after-positive-positron-emission-tomography-and-biopsy
#9
Adrian Zehnder, Gregor J Kocher, Michael Seitz, Ralph A Schmid
Immunoglobulin G4-related disease is a rare immune-mediated condition that often causes serious diagnostic problems. Symptoms are unspecific, and several organs can be involved. To date, IgG4-related lung disease has seldom been reported in literature. Nevertheless, a variety of pulmonary involvement has been described, which can mimic malignancy. The gold standard for the diagnosis is the identification of typical histopathological features, even if diagnostic biomarker such as serum IgG4 concentration can be an indicator for a more aggressive course of the disease...
November 1, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28922375/elevated-igg4-serum-levels-in-patients-with-cystic-fibrosis
#10
Axelle Clerc, Quitterie Reynaud, Stéphane Durupt, Colette Chapuis-Cellier, Raphaële Nové-Josserand, Isabelle Durieu, Jean Christophe Lega
OBJECTIVE: Serum immunoglobulin (Ig) G4 elevation has been associated with several pathological conditions other than IgG4-related disease (IgG4-RD). In cystic fibrosis (CF), an elevation of specific IgG4 has been associated with colonization and infection by Pseudomonas aeruginosa. IgG4 elevation may be a marker of chronic infection or inflammatory stimulation. The aim of this study was to explore the prevalence of elevated IgG4 levels in CF and its correlation with the major clinical and microbiological features found in CF patients...
2017: PloS One
https://www.readbyqxmd.com/read/28910817/the-lung-in-dysregulated-states-of-humoral-immunity
#11
Yurdagül Uzunhan, Florence Jeny, Marianne Kambouchner, Morgane Didier, Diane Bouvry, Hilario Nunes, Jean-François Bernaudin, Dominique Valeyre
In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis...
2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28870031/-the-clinical-characteristics-of-346-patients-with-igg4-related-disease
#12
P P Zhang, J Z Zhao, M Wang, R E Feng, X W Liu, X M Lai, X J Li, J G Zeng, H J Shi, H D Zhu, W Xue, H Zhang, Y Y Chen, L Y Fei, X F Peng, F C Zeng, Y M Zhang, W Zhang
Objective: To analyze the clinical characteristics of IgG4-related disease (IgG4-RD)so as to improve the understanding of IgG4-RD in China. Methods: IgG4-RD patients were recruited from Peking Union Medical College Hospital between January 2011 and January 2016. All patients were followed-up for more than 6 months. The demographic characteristics, symptoms, organ involvements, laboratory examinations and treatment efficacy were evaluated and analyzed. Results: A total of 346 patients were finally enrolled, including 230 males (66...
September 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28744088/igg4-related-disease-simulating-paraneoplastic-syndrome-role-of-18-fdg-pet-ct-imaging
#13
Madhuri S Mahajan, Sudeshna Maitra, Natasha Singh, Melvika Pereira
Immunoglobulin G4 (IgG4)-related systemic disease (IgG4-RSD) is a new systemic entity associated with autoimmune pancreatitis (AIP). Other organ involvements take the form of sclerosing cholangitis, sclerosing cholecystitis, sclerosing sialadenitis, retroperitoneal fibrosis, and interstitial nephritis. Recently, lung diseases related to IgG4 have been described to occur with or without other organ involvement. These diseases include interstitial lung disease (ILD), pulmonary inflammatory pseudotumor, and lymphomatoid granulomatosis...
April 2017: Indian Journal of Radiology & Imaging
https://www.readbyqxmd.com/read/28725444/post-tuberculous-fibrosing-mediastinitis-a-review-of-the-literature
#14
REVIEW
Zhe Wu, Hannah Jarvis, Luke S Howard, Corrina Wright, Onn Min Kon
Fibrosing mediastinitis (FM) is a rare disease where there is thickening of the fibrous tissue in the mediastinum. While histoplasmosis is the the most common recognised cause, the link with tuberculosis (TB) has been rarely documented. We review the link between TB and FM, and describe a case of probable TB-related FM.Our case is of a 74-year-old man who presented with breathlessness 3 years after fully treated TB. Scans revealed a calcified soft tissue mass within the mediastinum, and a diagnosis of fibrosing mediastinitis resulting in pulmonary hypertension was made...
