2,276 results match your criteria Pulmonary Alveolar Proteinosis


Correction of a chronic pulmonary disease through lentiviral vector-mediated protein expression.

Mol Ther Methods Clin Dev 2022 Jun 14;25:382-391. Epub 2022 Apr 14.

National Heart and Lung Institute, Gene Therapy Group, Imperial College London, Faculty of Medicine, Manresa Road, London SW3 6LR, UK.

We developed a novel lentiviral vector, pseudotyped with the F and HN proteins from Sendai virus (rSIV.F/HN), that produces long-lasting, high-efficiency transduction of the respiratory epithelium. Here we addressed whether this platform technology can secrete sufficient levels of a therapeutic protein into the lungs to ameliorate a fatal pulmonary disease as an example of its translational capability. Read More

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Single lung transplantation for idiopathic pulmonary alveolar proteinosis with intraoperative veno-venous extracorporeal membrane oxygenation support.

Transpl Immunol 2022 May 11:101627. Epub 2022 May 11.

Department of Thoracic Surgery, The Affiliated Wuxi People's Hospital of Nanjing Medical University, Wuxi, Jiangsu, China. Electronic address:

Pulmonary alveolar proteinosis (PAP) is a rarely progressive disease. This disease is characterized by the accumulation of a large amount of pulmonary surfactant in the alveolar cavity and terminal bronchiole, which is caused by the obstruction of clearance due to the weakened function of alveolar macrophages in vivo. Idiopathic PAP(IPAP) is the most common type of PAP, accounting for about 90%, and its pathogenesis remains unclear. Read More

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Autoimmune pulmonary alveolar proteinosis with features similar to nonspecific interstitial pneumonia.

Respir Med Case Rep 2022 3;36:101591. Epub 2022 Feb 3.

Division of Respirology, NTT Medical Center Tokyo, Tokyo, Japan.

A 58-year-old woman with cough and dyspnea who was suspected of having idiopathic interstitial pneumonia had been treated with corticosteroids and cyclosporine, but the symptoms had worsened. There were no findings to suspect pulmonary alveolar proteinosis (PAP) in the bronchoalveolar lavage fluid, 17 months after the start of treatment. The transbronchial lung biopsy specimens showed eosinophilic bodies that strongly stained with periodic acid-Schiff staining. Read More

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February 2022

Opportunistic Infection Associated With Elevated GM-CSF Autoantibodies: A Case Series and Review of the Literature.

Open Forum Infect Dis 2022 May 9;9(5):ofac146. Epub 2022 Apr 9.

UCLA Division of Pulmonary, Critical Care, and Sleep Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California, USA.

Granulocyte-macrophage colony-stimulating factor (GM-CSF) is known to play a key role in enhancing multiple immune functions that affect response to infectious pathogens including antigen presentation, complement- and antibody-mediated phagocytosis, microbicidal activity, and neutrophil chemotaxis. Reduced GM-CSF activity and immune response provides a mechanism for increased infection risk associated with autoimmune pulmonary alveolar proteinosis (aPAP) and other disorders involving the presence of GM-CSF autoantibodies. We present a case series of five patients with persistent or unusual pulmonary and central nervous system opportunistic infections (Cryptococcus gattii, Flavobacterium, Nocardia) and elevated GM-CSF autoantibody levels, as well as 27 cases identified on systematic review of the literature. Read More

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An Infant With Interstitial Lung Disease of Rare Cause.

Chest 2022 May;161(5):e273-e278

Department of Pediatrics, Peking University First Hospital, Beijing, China. Electronic address:

Case Presentation: An 11-month-old boy was admitted to our hospital because of "recurrent cough with intermittent dyspnea for more than 8 months, aggravated for 1 month." The baby began experiencing a recurrent milk-choking problem within 1.5 months after birth. Read More

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Targeted therapy for pulmonary alveolar proteinosis: the time is now.

Eur Respir J 2022 04 21;59(4). Epub 2022 Apr 21.

Université de Paris, Inserm, U1152, laboratoire d'excellence INFLAMEX, Paris, France.

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Efficacy and safety of whole-lung lavage for pulmonary alveolar proteinosis: a protocol for a systematic review and meta-analysis.

BMJ Open 2022 Apr 20;12(4):e057671. Epub 2022 Apr 20.

