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Infectious, Rare and Idiopathic Pulmonary Diseases

Section edited by Sanjay Chotirmall and Giovanni Sotgiu

This section concerns all aspects of pulmonary infection, rare and idiopathic lung diseases including, but not limited to: prevention, diagnosis and management, as well as related molecular genetics, and pathophysiology.

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  1. Acute exacerbation (AE) is the major cause of morbidity and mortality in patients with idiopathic pulmonary fibrosis (IPF). AEs also occur in other forms of fibrosing interstitial lung disease (fILD). The clin...

    Authors: Mengshu Cao, Jian Sheng, Xiaohua Qiu, Dandan Wang, Dongmei Wang, Yang Wang, Yonglong Xiao and Hourong Cai

    Citation: BMC Pulmonary Medicine 2019 19:215

    Content type: Research article

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  2. Fibrosing, non-idiopathic pulmonary fibrosis (non-IPF) interstitial lung diseases (fILDs) are a heterogeneous group of diseases characterized by a different amount of inflammation and fibrosis. Therapy is curr...

    Authors: Sebastiano Emanuele Torrisi, Nicolas Kahn, Julia Wälscher, Nilab Sarmand, Markus Polke, Kehler Lars, Monika Eichinger, Claus Peter Heussel, Stefano Palmucci, Francesca Maria Sambataro, Gianluca Sambataro, Domenico Sambataro, Carlo Vancheri and Michael Kreuter

    Citation: BMC Pulmonary Medicine 2019 19:213

    Content type: Research article

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  3. A three-drug regimen (macrolide, ethambutol, and rifampicin) is recommended for the treatment of Mycobacterium avium complex pulmonary disease (MAC-PD). Although macrolide has proven efficacy, the role of ethambu...

    Authors: Hyung-Jun Kim, Jong Sik Lee, Nakwon Kwak, Jaeyoung Cho, Chang-Hoon Lee, Sung Koo Han and Jae-Joon Yim

    Citation: BMC Pulmonary Medicine 2019 19:212

    Content type: Research article

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  4. Delay in diagnosis and treatment of pulmonary tuberculosis (PTB) leads to severe disease, adverse outcomes and increased transmission. Assessing the extent of delay and its effect on disease progression in TB ...

    Authors: Fentabil Getnet, Meaza Demissie, Alemayehu Worku, Tesfaye Gobena, Rea Tschopp, Michael Girmachew, Gebeyehu Assefa and Berhanu Seyoum

    Citation: BMC Pulmonary Medicine 2019 19:201

    Content type: Research article

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  5. Myeloperoxidase anti-neutrophil cytoplasmic antibody-related nephritis (MPO-ANCA nephritis) is occasionally accompanied by lung abnormalities such as pulmonary fibrosis. However, the clinical features of pulmo...

    Authors: Toshikazu Watanabe, Tomoyuki Minezawa, Midori Hasegawa, Yasuhiro Goto, Takuya Okamura, Yosuke Sakakibara, Yoshikazu Niwa, Atsushi Kato, Masamichi Hayashi, Sumito Isogai, Masashi Kondo, Naoki Yamamoto, Naozumi Hashimoto and Kazuyoshi Imaizumi

    Citation: BMC Pulmonary Medicine 2019 19:194

    Content type: Research article

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  6. Connective tissue disease-associated interstitial lung disease (CTD-ILD) is associated with reduced quality of life and poor prognosis. Prior studies have not identified a consistent combination of variables t...

    Authors: Chrystal Chan, Christopher J. Ryerson, James V. Dunne and Pearce G. Wilcox

    Citation: BMC Pulmonary Medicine 2019 19:192

    Content type: Research article

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  7. In 2015, South Korea experienced an outbreak of Middle East respiratory syndrome (MERS), and our hospital experienced a nosocomial MERS infection. We performed a comprehensive analysis to identify the MERS tra...

    Authors: Hyun Kyun Ki, Sang Kuk Han, Jun Seong Son and Sang O Park

    Citation: BMC Pulmonary Medicine 2019 19:190

    Content type: Research article

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  8. Diffuse alveolar haemorrhage (DAH) is characterized by the diffuse accumulation of red blood cells within the alveoli, presence of ground glass opacities and/or consolidation on computed tomography (CT). Aside...