2017: BMJ Open Respiratory Research
https://www.readbyqxmd.com/read/28614225/immunoglobulin-g4-related-disease-preceded-by-lung-involvement-a-case-report
#15
Miki Abo, Hazuki Takato, Satoshi Watanabe, Kazumasa Kase, Tamami Sakai, Hayato Koba, Johsuke Hara, Takashi Sone, Hideharu Kimura, Kazuo Kasahara
RATIONALE: Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition involving various organs and vessels including the pancreas, bile duct, salivary glands, periorbital tissues, kidneys, lungs, lymph nodes, meninges, and aorta. Recently, some cases of IgG4-RD have been reported, in which only pulmonary lesions were present. It is not known whether IgG4-RD can be diagnosed on the basis of pulmonary lesions only, because increases in serum IgG4 levels and infiltration of IgG4-positive plasma cells into the lung tissue also occur in other inflammatory conditions...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28449468/pulmonary-inflammatory-myofibroblastic-tumor-versus-igg4-related-inflammatory-pseudotumor-differential-diagnosis-based-on-a-case-series
#16
Longfei Zhu, Jian Li, Chengwu Liu, Wenshuang Ding, Feng Lin, Chenglin Guo, Lunxu Liu
BACKGROUND: Pulmonary inflammatory myofibroblastic tumor (IMT) has been considered as a synonym for inflammatory pseudotumor (IPT) for a long time. Recent studies have indicated that IMT and IgG4-related IPT are distinct diseases. However, no consensus criteria have been recommended. Here we propose a set of criteria for the differential diagnosis. METHODS: Twenty-six archived IMT and IgG4-related IPT samples were examined for histological characteristics and the expression of IgG, IgG4, SMA and ALK-1...
March 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28449462/pulmonary-function-tests-findings-and-their-diagnostic-value-in-patients-with-igg4-related-disease
#17
Lu Cao, Yi-Bing Chen, Da-Hui Zhao, Wen-Fang Shi, Song Meng, Li-Xin Xie
BACKGROUND: IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder that can affect most organs. To date, there have been no detailed assessments of pulmonary function in patients with IgG4-RD. In this study, we investigated pulmonary function in IgG4-RD patients and evaluated the value of pulmonary function tests (PFTs) in diagnosing IgG4-related respiratory disease (IgG4-RRD). METHODS: This was a retrospective study of 17 patients with IgG4-RD...
March 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28357112/immunoglobulin-g4-related-disease-a-rare-steroid-responsive-disease
#18
Vorawut Thanthitaweewat, Poonchavist Chantranuwatana, Naricha Chirakalwasan
A 70-year-old man presented with progressive dyspnoea and weight loss. Physical examination revealed only mild pale conjunctiva. The workup showed mild anaemia, mild impaired renal function, and high globulin level. Multiple myeloma was excluded by normal serum protein electrophoresis. The chest radiography and computed tomography (CT) revealed bilateral multifocal patchy infiltration with mediastinal adenopathy. Bronchoscopy was performed. Bronchoalveolar lavage (BAL) fluid examination was negative for infection and malignancy...
May 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28304239/immunoglobulin-g4-related-epidural-inflammatory-pseudotumor-presenting-with-pulmonary-complications-and-spinal-cord-compression-case-report
#19
Kavelin Rumalla, Kyle A Smith, Paul M Arnold
Immunoglobulin G4-related disease (IgG4-RD) is a recently defined condition characterized by inflammatory tumefactive lesions in various organ systems. IgG4-RD is a clinical and radiological diagnosis of exclusion and requires the presence of specific histopathological criteria for diagnosis. A 50-year-old man presented to an outside hospital with a 3-month history of progressively worsening back pain and symptoms of pleurisy, nasal crusting, and hematochezia. Radiological workup revealed an epidural-paraspinal mass with displacement of the spinal cord, destruction of the T5-6 vertebrae, and extension into the right lung...
June 2017: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/28297814/-igg4-related-lung-disease-analysis-of-8-cases-and-literature-review
#20
REVIEW
G J Han, H Hu, D Mao, X Bai, D Y She, S F Zhao, Z L Wen, J Gao
Objective: To improve the understanding and treatment of IgG4-related lung disease (IgG4-RLD). Methods: The clinical characteristics, serum IgG4 levels, pathological features, chest CT, therapy and prognosis of 8 patients with IgG4-RLD were retrospectively analyzed. These patients were admitted to the People's Liberation Army General Hospital and the pathological diagnosis was made between December 2005 and March 2016. Relevant literatures were reviewed. Results: The 8 patients with IgG4-RLD included 4 men and 4 women, with an average age of (59±4) years (range, 37-74)...
March 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
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