2,183 results match your criteria Pulmonary Alveolar Proteinosis


Long-term follow-up and successful treatment of pulmonary alveolar proteinosis without hypercholesterolemia with statin therapy: a case report.

J Int Med Res 2021 Apr;49(4):3000605211010046

Department of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Beijing, China.

Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by the accumulation of excessive surfactant lipids and proteins in alveolar macrophages and alveoli. Oral statin therapy is a novel treatment for PAP with hypercholesterolemia. However, this treatment has never been described in a patient without hypercholesterolemia. Read More

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Can YKL-40 be used as a biomarker for interstitial lung disease?: A systematic review and meta-analysis.

Medicine (Baltimore) 2021 Apr;100(17):e25631

Department of Respiratory Medicine and Critical Care Medicine, West China Hospital/West China School of Medicine, Sichuan University.

Background: Interstitial lung disease (ILD) has a poor prognosis and lacks specific biomarkers for early diagnosis, assessment of disease severity, and prognosis. YKL-40 levels were found to be elevated in patients with ILD, but these results are inconsistent. Therefore, we conducted a systematic review and meta-analysis to accurately study the relation between YKL-40 and ILD. Read More

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[Pulmonary alveolar proteinosis].

Medicina (B Aires) 2021 ;81(2):301-303

Servicio de Clínica Médica, Instituto de Investigaciones Médicas Alfredo Lanari, Buenos Aires, Argentina.

Pulmonary alveolar proteinosis (PAP) is a rare, diffuse pulmonary disease due to abnormal surfactant homeostasis. We present the case of a 69-year-old woman who was admitted to the hospital for progressive dyspnea with marked limitation in activity, and non-productive cough, of three months of evolution. Type I respiratory failure was confirmed. Read More

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Recommended Reading from the University of Florida Fellows.

Am J Respir Crit Care Med 2021 Apr 23. Epub 2021 Apr 23.

University of Florida, 3463, Pulmonary, Critical Care and Sleep Medicine, Gainesville, Florida, United States;

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Mild dyspnea presenting as 'crazy-paving' on chest computed tomography.

Authors:
M Oudah D Slack

J Community Hosp Intern Med Perspect 2021 Mar 23;11(2):273-276. Epub 2021 Mar 23.

Department of Pulmonology and Critical Care Medicine, Greater Baltimore Medical Center, Towson, Maryland, USA.

Pulmonary alveolar proteinoisis (PAP) is a rare disease characterized by accumulation of proteinaceous material in the alveolar spaces. Here, we report a case of mild dyspnea with incidental 'crazy-paving' pattern on chest computed tomography (CT). Further evaluation and bronchoscopy found to have PAP. Read More

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Bilateral whole lung lavage by lung isolation in a child with pulmonary alveolar proteinosis: A new technique.

Ann Card Anaesth 2021 Apr-Jun;24(2):266-268

Department of Cardiac Anaesthesia, Cardiothoracic Centre, AIIMS, New Delhi, India.

We present two rare cases of children who presented with progressive exertional breathlessness and dry cough. They also had history of bluish discoloration of mucous membranes, hands and feet on exertion. Both were diagnosed to have pulmonary alveolar proteinosis after a high-resolution computed tomography and bronchoalveolar lavage. Read More

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A lung mass: Not the usual suspect, but not above suspicion.

Respir Med Case Rep 2021 18;33:101389. Epub 2021 Mar 18.

Department of Respiratory and Critical Care Medicine, Changi General Hospital, Singapore.

Lipoid pneumonia is an entity that is not commonly thought of when faced with opacities on the chest radiograph. The radiological changes of lipoid pneumonia can mimic pneumonia or malignancy. Due to this mimicry, it is commonly missed and alternative diagnoses should always be considered and ruled out when suspecting lipoid pneumonia. Read More

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A case of progressive pulmonary alveolar proteinosis with lymphadenopathy revealed on F-FDG PET/CT.

Hell J Nucl Med 2021 Jan-Apr;24(1):94-95. Epub 2021 Apr 20.

Department of Nuclear Medicine, West China Hospital, Sichuan University, No. 37. Guoxue Alley, 610041 Chengdu, P.R. China. zhang_

Pulmonary alveolar proteinosis (PAP) is a rare lung disease, which may cause repeating infections. A 36-year-old man had repetitive admissions to our hospital, beginning two years ago, due to episodes of severe dyspnea. Serial computed tomography (CT) scans revealed extensive ground-glass opacities with interlobular/intralobular septal thickening, diffuse consolidations in both lungs and enlarged lower paratracheal lymph nodes. Read More

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Expression profiles and potential functions of long noncoding RNAs and mRNAs in autoimmune pulmonary alveolar proteinosis patients.

Aging (Albany NY) 2021 Apr 4;13(7):10535-10554. Epub 2021 Apr 4.

Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.

Autoimmune pulmonary alveolar proteinosis (APAP) is a rare lung disease that may be associated with surfactant overaccumulation. To assess the function of long noncoding RNAs (lncRNAs) in APAP, we performed microarray analyses to identify differentially expressed (DE) lncRNAs and mRNAs between peripheral blood samples from five APAP patients and five healthy volunteers. In total, 12459 DE lncRNAs and 9331 DE mRNAs were identified in APAP patient samples. Read More

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ABCA3 deficiency dramatically improved by azithromycin administration.

Pediatr Int 2021 Apr 5. Epub 2021 Apr 5.

Aichi Children's Health and Medical Center, Obu, Aichi, Japan.

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Correlation of Lipid Ratios With the Severity of Pulmonary Alveolar Proteinosis: A Cross-Sectional Study.

Front Nutr 2021 18;8:610765. Epub 2021 Mar 18.

Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, China.

Lipids are known to accumulate abnormally in the alveoli and circulate during pulmonary alveolar proteinosis (PAP). However, the relationship between lipid ratios and PAP is not clear. In this study, we investigated the lipid ratios in PAP patients and explored the relationships between lipid ratios and the severity of PAP. Read More

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Autoimmune pulmonary alveolar proteinosis and idiopathic pulmonary haemosiderosis: a dual pathology.

BMJ Case Rep 2021 Apr 2;14(4). Epub 2021 Apr 2.

Department of Respiratory Medicine, Cork University Hospital, Cork, Ireland

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary condition which leads to excessive accumulation of proteinaceous material within the alveoli. Idiopathic pulmonary haemosiderosis (IPH) is another orphan lung disease and results in recurrent alveolar haemorrhage. This case study describes a case of these two rare pathologies occurring together. Read More

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Beyond "Big Eaters": The Versatile Role of Alveolar Macrophages in Health and Disease.

Int J Mol Sci 2021 Mar 24;22(7). Epub 2021 Mar 24.

REBIRTH Research Center for Translational and Regenerative Medicine, Hannover Medical School, 30625 Hannover, Germany.

Macrophages act as immune scavengers and are important cell types in the homeostasis of various tissues. Given the multiple roles of macrophages, these cells can also be found as tissue resident macrophages tightly integrated into a variety of tissues in which they fulfill crucial and organ-specific functions. The lung harbors at least two macrophage populations: interstitial and alveolar macrophages, which occupy different niches and functions. Read More

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Systemic Pulmonary Events Associated with Myelodysplastic Syndromes: A Retrospective Multicentre Study.

J Clin Med 2021 Mar 10;10(6). Epub 2021 Mar 10.

Department of Internal Medicine and Clinical Immunology, National Reference Centre for Rare Systemic Autoimmune Disease North and North-West of France, University of Lille, CHU Lille, F-59000 Lille, France.

Although pulmonary events are considered to be frequently associated with malignant haemopathies, they have been sparsely studied in the specific context of myelodysplastic syndromes (MDS). We aimed to describe their different types, their relative proportions and their relative effects on overall survival (OS). We conducted a multicentre retrospective cohort study. Read More

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Usefulness and safety of transbronchial biopsy with large forceps during flexible bronchoscopy.

Proc (Bayl Univ Med Cent) 2020 Oct 28;34(2):232-236. Epub 2020 Oct 28.

Division of Pulmonary and Critical Care, Cooper Medical School at Rowan University, Camden, New Jersey.

Transbronchial lung biopsy (TBLB) using forceps is one of the most common procedures used to obtain lung tissue. The procedure's usefulness remains limited when diagnosing interstitial lung diseases. This retrospective descriptive study analyzed the feasibility and safety of using large forceps for TBLB in all patients who underwent TBLB from 2014 to 2018 for diffuse lung disease where the diagnosis could not be made by high-resolution chest computed tomography. Read More

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October 2020

Clinical characteristics of pulmonary alveolar proteinosis.

Zhong Nan Da Xue Xue Bao Yi Xue Ban 2021 Feb;46(2):156-161

Department of Respiratory and Critical Care Medicine, Second Xiangya Hospital; Research Unit of Respiratory Disease, Central South University; Respiratory Disease Diagnosis and Treatment Center of Hunan Province, Changsha 410011, China.

Objectives: Pulmonary alveolar proteinosis (PAP) is a rare disease with non-specific and various clinical manifestations, often leading to misdiagnosis. This study aims to raise the awareness of this disease via summarizing the clinical characteristics, diagnosis, and therapy of PAP.

Methods: We retrospectively analyzed clinical data of 25 hospitalized cases of PAP during 2008 and 2019 in the Department of Respiratory and Critical Care Medicine of the Second Xiangya Hospital of Central South University. Read More

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February 2021

B cell-activating factors in autoimmune pulmonary alveolar proteinosis.