    Authors: Anna Stainer, Alex Rice, Anand Devaraj, Joseph Luke Barnett, Jacqueline Donovan, Maria Kokosi, Andrew Gordon Nicholson, Tom Cairns, Athol Umfrey Wells and Elisabetta Augusta Renzoni

    Citation: BMC Pulmonary Medicine 2019 19:185

    Content type: Case report

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  9. Chronic obstructive pulmonary disease (COPD) and pulmonary tuberculosis (PTB) share a number of common risk factors, including innate immunity-related genetic factors. In the present study, we compared the rol...

    Authors: Zhongqi Li, Xuhua Mao, Qiao Liu, Huan Song, Biyu He, Peiyi Shi, Qun Zhang, Xiaona Li and Jianming Wang

    Citation: BMC Pulmonary Medicine 2019 19:184

    Content type: Research article

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  10. Fibrotic interstitial lung disease (FILD) patients are typically dyspneic and exercise-intolerant with consequent impairment of health-related quality of life (HRQoL). Respiratory muscle dysfunction is among t...

    Authors: Pauliane Vieira Santana, Leticia Zumpano Cardenas, André Luis Pereira de Albuquerque, Carlos Roberto Ribeiro de Carvalho and Pedro Caruso

    Citation: BMC Pulmonary Medicine 2019 19:183

    Content type: Research article

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  11. Most international guidelines recommend empirical therapy for community-acquired pneumonia (CAP) to be based on site of care. Some patients with severe CAP are managed in general wards because of limited inten...

    Authors: Phunsup Wongsurakiat and Napat Chitwarakorn

    Citation: BMC Pulmonary Medicine 2019 19:179

    Content type: Research article

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  12. Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder that is associated with oculocutaneous albinism, bleeding diathesis, granulomatous colitis, and highly penetrant pulmonary fibrosis in some su...

    Authors: Martina Doubková, Jakub Trizuljak, Zuzana Vrzalová, Anna Hrazdirová, Ivona Blaháková, Lenka Radová, Šárka Pospíšilová and Michael Doubek

    Citation: BMC Pulmonary Medicine 2019 19:178

    Content type: Case report

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  13. Idiopathic Pulmonary Fibrosis (IPF) is an aggressive interstitial lung disease with an unpredictable course. Occupational dust exposure may contribute to IPF onset, but its impact on antifibrotic treatment and...

    Authors: Valeria Casillo, Stefania Cerri, Andrea Ciervo, Mariarita Stendardo, Lamberto Manzoli, Maria Elena Flacco, Maurizio Manno, Marialuisa Bocchino, Fabrizio Luppi and Piera Boschetto

    Citation: BMC Pulmonary Medicine 2019 19:170

    Content type: Research article

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  14. Pulmonary hypertension (PH) is traditionally defined as a resting mean pulmonary artery pressure (mPAP) of ≥25 mmHg, while mPAP in the range of 21 to 24 mmHg is recognized as “borderline PH.” Interstitial lung...

    Authors: Kenji Nemoto, Shuji Oh-ishi, Tatsuya Akiyama, Yuki Yabuuchi, Hitomi Goto, Mizu Nonaka, Yuika Sasatani, Hiroaki Tachi, Naoki Arai, Hiroaki Ishikawa, Kentaro Hyodo, Isano Hase, Yukiko Miura, Takio Takaku, Kenji Hayashihara and Takefumi Saito

    Citation: BMC Pulmonary Medicine 2019 19:167

    Content type: Research article

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  15. Myelolipoma is a rare benign tumor composed of mature adipose and hematopoietic tissues. Most myelolipomas are found in the adrenal glands, whereas intrathoracic myelolipoma is extremely rare. In particular, b...

    Authors: Hyun Sung Chung, Kyu Min Lee, Jung Seop Eom, Insu Kim, Seyeon Park, Jihyun Ahn, Ahrong Kim, Chang Hun Lee, Geewon Lee and Min Ki Lee

    Citation: BMC Pulmonary Medicine 2019 19:151

    Content type: Case report

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  16. Recent studies have highlighted the contribution of senescent mesenchymal and epithelial cells in Idiopathic Pulmonary Fibrosis (IPF), but little is known regarding the molecular mechanisms that regulate the a...

