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Front Immunol 2022 2;13:842745. Epub 2022 Jun 2.

Department of Pediatrics, Pennsylvania State University College of Medicine, Hershey, PA, United States.

Surfactant proteins (SPs) are important for normal lung function and innate immunity of the lungs and their genes have been identified with significant genetic variability. Changes in quantity or quality of SPs due to genetic mutations or natural genetic variability may alter their functions and contribute to the host susceptibility for particular diseases. Alternatively, SP single nucleotide polymorphisms (SNPs) can serve as markers to identify disease risk or response to therapies, as shown for other genes in a number of other studies. Read More

BMC Pulm Med 2022 May 25;22(1):207. Epub 2022 May 25.

Department of Occupational Medicine and Toxicology, Clinical Center for Interstitial Lung Diseases, Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, No. 8 Workers' Stadium South Road, Chao-Yang District, Beijing, China.

Background: Asbestosis and fibrotic hypersensitivity pneumonitis (FHP) share the pathogenetic mechanisms induced bronchiolocentric fibrotic process secondary to inhalation exposure. Under the occupational and environmental mixed exposures, asbestosis and FHP are needed to make the differential diagnoses on high-resolution computed tomography (HRCT), especially in the countries still using asbestos. The study aimed to analyze the HRCT features of asbestosis versus FHP. Read More

Am J Respir Crit Care Med 2022 May;205(10):P20-P21

Sci Rep 2022 05 4;12(1):7289. Epub 2022 May 4.

Department of Pulmonology, ISABIAL, Hospital General Universitario Dr. Balmis, Pintor Baeza, 11, 03010, Alicante, Spain.

The new radiological diagnostic criteria for diagnosing idiopathic pulmonary fibrosis (IPF) seek to optimize the indications for surgical lung biopsy (SLB). We applied the new criteria to a retrospective series of patients with interstitial lung disease (ILD) who underwent SLB in order to analyse the correlation between the radiological findings suggestive of another diagnosis (especially mosaic attenuation and its location with respect to fibrotic areas) and the usual interstitial pneumonia (UIP) pathologic diagnosis. Two thoracic radiologists reviewed the HRCT images of 83 patients with ILD and SLB, describing the radiological findings and patterns based on the new criteria. Read More

BMC Pulm Med 2022 Apr 23;22(1):153. Epub 2022 Apr 23.

Boehringer Ingelheim Pharmaceuticals, Inc., 900 Ridgebury Rd, Ridgefield, CT, 06877, USA.

Background: Interstitial lung diseases (ILDs) include a variety of parenchymal lung diseases. The most common types of ILDs are idiopathic pulmonary fibrosis (IPF), autoimmune ILDs and hypersensitivity pneumonitis (HP). There is limited real world data on care patterns of patients with chronic fibrosing ILDs with a progressive phenotype other than IPF. Read More

Transpl Int 2022 31;35:10450. Epub 2022 Mar 31.

Foundation IRCCS Cà Granda Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.

Repeated exposure to antigens inhalation is the primary cause of hypersensitivity pneumonitis, a form of interstitial pneumonia. The chronic form of hypersensitivity pneumonitis leads to progressive loss of respiratory function; lung transplantation is the only therapeutic option for chronically ill patients. The ESTS Lung Transplantation Working Group conducted a retrospective multicentred cohort study to increase the body of knowledge available on this rare indication for lung transplantation. Read More

J Immunol Methods 2022 06 11;505:113267. Epub 2022 Apr 11.

Department of Parasitology-Mycology, University Hospital of Besancon, Besancon, France; Chrono-Environment Research Team UMR/CNRS-6249, University of Bourgogne Franche-Comté, Besancon, France; Referent Biology Medical Laboratory for the serological diagnosis of hypersensitivity pneumonitis, LBMR-PHS, University Hospital of Besancon, France.

