2,344 results match your criteria Alveolar Proteinosis


A case of autoimmune pulmonary alveolar proteinosis with severe respiratory failure treated with segmental lung lavage and oral statin therapy.

Respir Med Case Rep 2022 6;38:101684. Epub 2022 Jun 6.

Department of Respiratory Medicine, Steel Memorial Yawata Hospital, Fukuoka, Japan.

Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease characterized by the accumulation of alveolar surfactants due to dysfunction of granulocyte-macrophage colony-stimulating factor-dependent cholesterol clearance. Whole-lung lavage is the current standard of care for PAP, but it can lead to the exacerbation of hypoxia. A medication targeting cholesterol homeostasis is a promising therapy for refractory PAP. Read More

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A 34-Year-Old Man With Lightheadedness and Dyspnea.

Chest 2022 Jun;161(6):e371-e376

Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, University of Arkansas for Medical Sciences, Little Rock, AR. Electronic address:

Case Presentation: A 34-year-old man presented to our institution with lightheadedness and dyspnea on exertion. His medical history included chronic pancreatitis, juvenile rheumatoid arthritis (JRA), gastroesophageal reflux disease, hypertension, lumbar degenerative disc disease, seizure disorder, anterior uveitis, and multiple vertebral fractures. In addition, he was a cigarette smoker with a 10-pack-year smoking history. Read More

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Difficulties in the treatment of an infant survivor with inherited surfactant protein-B deficiency in Tunisia.

Ann Thorac Med 2022 Apr-Jun;17(2):132-135. Epub 2022 Apr 19.

Children's Department B, Bechir Hamza Children's Hospital of Tunis, University El Manar, Tunis, Tunisia.

A female-term neonate showed a severe respiratory distress syndrome (RDS) at hour 3 of life requiring her transfer to intensive care. She was intubated and started on assist-control mechanical ventilation associated with inhaled nitric oxide then high-frequency oscillation ventilation at day 12. Chest X-ray was gradually deteriorating. Read More

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Pulmonary Alveolar Proteinosis due to Familial Myelodysplastic Syndrome with resolution after stem cell transplant.

Autops Case Rep 2022 13;12:e2021382. Epub 2022 May 13.

University of Connecticut, Department of Internal Medicine, Farmington, Connecticut, USA.

Pulmonary alveolar proteinosis (PAP) is a rare lung disease with an incidence of 0.2 cases per million. PAP has multiple causes, including autoimmune, hereditary, congenital, or secondary. Read More

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A case of pulmonary alveolar proteinosis misdiagnosed as COVID-19 pneumonia.

Cir Cir 2022 ;90(3):402-405

Department of Thoracic Surgery.

A 48-year-old female patient with complaints of shortness of breath and coughing had bilateral diffuse infiltration in her chest X-ray and diffuse ground-glass opacities in her chest computed tomography. Despite her polymerase chain reaction test being negative, she was treated 10 days for coronavirus disease 2019 (COVID-19) pneumonia due to her radiological images and clinical hypoxia. As there was no improvement in her symptoms, she was administered fiberoptic bronchoscopy and she was diagnosed with pulmonary alveolar proteinosis (PAP). Read More

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An exploratory study investigating biomarkers associated with autoimmune pulmonary alveolar proteinosis (aPAP).

Sci Rep 2022 May 24;12(1):8708. Epub 2022 May 24.

Boehringer Ingelheim International GmbH, Biberach an der Riss, Germany.

Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare lung disorder involving production of autoantibodies against endogenous granulocyte-macrophage colony-stimulating factor (GM-CSF). This study aimed to identify biomarkers that could be used to monitor for aPAP, particularly in patients treated with anti-GM-CSF antibodies. This was an exploratory, prospective, observational, single-center study. Read More

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GATA2 Deficiency in Adult Life Is Characterized by Phenotypic Diversity and Delayed Diagnosis.

Front Immunol 2022 6;13:886117. Epub 2022 May 6.

Allergy and Clinical Immunology Unit, Shaare Zedek Medical Center, Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel.

The transcription factor GATA2 plays a key role in the survival and self-renewal of hematopoietic stem and progenitor cells. Autosomal dominant variants in cause a broad spectrum of heterogeneous phenotypes. Here, we present our experience with GATA2 deficiency in a retrospective multicenter analysis of computerized medical records of adult patients (age ≥18 years) treated between 2018 and 2022 at Shaare Zedek Medical Center in Jerusalem and Sheba Tel-Hashomer Medical Center in Ramat Gan, Israel. Read More

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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 04 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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Protein estimation in whole lung lavage fluid in hereditary pulmonary alveolar proteinosis due to a novel GM-CSF receptor mutation.

Pediatr Pulmonol 2022 Jul 26;57(7):1802-1805. Epub 2022 Apr 26.

Pediatric Pulmonology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

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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 Jun;205(12):e55

Hematopoietic Cell Transplant and Cell Therapy Program, Massachusetts General Hospital, Boston, Massachusetts; and Harvard Medical School, Boston, Massachusetts.

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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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Alveolar Proteinosis Secondary to , in a Patient with Transient CD4 Lymphocytopenia Due to Infection: First Case in the Literature.

Infect Dis Rep 2022 Mar 4;14(2):169-175. Epub 2022 Mar 4.

Hospital Universitario la Samaritana, Bogotá 110111, Colombia.

Transient CD4 lymphocytopenia is defined as the transitory presence of CD4+ T lymphocyte fewer than 300 cells/mm or less than 20% of T cells without HIV infection. It can occur due to multiple causes; however, it is rare for it to occur due to opportunistic infections. Few cases have been described in the literature where antimicrobial treatment normalizes the CD4 count, being more frequent in infections. Read More

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