    Authors: David M. Habiel, Miriam S. Hohmann, Milena S. Espindola, Ana Lucia Coelho, Isabelle Jones, Heather Jones, Richard Carnibella, Isaac Pinar, Freda Werdiger and Cory M. Hogaboam

    Citation: BMC Pulmonary Medicine 2019 19:165

    Content type: Research article

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  17. Burkholderia contaminans is one of the 20 closely related bacterial of the Burkholderia cepacia complex, a group of bacteria that are ubiquitous in the environment and capable of infecting people with cystic fibr...

    Authors: Daniela Savi, Serena Quattrucci, Maria Trancassini, Claudia Dalmastri, Riccardo V. De Biase, Marta Maggisano, Paolo Palange and Annamaria Bevivino

    Citation: BMC Pulmonary Medicine 2019 19:164

    Content type: Case report

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  19. CTD-related pleural effusions are rare and challenging to diagnose. Our lung inflammation service (with expertise in rheumatology, interstitial lung disease and respiratory failure) works closely with the pleu...

    Authors: Hugh Ip, Parthipan Sivakumar, Eugene Ace McDermott, Sangita Agarwal, Boris Lams, Alex West and Liju Ahmed

    Citation: BMC Pulmonary Medicine 2019 19:161

    Content type: Research article

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  20. Lung ultrasound (LUS) is nowadays a fast-growing field of study since the technique has been widely acknowledged as a cost-effective, radiation free, and ready available alternative to standard X-ray imaging. ...

    Authors: Danilo Buonsenso, Anna Maria Musolino, Antonio Gatto, Ilaria Lazzareschi, Antonietta Curatola and Piero Valentini

    Citation: BMC Pulmonary Medicine 2019 19:159

    Content type: Correspondence

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  21. Nintedanib is an inhibitor of receptor tyrosine kinases, including vascular endothelial growth factor receptor, but its effects on pulmonary hypertension (PH) in idiopathic pulmonary fibrosis (IPF) patients wi...

    Authors: Masahiro Tahara, Keishi Oda, Kei Yamasaki, Takako Kawaguchi, Konomi Sennari, Shingo Noguchi, Noriho Sakamoto, Toshinori Kawanami, Hiroshi Mukae and Kazuhiro Yatera

    Citation: BMC Pulmonary Medicine 2019 19:157

    Content type: Research article

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  22. Nintedanib is a tyrosine kinase inhibitor that efficiently slows the progression of idiopathic pulmonary fibrosis (IPF) and has an acceptable tolerability profile. In contrast, immune checkpoint inhibitors (IC...

    Authors: Hideaki Yamakawa, Tomohiro Oba, Hiroki Ohta, Yuta Tsukahara, Gen Kida, Emiri Tsumiyama, Tomotaka Nishizawa, Rie Kawabe, Shintaro Sato, Keiichi Akasaka, Masako Amano, Kazuyoshi Kuwano and Hidekazu Matsushima

    Citation: BMC Pulmonary Medicine 2019 19:156

    Content type: Case report

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  23. Atypical manifestations, such as elevated serum immunoglobulin-G4 (IgG4) and extra-pulmonary IgG4 positive plasmacyte infiltration, have been described in patients with eosinophilic granulomatosis with polyang...

    Authors: Li Zhou, Fen Cao, Songqing Fan, Ping Chen, Shuizi Ding, Guiqian Liu and Ruoyun Ouyang

    Citation: BMC Pulmonary Medicine 2019 19:154

    Content type: Case report

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  24. Honeycombing on high-resolution computed tomography (HRCT) is a distinguishing feature of usual interstitial pneumonia and predictive of poor outcome in interstitial lung diseases (ILDs). Although fine crackle...

    Authors: Toshikazu Fukumitsu, Yasushi Obase, Yuji Ishimatsu, Shota Nakashima, Hiroshi Ishimoto, Noriho Sakamoto, Kosei Nishitsuji, Shunpei Shiwa, Tomoya Sakai, Sueharu Miyahara, Kazuto Ashizawa, Hiroshi Mukae and Ryo Kozu

    Citation: BMC Pulmonary Medicine 2019 19:153

    Content type: Research article

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  25. The phase III REMoxTB study prospectively enrolled HIV-positive (with CD4+ count > 250 cells, not on anti-retroviral therapy) and HIV-negative patients. We investigated the incidence of adverse events and cure...