Guanganmen Hospital, China Academy of Traditional Chinese Medicine, Beijing, China

Introduction: Pulmonary alveolar proteinosis (PAP) is an ultrarare disorder characterised by the accumulation of alveolar surfactant and the dysfunction of alveolar macrophages that results in hypoxemic respiratory failure. Whole-lung lavage (WLL) is currently the primary therapy for PAP. However, systematic evaluation of the clinical efficacy of WLL is lacking. Read More

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Dilutional acidosis during whole lung lavage under general anesthesia due to excessive absorption of normal saline.

JA Clin Rep 2022 Apr 14;8(1):30. Epub 2022 Apr 14.

Department of Anesthesiology and Pain Medicine, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo, 113-8421, Japan.

Background: Whole lung lavage (WLL) is an effective therapy for pulmonary alveolar proteinosis. We report a rare dilutional acidosis following WLL in a female patient.

Case Presentation: Under general anesthesia, a left-sided double-lumen tube was inserted with its bronchial lumen connected to the saline delivery system. Read More

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Gene therapy of Csf2ra deficiency in mouse fetal monocyte precursors restores alveolar macrophage development and function.

JCI Insight 2022 04 8;7(7). Epub 2022 Apr 8.

Institute of Molecular Health Sciences, Department of Biology, ETH Zurich, Zurich, Switzerland.

Tissue-resident macrophage-based immune therapies have been proposed for various diseases. However, generation of sufficient numbers that possess tissue-specific functions remains a major handicap. Here, we showed that fetal liver monocytes cultured with GM-CSF (CSF2-cFLiMo) rapidly differentiated into a long-lived, homogeneous alveolar macrophage-like population in vitro. Read More

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A Bibliometric Analysis of Pulmonary Alveolar Proteinosis From 2001 to 2021.

Front Med (Lausanne) 2022 22;9:846480. Epub 2022 Mar 22.

Guanganmen Hospital, China Academy of Chinese Medical Sciences, Beijing, China.

Background: Pulmonary alveolar proteinosis (PAP) is a rare syndrome first described by Rosen et al. in 1958. Despite our considerably evolved understanding of PAP over the past decades, no bibliometric studies have been reported on this field. Read More

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[Whole lung lavage in the case of hereditary pulmonary alveolar proteinosis].

Zhonghua Er Ke Za Zhi 2022 Apr;60(4):350-352

Department of Pediatrics, the First Affiliated Hospital of Guangxi Medical University, Nanning 530021,China.

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Unilateral Autoimmune Pulmonary Alveolar Proteinosis with Polymyositis-related Interstitial Lung Disease.

Intern Med 2022 Apr 2. Epub 2022 Apr 2.

Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Japan.

A 61-year-old patient with cystic bronchiectasis and bronchial artery hyperplasia in the left lung was diagnosed with polymyositis-related interstitial lung disease. After nine months of immunosuppressive therapy, he developed unilateral autoimmune pulmonary alveolar proteinosis (APAP) in the right lung with respiratory failure. After bronchial artery embolization to prevent massive hemoptysis, whole-lung lavage was performed using veno-venous extracorporeal membrane oxygenation. Read More

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Autoimmune pulmonary alveolar proteinosis exacerbated by steroid therapy due to misdiagnosis as anti-aminoacyl-tRNA synthetase (ARS) antibody positive- interstitial pneumonia: a case report.

BMC Pulm Med 2022 Mar 31;22(1):120. Epub 2022 Mar 31.

Department of Respiratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan.

Background: Anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies are myositis-specific autoantibodies that have been identified in a subset of patients with interstitial pneumonia who do not present with dermatomyositis or polymyositis. Anti-ARS antibody-positive interstitial pneumonia is commonly treated with steroids or immunosuppressive agents and is usually responsive to these therapies. Here, we present in detail a case in which respiratory failure of a patient diagnosed with anti-ARS antibody-positive interstitial pneumonia was exacerbated by treatment with steroids and immunosuppressive agents. Read More

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Pathology and Mineralogy Demonstrate Respirable Crystalline Silica is a Major Cause of Severe Pneumoconiosis in US Coal Miners.

Ann Am Thorac Soc 2022 Mar 30. Epub 2022 Mar 30.

University of Calgary Medical Centre, 70401, Histopathology, Calgary, Alberta, Canada.

Rationale: The reasons for resurgent coal workers' pneumoconiosis and its most severe forms, rapidly progressive pneumoconiosis and progressive massive fibrosis (PMF), in the United States (US) are not yet fully understood.

Objective: To compare the pathologic and mineralogic features of contemporary coal miners suffering severe pneumoconiosis to their historical counterparts.

Methods: Lung pathology specimens from 85 coal miners with PMF were included for evaluation and analysis. Read More

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Paraneoplastic Pulmonary Alveolar Proteinosis.