Orphanet J Rare Dis 2021 03 2;16(1):115. Epub 2021 Mar 2.

Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, 1180 Nagasone-Cho, Kita-Ku, Sakai City, Osaka, 591-8555, Japan.

Background: Autoimmune pulmonary alveolar proteinosis (APAP) results from the suppression of granulocyte-macrophage colony-stimulating factor (GM-CSF) signaling by a neutralizing autoantibody against GM-CSF. B cell-activating factor (BAFF) and a proliferation-inducing ligand (APRIL) are involved in immunoglobulin G production and are overproduced in various autoimmune disorders. We hypothesized that BAFF and/or APRIL levels would be elevated in serum and bronchoalveolar lavage fluid (BALF) and serum and BALF levels of BAFF and APRIL respond to the treatments (whole lung lavage (WLL) or inhalation of recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF)) in patients with APAP. Read More

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Chronic myelomonocytic leukemia-associated pulmonary alveolar proteinosis: A case report and review of literature.

World J Clin Cases 2021 Feb;9(5):1156-1167

Department of Hematology, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China.

Background: Pulmonary alveolar proteinosis (PAP) is a rare condition that can cause progressive symptoms including dyspnea, cough and respiratory insufficiency. Secondary PAP is generally associated with hematological malignancies including chronic myelomonocytic leukemia (CMML). To the best of our knowledge, this is the first reported case of PAP occurring secondary to CMML. Read More

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February 2021

Hereditary pulmonary alveolar proteinosis as collateral damage from a large chromosomal deletion.

Pediatr Pulmonol 2021 Feb 25. Epub 2021 Feb 25.

Department of Pediatric Respiratory Medicine, Royal Brompton Hospital, London, UK.

A girl with a known chromosomal deletion at Xp22.33, learning difficulties and short stature presented with dyspnea and dry cough and an abnormal chest X-ray. Computed tomography was typical for pulmonary alveolar proteinosis (PAP), and the diagnosis was confirmed invasively. Read More

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February 2021

Granulocyte Macrophage Colony-Stimulating Factor-Specific Autoantibodies and Cerebral Nocardia With Pulmonary Alveolar Proteinosis.

Open Forum Infect Dis 2021 Feb 13;8(2):ofaa612. Epub 2020 Dec 13.

Aix-Marseille Université, IRD Institut de Recherche et de Developpement, APHM Assistance publique des Hôpitaux de Marseille, MEPHI Microbes, Evolution, Phylogénie et Infection, Marseille, France.

In this study, we report the history of a 40-year-old man with a primary cerebral abscess caused by that led to the discovery of autoimmune pulmonary alveolar lipoproteinosis (anti-granulocyte-macrophage colony-stimulating factor [GM-CSF] autoantibodies). Anti-GM-CSF autoantibodies promote immunodeficiency and should be monitored to prevent opportunistic and disseminated infections and to diagnose asymptomatic pulmonary alveolar lipoproteinosis. Read More

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February 2021

The actin-regulatory protein Hem-1 is essential for alveolar macrophage development.

J Exp Med 2021 Apr;218(4)

Department of Comparative Medicine, University of Washington, Seattle, WA.

Hematopoietic protein-1 (Hem-1) is a hematopoietic cell-specific actin-regulatory protein. Loss-of-function (LOF) variants in the NCKAP1L gene encoding Hem-1 have recently been found to result in primary immunodeficiency disease (PID) in humans, characterized by recurring respiratory infections, asthma, and high mortality. However, the mechanisms of how Hem-1 variants result in PID are not known. Read More

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Genetic determinants of risk in autoimmune pulmonary alveolar proteinosis.

Nat Commun 2021 02 15;12(1):1032. Epub 2021 Feb 15.

Department of Respiratory Medicine, Kobe City Medical Center General Hospital, Kobe, Japan.

Pulmonary alveolar proteinosis (PAP) is a devastating lung disease caused by abnormal surfactant homeostasis, with a prevalence of 6-7 cases per million population worldwide. While mutations causing hereditary PAP have been reported, the genetic basis contributing to autoimmune PAP (aPAP) has not been thoroughly investigated. Here, we conducted a genome-wide association study of aPAP in 198 patients and 395 control participants of Japanese ancestry. Read More

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February 2021

Hypersensitivity pneumonitis in a patient with pulmonary alveolar proteinosis.

Pulmonology 2021 Feb 4. Epub 2021 Feb 4.

Department of Pulmonology, Centro Hospitalar Vila Nova de Gaia, Espinho, Portugal.

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February 2021

Sleep in children and young adults with interstitial and diffuse lung disease.

Sleep Med 2021 Apr 20;80:23-29. Epub 2021 Jan 20.