    Authors: Conor D. Tweed, Angela M. Crook, Rodney Dawson, Andreas H. Diacon, Timothy D. McHugh, Carl M. Mendel, Sarah K. Meredith, Lerato Mohapi, Michael E. Murphy, Andrew J. Nunn, Patrick P. J. Phillips, Kasha P. Singh, Melvin Spigelman and Stephen H. Gillespie

    Citation: BMC Pulmonary Medicine 2019 19:152

    Content type: Research article

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  26. Clinical studies demonstrate that ivacaftor (IVA) improves health-related quality of life (HRQoL) in patients aged ≥6 years with cystic fibrosis (CF). The real-world impact of IVA and standard of care (SOC) in...

    Authors: Scott C. Bell, Jochen G. Mainz, Gordon MacGregor, Susan Madge, Julie Macey, Moshe Fridman, Ellison D. Suthoff, Siva Narayanan and Nils Kinnman

    Citation: BMC Pulmonary Medicine 2019 19:146

    Content type: Research article

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  27. A minority of patients presenting with lower respiratory tract infection (LRTI) to their general practitioner (GP) have community-acquired pneumonia (CAP) and require antibiotic therapy. Identifying them is ch...

    Authors: Loïc Lhopitallier, Andreas Kronenberg, Jean-Yves Meuwly, Isabella Locatelli, Julie Dubois, Joachim Marti, Yolanda Mueller, Nicolas Senn, Valérie D’Acremont and Noémie Boillat-Blanco

    Citation: BMC Pulmonary Medicine 2019 19:143

    Content type: Study protocol

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  29. Primary ciliary dyskinesia (PCD) is a rare genetic disorder. Although the genetic tests and new diagnostic algorithms have recently been recommended, clinical signs and electron microscope (EM) findings have h...

    Authors: Atsushi Inaba, Masanori Furuhata, Kozo Morimoto, Mahbubur Rahman, Osamu Takahashi, Minako Hijikata, Michael R. Knowles and Naoto Keicho

    Citation: BMC Pulmonary Medicine 2019 19:135

    Content type: Research article

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  30. Pulmonary capillary hemangiomatosis (PCH) is a progressive and refractory vascular disease in the lung. Pulmonary hypertension is frequently combined with PCH when capillary proliferation invades to nearby pul...

    Authors: Chang Dong Yeo, Deokjae Han, Jongmin Lee, Woo-Baek Chung, Jung Im Jung, Kyo-Young Lee, Tae-Jung Kim, Woori Jang, Myungshin Kim and Ji Young Kang

    Citation: BMC Pulmonary Medicine 2019 19:133

    Content type: Case report

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  31. Culturing of bronchoalveolar lavage (BAL) fluid is a commonly used method for pathogen detection in pneumonia. However, the sensitivity is low, especially in patients pre-treated with anti-infective agents. Th...

    Authors: Eva Tschiedel, Arkadius Goralski, Jörg Steinmann, Peter-Michael Rath, Margarete Olivier, Uwe Mellies, Tanja Kottmann and Florian Stehling

    Citation: BMC Pulmonary Medicine 2019 19:132

    Content type: Research article

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  32. The aim of this study was to analyze the relative frequency, clinical characteristics, disease onset and progression in f-IPF vs. sporadic IPF (s-IPF).

    Authors: Ekaterina Krauss, Godja Gehrken, Fotios Drakopanagiotakis, Silke Tello, Ruth C. Dartsch, Olga Maurer, Anita Windhorst, Daniel von der Beck, Matthias Griese, Werner Seeger and Andreas Guenther

    Citation: BMC Pulmonary Medicine 2019 19:130

    Content type: Research article

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  33. Granulomatosis with polyangiitis (GPA) is characterised by the main violation of the upper and lower respiratory tract and kidney. GPA is considered a systemic vasculitis of medium-sized and small blood vessel...