Am J Respir Crit Care Med 2022 Mar 30. Epub 2022 Mar 30.

Massachusetts General Hospital Cancer Center, 136312, Boston, Massachusetts, United States.

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Autoimmune pulmonary alveolar proteinosis in children.

ERJ Open Res 2022 Jan 21;8(1). Epub 2022 Mar 21.

2nd Pulmonary Medicine Dept, General University Hospital "Attikon", Medical School, National and Kapodistrian University of Athens, Athens, Greece.

In childhood, a multitude of causes lead to pulmonary alveolar proteinosis (PAP), an excessive surfactant accumulation in the alveolar space, limiting gas exchange. Autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) causing autoimmune PAP, the principal aetiology in adults, are rare. In this first case series on autoimmune PAP, we detail the presentation and management issues of four children. Read More

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January 2022

Rituximab rescue therapy for autoimmune pulmonary alveolar proteinosis.

Respir Med Case Rep 2022 21;37:101637. Epub 2022 Mar 21.

Department of Respiratory Medicine, Gold Coast University Hospital, Hospital Blvd, Southport, Queensland, Australia, 4215.

Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare lung disease characterised by abnormal alveolar surfactant accumulation due to macrophage dysfunction. Whole lung lavage (WLL) is the cornerstone of first-line aPAP therapy, but effective rescue treatments have not yet been well established. We report a case of a 41-year-old man with aPAP in whom further WLL is contraindicated. Read More

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A case series of pulmonary alveolar proteinosis: Response differently to whole lung lavage.

Med J Malaysia 2022 03;77(2):261-263

Pusat Perubatan Universiti Kebangsaan Malaysia, Department of Internal Medicine, Respiratory Unit, Malaysia.

Pulmonary alveolar proteinosis (PAP) is a rare disease and its prognosis can be improved by whole lung lavage (WLL). Herein, we present three cases with idiopathic PAP treated successfully with either single or double WLL in the same setting. All three of them presented with exertional dyspnoea with radiographic findings of pulmonary infiltrates. Read More

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Pulmonary Alveolar Proteinosis and Multiple Infectious Diseases in a Child with Autosomal Recessive Complete IRF8 Deficiency.

J Clin Immunol 2022 Mar 26. Epub 2022 Mar 26.

Department of Immunology and Rheumatology, "J. P.Garrahan" National Hospital of Pediatrics, Buenos Aires, Argentina.

Background: Autosomal recessive (AR) complete IRF8 deficiency is a rare severe inborn error of immunity underlying an absence of blood myeloid mononuclear cells, intracerebral calcifications, and multiple infections. Only three unrelated patients have been reported.

Materials And Methods: We studied an Argentinian child with multiple infectious diseases and severe pulmonary alveolar proteinosis (PAP). Read More

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Human autoantibodies underlying infectious diseases.

J Exp Med 2022 04 23;219(4). Epub 2022 Mar 23.

Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Institut national de la santé et de la recherche médicale, Necker Hospital for Sick Children, Paris, France.

The vast interindividual clinical variability observed in any microbial infection-ranging from silent infection to lethal disease-is increasingly being explained by human genetic and immunological determinants. Autoantibodies neutralizing specific cytokines underlie the same infectious diseases as inborn errors of the corresponding cytokine or response pathway. Autoantibodies against type I IFNs underlie COVID-19 pneumonia and adverse reactions to the live attenuated yellow fever virus vaccine. Read More

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A mini-whole lung lavage to treat autoimmune pulmonary alveolar proteinosis (PAP).

Respir Res 2022 Mar 17;23(1):60. Epub 2022 Mar 17.

Pneumology Unit, IRCCS San Matteo Hospital Foundation, Pavia, Italy.

Background: PAP is an ultra-rare respiratory syndrome characterized by the accumulation of surfactant within the alveoli. Whole lung lavage (WLL) is the current standard of care of PAP, however it is not a standardized procedure and the total amount of fluid used to wash each lung is still debated. Considering ICU hospitalization associated risks, a "mini-WLL" with anticipated manual clapping and reduced total infusion volume and has been proposed in our center. Read More

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Pediatric Lung MRI in Pulmonary Alveolar Proteinosis: An Alternative to CT as a Radiation-Free Modality.

Indian J Pediatr 2022 06 9;89(6):616-617. Epub 2022 Mar 9.

Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

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[Segmental Bronchoalveolar Lavage Provided a Favorable Clinical Course in a Case of Autoimmune Pulmonary Alveolar Proteinosis].