AP-HP, Hôpital Necker, Pediatric Noninvasive Ventilation and Sleep Unit, F-75015, Paris, France; Université de Paris, VIFASOM, F-75004, Paris, France. Electronic address:

Objective: Obstructive sleep apnea (OSA) is common in adult patients with interstitial lung disease (ILD). The aim of the study was to evaluate the prevalence of OSA and sleep quality in children and young adults with children's interstitial and diffuse lung disease (chILD).

Methods: A polysomnography (PSG) was performed in room air in all consecutive patients followed at a national reference centre between June 2018 and September 2019. Read More

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The Significance of Subpleural Sparing in CT Chest: A State-of-the-Art Review.

Am J Med Sci 2021 04 9;361(4):427-435. Epub 2021 Jan 9.

Department of Pulmonary and Critical Care, Albany Medical Center, Albany, New York.

The subpleural sparing pattern is a common finding on computed tomography (CT) of the lungs. It comprises of pulmonary opacities sparing the lung peripheries, typically 1cm and less from the pleural surface. This finding has a variety of causes, including idiopathic, inflammatory, infectious, inhalational, cardiac, traumatic, and bleeding disorders. Read More

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Whole-Lung Lavage-a Narrative Review of Anesthetic Management.

J Cardiothorac Vasc Anesth 2020 Dec 5. Epub 2020 Dec 5.

Department of Anesthesiology, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.

Pulmonary alveolar proteinosis is a rare disease characterized by progressive accumulation of lipoprotein material in the alveoli as a result of a dysfunction in surfactant clearance. The whole-lung lavage procedure is considered the current standard of care and consists of the sequential lavage of both lungs for mechanical removal of residual material in the alveoli. However, a lack of standardization has resulted in different procedural techniques among institutions. Read More

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December 2020

Nocardia farcinica Brain Abscess in an Immunocompetent Host With Pulmonary Alveolar Proteinosis: A Case Report and Review of the Literature.

Cureus 2020 Nov 15;12(11):e11494. Epub 2020 Nov 15.

Internal Medicine, Medical College of Wisconsin, Wauwatosa, USA.

A 55-year-old immunocompetent male presented with new-onset seizures and acute respiratory failure requiring intubation and a stay in the medical intensive care unit. Magnetic Resonance Imaging (MRI) of the brain revealed ring-enhancing lesions, and Computed Tomography (CT) chest showed ground-glass opacity. The patient underwent craniotomy and bronchoscopy, followed by culture of the purulent aspirate from lesions in the brain and bronchoalveolar lavage (BAL). Read More

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November 2020

Serum defensin levels in patients with systemic sclerosis.

Adv Rheumatol 2020 12 22;60(1):54. Epub 2020 Dec 22.

Department of Internal Medicine, Division of Rheumatology, Gazi University Faculty of Medicine, Ankara, Turkey.

Background: Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of skin and lung as well as involvement of kidney, gastrointestinal system and heart. Aetiology and exact mechanism of disease is poorly understood. The association between antimicrobial peptides (AMPs) and other diseases such as idiopathic pulmonary fibrosis, diffuse panbronchiolitis, pulmoner alveolar proteinosis and psoriasis have been reported. Read More

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December 2020

[Mechanism of nano-indium-tin oxide inducing pulmonary alveolar proteinosis in Sprague-Dawley rats].

Zhonghua Lao Dong Wei Sheng Zhi Ye Bing Za Zhi 2020 Nov;38(11):815-818

School of Public Health, North China University of Science and Technology, Hebei Coal Mine Health and Safety Laboratory, Tangshan 063000, China; Xinxiang Medical University, Xinxiang 453003, China.

To investigate the pathogenesis of pulmonary alveolar proteinosis in rats induced by nano-indium-tin oxide exposure, and to provide a basis for further determining the limit of occupational exposure to indium and developing related protection measures. In August 2018, a total of 40 specific pathogen-free Sprague-Dawley rats, with an age of 6-8 weeks and a body weight of (200±10) g, were randomly divided into control group, low-dose group (1.2 mg/kg) , middle-dose group (3 mg/kg) , and high-dose group (6 mg/kg) , with 10 rats in each group. Read More

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November 2020

Secondary pulmonary alveolar proteinosis in GATA-2 deficiency (MonoMAC syndrome).

BMJ Case Rep 2020 Nov 30;13(11). Epub 2020 Nov 30.

Departement of Medicine, Detroit Medical Center, Detroit, Michigan, USA.

We present here a case of a 29-year-old woman with a medical history of GATA-2 deficiency, who was under treatment for pneumonia. She presented with worsening dyspnoea with cough and fever. It was initially thought she had pneumonia but she was later diagnosed with Pulmonary Alveolar Proteinosis (PAP). Read More

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November 2020