Context: Bird fancier's lung (BFL) is the most prevalent form of hypersensitivity pneumonitis (HP) worldwide. The current techniques used for the serological diagnosis of BFL all use crude extracts from feathers, droppings, and blooms as test antigens, which is associated with a lack of standardization and variability of the results. An antigenic protein, immunoglobulin lambda-like polypeptide-1 (IgLL1), isolated from pigeon droppings, was recently identified to be associated with BFL. Read More

Ital J Pediatr 2022 Mar 28;48(1):51. Epub 2022 Mar 28.

Allergy Unit, Department of Pediatrics, Meyer Children's University Hospital, Florence, Italy.

Hypersensitivity pneumonitis (HP) is a rare disease in childhood with the prevalence of 4 cases per 1 million children and an incidence of 2 cases per year. The average age of diagnosis at pediatric age is approximately 10 years. The pathogenesis of HP is characterized by an immunological reaction caused by recurrent exposure to triggering environmental agents (mostly bird antigens in children). Read More

J Antimicrob Chemother 2022 05;77(6):1779-1784

University of Cologne, Faculty of Medicine and University Hospital Cologne, Department I of Internal Medicine, Excellence Center for Medical Mycology (ECMM), Cologne, NRW, Germany.

Background: Invasive infections due to Trichosporon spp. are life-threatening opportunistic fungal infections that require complex clinical management. Guidelines assist clinicians but can be challenging to comply with. Read More

Immunohorizons 2022 03 10;6(3):224-242. Epub 2022 Mar 10.

Integrated Biomedical Science Graduate Program, The University of Tennessee Health Science Center, Memphis, TN;

Protein kinase D1 (PKD1), a ubiquitously expressed serine/threonine kinase, regulates diverse cellular processes such as oxidative stress, gene expression, cell survival, vesicle trafficking, Ag receptor signaling, and pattern recognition receptor signaling. We found previously that exposure to hypersensitivity pneumonitis (HP) inciting Ag leads to the activation of PKD1 in a MyD88-dependent manner in various types of murine cells in vitro and in the mouse lung in vivo. However, it is currently unknown whether PKD1 plays a role in the -induced HP. Read More

BMC Pulm Med 2022 Mar 4;22(1):76. Epub 2022 Mar 4.

Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-Higashi, Kanazawa-ku, Yokohama City, Kanagawa, 236-0051, Japan.

Background: Centrilobular nodules, ground-glass opacity (GGO), mosaic attenuation, air trapping, and three-density pattern were reported as high-resolution computed tomography (HRCT) findings characteristic of fibrotic hypersensitivity pneumonitis (HP). However, it is often difficult to differentiate fibrotic HP from idiopathic pulmonary fibrosis (IPF). In fibrotic HP, the HRCT sometimes shows tortoiseshell-like interlobular septal thickening that extends from the subpleural lesion to the inner layers. Read More

Pharmacoepidemiol Drug Saf 2022 05 9;31(5):519-523. Epub 2022 Mar 9.

Department of Medicine, The University of Hong Kong, Pokfulam, Hong Kong SAR, China.

Objective: Large electronic medical record (EMR) databases can facilitate epidemiology research into uncommon diseases such as interstitial lung disease (ILD). Given the rarity and diagnostic difficulty of ILD, the validity of the coding in EMR requires clarification. We aimed to assess the validity of International Classification of Diseases, 9th Revision (ICD-9) code algorithms for identifying ILD in the territory-wide electronic medical health record system of Clinical Data Analysis and Reporting System (CDARS) in Hong Kong. Read More

Commun Biol 2022 02 24;5(1):162. Epub 2022 Feb 24.

Department of Microbiology & Immunology, Cornell Center for Immunology, Cornell Institute for Host Microbe-Interactions and Disease, Cornell University, Ithaca, NY, USA.

T helper 17 (Th17) cells develop in response to T cell receptor signals (TCR) in the presence of specific environments, and produce the inflammatory cytokine IL17A. These cells have been implicated in a number of inflammatory diseases and represent a potential target for ameliorating such diseases. The kinase ITK, a critical regulator of TCR signals, has been shown to be required for the development of Th17 cells. Read More

J Pediatr Hematol Oncol 2022 Mar;44(2):e514-e517

Department of Pediatric Hematology/Oncology, Stanford University School of Medicine, Stanford, CA.