J UOEH 2022 ;44(1):83-89

Department of Thoracic Surgery, Kitakyushu General Hospital. Kokurakita-ku, Kitakyushu 802-8517 Japan.

A 49-year-old man was diagnosed with autoimmune pulmonary alveolar proteinosis. Chest computed tomography (CT) showed typical CT findings of pulmonary alveolar proteinosis: thickening of septa with ground-glass opacities in both lung fields. The diagnosis of autoimmune pulmonary alveolar proteinosis (PAP) was based on findings of bronchoalveolar lavage (BAL) fluid with milky appearance and elevated serum titer of anti-granulocyte-macrophage colony-stimulating factor antibody. Read More

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Autoimmune Pulmonary Alveolar Proteinosis.

Am J Respir Crit Care Med 2022 May;205(9):1016-1035

Translational Pulmonary Science Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; and.

Autoimmune pulmonary alveolar proteinosis (PAP) is a rare disease characterized by myeloid cell dysfunction, abnormal pulmonary surfactant accumulation, and innate immune deficiency. It has a prevalence of 7-10 per million; occurs in individuals of all races, geographic regions, sex, and socioeconomic status; and accounts for 90% of all patients with PAP syndrome. The most common presentation is dyspnea of insidious onset with or without cough, production of scant white and frothy sputum, and diffuse radiographic infiltrates in a previously healthy adult, but it can also occur in children as young as 3 years. Read More

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Pulmonary and Systemic Toxicity in a Rat Model of Pulmonary Alveolar Proteinosis Induced by Indium-Tin Oxide Nanoparticles.

Int J Nanomedicine 2022 17;17:713-731. Epub 2022 Feb 17.

School of Public Health, North China University of Science and Technology, Tangshan, 063210, Hebei, People's Republic of China.

Purpose: The main objective of this study was to clarify the biodistribution and in vivo toxicological effects of indium-tin oxide nanoparticles (Nano-ITO) in male rats.

Methods: Dose-response (three divided doses) and time-course studies (six exposure durations) were performed to examine Nano‑ITO-induced pulmonary and systemic toxicity. At the end of the experiment, hematology and serum biochemical parameters were determined, and cytokines levels and oxidative stress were analyzed in the bronchoalveolar lavage fluid. Read More

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February 2022

Serum proteome analysis of systemic JIA and related lung disease identifies distinct inflammatory programs and biomarkers.

Arthritis Rheumatol 2022 Feb 21. Epub 2022 Feb 21.

Pediatrics, UPMC Children's Hospital & University of Pittsburgh Medical Center, Pittsburgh, PA, USA.

Objectives: Recent observations in systemic Juvenile Idiopathic Arthritis (sJIA) suggest an increasing incidence of high-mortality interstitial lung disease (sJIA-LD) often characterized by a variant of pulmonary alveolar proteinosis (PAP). Co-occurrence of macrophage activation syndrome (MAS) and PAP in sJIA suggested a shared pathology, but sJIA-LD patients also commonly experience features of drug reaction such as atypical rashes and eosinophilia. We sought to investigate immunopathology and identify biomarkers in sJIA, MAS, and sJIA-LD. Read More

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February 2022

Nebulised sargramostim in pulmonary alveolar proteinosis.

Br J Clin Pharmacol 2022 Feb 13. Epub 2022 Feb 13.

Department of Medicine, Monash University, Melbourne, Australia.

Five patients, comprising nine treatment courses of sargramostim use in pulmonary alveolar proteinosis, are described. The prevailing standard of treatment, whole lung lavage (WLL), is highly invasive, resource intensive and carries some procedural risk. Nebulised recombinant human GM-CSF (sargramostim) offers a pharmacological treatment option, allowing patients to be treated at home, possessing potential advantages in patient experience and wider health resourcing. Read More

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February 2022

Pulmonary Alveolar Proteinosis Refractory to Plasmapheresis and Rituximab despite GM-CSF Antibody Reduction.

Case Reports Immunol 2022 30;2022:2104270. Epub 2022 Jan 30.

Department of Pathology, University of Wisconsin Hospital, 600 Highland Ave, Madison, WI 53792, USA.

We share our experience of a patient with pulmonary alveolar proteinosis who was refractory to plasmapheresis and rituximab despite a significant reduction in the offending antibody. He presented with shortness of breath, fevers, chills, and sweats for 4 months. He was diagnosed with autoimmune PAP based on typical radiology findings, bronchoalveolar fluid analysis, and elevated anti-GM-CSF levels. Read More

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January 2022