    Authors: Lei Pan, Jun-Hong Yan, Fu-Quan Gao, Hong Li, Sha-Sha Han, Guo-Hong Cao, Chang-Jun Lv and Xiao-Zhi Wang

    Citation: BMC Pulmonary Medicine 2019 19:122

    Content type: Case report

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  34. Patients with interstitial lung disease (ILD) who show features related to autoimmunity without meeting criteria for a defined connective tissue disease are categorized as interstitial pneumonia with autoimmun...

    Authors: Jeong Uk Lim, Bo Mi Gil, Hye Seon Kang, Jongyeol Oh, Yong Hyun Kim and Soon Seog Kwon

    Citation: BMC Pulmonary Medicine 2019 19:120

    Content type: Research article

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  35. There are few cases of multiple bronchial stenoses reported in the literature and none of the severity described here. The case is relevant due to its rareness, the pathophysiological insights derived, the suc...

    Authors: Thomas Crowhurst, Joshua Lightfoot, Aeneas Yeo, Benjamin Reddi, Phan Nguyen, Helen Whitford and Chien-Li Holmes-Liew

    Citation: BMC Pulmonary Medicine 2019 19:119

    Content type: Case report

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  36. Although infections caused by nontuberculous mycobacteria (NTM) are increasing in prevalence, there are few data about hemoptysis in patients with NTM lung disease. This study investigated the characteristics ...

    Authors: Su Hwan Lee, Jin Hwa Lee, Jung Hyun Chang, Soo Jung Kim, Hee-Young Yoon, Sung Shine Shim, Min Uk Kim, Sun Young Choi and Yon Ju Ryu

    Citation: BMC Pulmonary Medicine 2019 19:117

    Content type: Research article

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  37. Here, we present real-world data on the incidence and risk factors of acute exacerbation (AE) in patients with chronic fibrotic interstitial pneumonia (CFIP) treated with antifibrotic agents, which has been pr...

    Authors: Kodai Kawamura, Kazuya Ichikado, Hidenori Ichiyasu, Keisuke Anan, Yuko Yasuda, Moritaka Suga and Takuro Sakagami

    Citation: BMC Pulmonary Medicine 2019 19:113

    Content type: Research article

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  38. Interstitial lung disease (ILD) is a severe systemic manifestation of rheumatoid arthritis (RA). High-resolution computed tomography (HRCT) represents the gold standard for the diagnosis of ILD, but its routin...

    Authors: A. Manfredi, G. Cassone, S. Cerri, V. Venerito, A. L. Fedele, M. Trevisani, F. Furini, O. Addimanda, F. Pancaldi, G. Della Casa, R. D’Amico, R. Vicini, G. Sandri, P. Torricelli, I. Celentano, A. Bortoluzzi…

    Citation: BMC Pulmonary Medicine 2019 19:111

    Content type: Research article

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  39. Treatment of patients with Cystic Fibrosis homozygous for the Phe508del gene, with Lumacaftor /Ivacaftor (LUM/IVA) improves outcomes in patients with FEV1 > 40% predicted. We set out to observe the most sensitive...

    Authors: Peter A. B. Wark, Kim Cookson, Theeba Thiruchelvam, John Brannan and Douglas J. Dorahy

    Citation: BMC Pulmonary Medicine 2019 19:106

    Content type: Research article

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  40. Bronchial Dieulafoy’s disease (BDD) is a rare disease that is known to be a cause of hemorrhage. The characteristics of this disease are still unknown. The present study describes the disorder based on a revie...

    Authors: Xin Qian, Qiong Du, Na Wei, Meifang Wang, Hansheng Wang and Yijun Tang

    Citation: BMC Pulmonary Medicine 2019 19:104

    Content type: Research article

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  41. Pleural involvement by non-tuberculous mycobacteria (NTM) in patients without distinct pulmonary disease is extremely rare. Vertebral osteomyelitis (VO) with or without pulmonary disease is also a rare clinica...

    Authors: Naoki Kadota, Tsutomu Shinohara, Hiroyuki Hino, Yuichiro Goda, Yoshiro Murase, Satoshi Mitarai and Fumitaka Ogushi

    Citation: BMC Pulmonary Medicine 2019 19:100

    Content type: Case report

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  42. Pulmonary rehabilitation improves exercise capacity and reduces risk of future exacerbation in COPD when performed after an exacerbation. There have been no previous studies of post-exacerbation rehabilitation...