Invasive fungal disease is a difficult to diagnose complication of therapy in patients with hematologic malignancy. Antifungal prophylaxis is recommended in high-risk populations, but its use in other populations is less clear. This brief report describes a patient with Trisomy 21 on caspofungin prophylaxis who died of disseminated Trichosporon asahii during induction therapy for new diagnosis low-risk B-cell acute lymphoblastic leukemia, accompanied by a review of similar cases in the literature. Read More

Arerugi 2022 ;71(1):42-45

Department of Respiratory Medicine, Hiroshima University Hospital.

A 70-year-old woman undergoing long-term treatment for systemic scleroderma and secondary Sjögren syndrome developed fever during tapering of steroids. Chest CT showed centrilobular granular shadow and ground glass opacities. The pathology of transbronchial lung biopsy and the findings of bronchoalveolar lavage fluid were consistent with hypersensitivity pneumonitis and positive for anti-Trichosporon asahii antibody. Read More

Arkh Patol 2022 ;84(1):59-66

N.I. Pirogov Russian National Research Medical University of the Ministry of Health of Russia, Moscow, Russia.

The paper presents an X-ray morphological differential diagnosis of idiopathic pulmonary fibrosis (IPF) and fibrotic hypersensitivity pneumonitis (FHP). It describes the etiology, pathogenesis, radiological signs, and pathoanatomy of IPF and FHP. For differential diagnosis, radiological and morphological signs were studied in 105 patients with IPF and in 111 patients with FHP. Read More

Am J Respir Crit Care Med 2022 04;205(8):967-968

Amsterdam University Medical Centers Amsterdam, the Netherlands.

Respirology 2022 03 10;27(3):192-193. Epub 2022 Feb 10.

Central Clinical School, Monash University, Melbourne, Victoria, Australia.

Eur Respir Rev 2022 Mar 9;31(163). Epub 2022 Feb 9.

Department of Respiratory Medicine, University Thomayer Hospital, Prague, Czech Republic.

Hypersensitivity pneumonitis (HP) is a complex and heterogeneous interstitial lung disease (ILD) that occurs when susceptible individuals develop an exaggerated immune response to an inhaled antigen. In this review, we discuss the latest guidelines for the diagnostic evaluation of patients with suspected HP, the importance of identifying patients with fibrotic and progressive disease, and the evidence supporting the drugs commonly used in the treatment of HP. Differential diagnosis of HP can be challenging and requires a thorough exposure history, multidisciplinary discussion of clinical and radiologic data, and, in some cases, assessment of bronchoalveolar lavage lymphocytosis and histopathologic findings. Read More

Am J Med 2022 May 5;135(5):e104-e105. Epub 2022 Feb 5.

Department of General Internal Medicine, Tokyo Metropolitan Tama Medical Center, Japan; Department of Respiratory Medicine, Tokyo Metropolitan Tama Medical Center, Japan.

Chest 2022 02;161(2):307-308

Department of Health Sciences, University of Leicester, Leicester, England. Electronic address:

BMC Pulm Med 2022 Feb 1;22(1):49. Epub 2022 Feb 1.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN, 55905, USA.

Background: Recent studies support the diagnostic role of bronchoalveolar lavage lymphocytosis (BALL) in patients with suspected hypersensitivity pneumonitis (HP). Our study aim was to determine the spectrum of BALL findings with elimination of incorporation bias in non-fibrotic and fibrotic patients and assess correlates of positive BALL cut-off and BALL association with long-term outcomes in those with fibrotic disease (f-HP).

Methods: A single-center retrospective cohort study was pursued of patients undergoing diagnostic bronchoscopy for interstitial lung disease. Read More

Respirology 2022 03 12;27(3):202-208. Epub 2022 Jan 12.

Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK.

Background And Objective: A proportion of patients with fibrotic hypersensitivity pneumonitis (fHP) follow a progressive disease course despite immunosuppressive treatment. Little is known about predictors of mortality in fHP. We aimed to investigate the impact of short-term lung function changes in fHP on mortality. Read More

Zhonghua Jie He He Hu Xi Za Zhi 2022 Jan;45(1):95-99

Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.

The significant scientific advances about interstitial lung disease from November 2020 to October 2021 which were published in the Chinese and international journals were systematically reviewed in this paper. The year 2021 brought advances in our understanding of the real-world adoption of the antifibrotic medications pirfenidone and nintedanib for US idiopathic pulmonary fibrosis patients, the European statement on the diagnosis and evaluation and treatment of pulmonary fibrosis, the CHEST guideline on the diagnosis and evaluation of hypersensitivity pneumonitis, Chinese expert viewpoints on the progressive fibrosing interstitial lung disease, and international updates on the diagnosis and management of connective tissue disease-associated interstitial lung diseases. Read More

Respir Investig 2022 May 5;60(3):369-378. Epub 2022 Jan 5.

Department of Respiratory Medicine, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan. Electronic address:

Background: Serum Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) are candidate diagnostic and activity markers for fibrotic hypersensitivity pneumonitis (HP), although their correlation with prognosis remains unclear. We aimed to evaluate the prognostic usefulness of serum KL-6 and SP-D in patients with fibrotic HP.

Methods: This was a retrospective medical record review of 185 patients with fibrotic HP at a single center from 2005 to 2019. Read More

Thorax 2022 05 7;77(5):508-510. Epub 2022 Jan 7.

Department of Medicine, University of Colorado School of Medicine, Aurora, Colorado, USA

A subset of patients with hypersensitivity pneumonitis (HP) develop lung fibrosis that is clinically similar to idiopathic pulmonary fibrosis (IPF). To address the aetiological determinants of fibrotic HP, we investigated whether the common IPF genetic risk variants were also relevant in study subjects with fibrotic HP. Our findings indicate that common genetic variants in , , and were significantly associated with fibrotic HP. Read More

Ochsner J 2021 ;21(4):425-430

Department of Internal Medicine, Michigan State University-Sparrow Hospital, Lansing, MI.

Hypersensitivity pneumonitis, also known as extrinsic allergic alveolitis, is a pulmonary disease with large knowledge gaps, including etiology, pathogenesis, diagnosis, and treatment. A 58-year-old male with a pertinent history of recurrent presented to a tertiary emergency department after 1 week of difficulty breathing. He also reported a productive cough and fevers. Read More

J Allergy Clin Immunol 2022 Jun 30;149(6):2091-2104. Epub 2021 Dec 30.

Institute for Immunology, Tsinghua University, Beijing, China; Department of Basic Medical Sciences, School of Medicine, Tsinghua University, Beijing, China; Beijing Key Lab for Immunological Research on Chronic Diseases, Tsinghua University, Beijing, China. Electronic address:

Background: Group 2 innate lymphoid cells (ILC2s), the innate counterpart of T2 cells, play a critical role in type 2 immune responses. However, the molecular regulatory mechanisms of ILC2s are still unclear.

Objective: The aim of this study was to explore the importance of signal transducer and activator of transcription 3 (STAT3) to ILC2 function in allergic lung inflammation. Read More

Cells 2021 12 15;10(12). Epub 2021 Dec 15.

Interstitial Lung Diseases Centre of Excellence, Department of Pulmonology, St. Antonius Hospital, 3435 CM Nieuwegein, The Netherlands.

Mechanistic target of rapamycin complex 1 (mTORC1) has been linked to different diseases. The mTORC1 signaling pathway is suggested to play a role in the granuloma formation of sarcoidosis. Recent studies demonstrated conflicting data on mTORC1 activation in patients with sarcoidosis by measuring activation of its downstream target S6 kinase (S6K) with either 33% or 100% of patients. Read More

Ophthalmology 2022 01;129(1):66

IRCCS - Fondazione Bietti, Rome, Italy.