    Authors: James D. Chalmers, Megan L. Crichton, Gill Brady, Simon Finch, Mike Lonergan and Thomas C. Fardon

    Citation: BMC Pulmonary Medicine 2019 19:85

    Content type: Research article

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  43. Gastroesophageal reflux disease (GORD) is highly prevalent in idiopathic pulmonary fibrosis (IPF) and may play a role in its pathogenesis. Recent IPF treatment guidelines suggest that all patients with IPF be ...

    Authors: Helen E. Jo, Tamera J. Corte, Ian Glaspole, Christopher Grainge, Peter M. A. Hopkins, Yuben Moodley, Paul N. Reynolds, Sally Chapman, E. Haydn Walters, Christopher Zappala, Heather Allan, Gregory J. Keir, Wendy A. Cooper, Annabelle M. Mahar, Samantha Ellis, Sacha Macansh…

    Citation: BMC Pulmonary Medicine 2019 19:84

    Content type: Research article

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  44. Rosai-Dorfman disease (RDD) is a rare histiocytosis which involves principally lymph nodes. Thyroid involvement in RDD is a very rare situation, and lung involvement is even rarer.

    Authors: Pietro Gianella, Nicolas Dulguerov, Grégoire Arnoux, Marc Pusztaszeri and Jörg D. Seebach

    Citation: BMC Pulmonary Medicine 2019 19:83

    Content type: Case report

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  45. There is a paucity of data on long term clinical effects of high frequency chest wall oscillation (HFCWO) in the Bronchiectasis population. Other therapies such as nebulized mucolytics and long term antibiotic...

    Authors: Jordan Powner, Andrew Nesmith, Denay P. Kirkpatrick, Jessica K. Nichols, Brent Bermingham and George M. Solomon

    Citation: BMC Pulmonary Medicine 2019 19:82

    Content type: Research article

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  46. In many clinical disorders, there is a relationship between the ratio of the diameter of the main pulmonary artery (mPA) to that of the aorta (Ao) on chest computed tomography (CT). The aim of this study was t...

    Authors: Ji Soo Choi, Sang Hoon Lee, Ah. Young Leem, Joo Han Song, Kyung Soo Chung, Ji Ye Jung, Young Ae Kang, Moo Suk Park, Young Sam Kim, Joon Chang and Song Yee Kim

    Citation: BMC Pulmonary Medicine 2019 19:81

    Content type: Research article

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  47. Systemic artery to pulmonary artery fistulas (SA-PAFs), are extremely rare in people without congenital heart disease. In this group of patients pulmonary arterial hypertension was reported in the single case....

    Authors: Wojciech Jacheć, Andrzej Tomasik, Marcin Kurzyna, Radosław Pietura, Adam Torbicki, Jan Głowacki, Ewa Nowalany-Kozielska and Celina Wojciechowska

    Citation: BMC Pulmonary Medicine 2019 19:80

    Content type: Case report

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  48. In 2017, Australia experienced its highest levels of influenza virus activity since the 2009 pandemic. This allowed detailed comparison of the characteristics of patients with community and hospital-acquired i...

    Authors: Nikita Parkash, Wendy Beckingham, Patiyan Andersson, Paul Kelly, Sanjaya Senanayake and Nicholas Coatsworth

    Citation: BMC Pulmonary Medicine 2019 19:79

    Content type: Research article

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  49. CFTR modulator therapy with ivacaftor is a treatment option for Cystic Fibrosis (CF) patients with at least one copy of a R117H-7T mutation in the CFTR gene. Desirable effects of this therapy are improvement o...

    Authors: Matthias Welsner, Svenja Straßburg, Christian Taube and Sivagurunathan Sutharsan

    Citation: BMC Pulmonary Medicine 2019 19:76

    Content type: Case report

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  50. Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease, with a median survival of 2–3 years and variable natural history, characterized by gradual and progressive deterioration. Acute exacerbation of idio...

    Authors: Jean-Marc Naccache, Melissa Montil, Jacques Cadranel, Marine Cachanado, Vincent Cottin, Bruno Crestani, Dominique Valeyre, Benoit Wallaert, Tabassome Simon and Hilario Nunes

    Citation: BMC Pulmonary Medicine 2019 19:75

    Content type: Study protocol

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