Pulmonary Alveolar Proteinosis Syndrome

2020 ◽  
Vol 41 (02) ◽  
pp. 288-298 ◽  
Author(s):  
Alan Kelly ◽  
Cormac McCarthy
Keyword(s):  
Alveolar Macrophage ◽  
Immune Modulation ◽  
Pulmonary Alveolar Proteinosis ◽  
Systemic Infection ◽  
Lung Lavage ◽  
Response To Therapy ◽  
Cholesterol Homeostasis ◽  
Current Standard ◽  
Gm Csf ◽  
Alveolar Proteinosis

AbstractPulmonary alveolar proteinosis (PAP) is a syndrome characterized by progressive accumulation of pulmonary surfactant. This results in dyspnea, secondary pulmonary and systemic infection, and in some cases respiratory failure. PAP syndrome occurs in distinct diseases, classified according to pathogenetic mechanism; these include primary PAP (due to disruption of granulocyte-macrophage colony-stimulating factor [GM-CSF] signaling), secondary PAP (due to reduction in alveolar macrophage numbers/functions), and congenital PAP (due to disruption of surfactant production). In primary PAP, the most common cause is autoimmune PAP, which accounts for over 90% of all PAP syndrome. The pathogenesis is driven by reduced GM-CSF-signaling causing abnormal alveolar macrophage function which subsequently results in impaired alveolar surfactant clearance. Autoimmune PAP can be accurately diagnosed by serum GM-CSF autoantibody levels and there now exist other diagnostic tests for rare causes of PAP syndrome. The current standard treatment is whole lung lavage; however, there is emerging evidence to support the use of novel therapeutic approaches, including inhaled GM-CSF, immune modulation, gene and cell therapy, and targeting macrophage cholesterol homeostasis. Furthermore, several innovative approaches to monitor disease severity and response to therapy have recently been developed.

scholarly journals Pulmonary alveolar proteinosis: diagnostic and therapeutic challenges

2012 ◽  
Vol 7 ◽  
Author(s):  
Ilaria Campo ◽  
Zamir Kadija ◽  
Francesca Mariani ◽  
Elena Paracchini ◽  
Giuseppe Rodi ◽  
...  
Keyword(s):  
Pulmonary Alveolar Proteinosis ◽  
Gene Mutations ◽  
Spontaneous Remission ◽  
Lung Lavage ◽  
Current Standard ◽  
Gm Csf ◽  
Genes Encoding ◽  
Current Standard Treatment ◽  
Alveolar Proteinosis ◽  
Macrophage Colony

Pulmonary Alveolar Proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals and in distinct clinical forms: autoimmune (previously referred to as the idiopathic form, represents the vast majority of PAP cases, and is associated with Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) auto-antibodies; GMAbs), secondary (is a consequence of underlying disorders), congenital (caused by mutations in the genes encoding for the GM-CSF receptor), and PAP-like syndromes (disorders associated with surfactant gene mutations). The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole lung lavage (WLL) is the current standard treatment for PAP patients and although it is effective in the majority of cases, disease persistence is not an unusual outcome, even if disease is well controlled by WLL. In this paper we review the therapeutic strategies which have been proposed for the treatment of PAP patients and the progress which has been made in the understanding of the disease pathogenesis.

scholarly journals Congenital Pulmonary Alveolar Proteinosis

2013 ◽  
Vol 2013 ◽  
pp. 1-2 ◽  
Author(s):  
Saber Hammami ◽  
Khaled Harrathi ◽  
Khaled Lajmi ◽  
Samir Hadded ◽  
Chebil Ben Meriem ◽  
...  
Keyword(s):  
Pulmonary Surfactant ◽  
Standard Treatment ◽  
Pulmonary Alveolar Proteinosis ◽  
Late Onset ◽  
Spontaneous Remission ◽  
Lung Lavage ◽  
Current Standard ◽  
Whole Lung Lavage ◽  
Current Standard Treatment ◽  
Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals. Two clinically different pediatric types have been defined as congenital PAP which is fatal and a late-onset PAP which is similar to the adult form and less severe. The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole-lung lavage is the current standard treatment for PAP patients. We report a new congenital case of PAP.

scholarly journals PULMONARY ALVEOLAR PROTEINOSIS - A CASE REPORT AND REVIEW OF LITERATURE

2020 ◽  
pp. 1-3
Author(s):  
Abhilash Narvenkar ◽  
Uday C. Kakodkar* ◽  
Chetan Kerkar ◽  
John Muchahary
Keyword(s):  
Case Report ◽  
Lower Respiratory Tract ◽  
Pulmonary Alveolar Proteinosis ◽  
Lung Lavage ◽  
Review Of Literature ◽  
Whole Lung Lavage ◽  
Gm Csf ◽  
Radiological Features ◽  
History Of ◽  
Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare disorder characterised by accumulation of lipoproteinaceous material in the alveolar air spaces. Diagnosis depends on histopathological and radiological features. Treatment includes whole lung lavage (WLL) and GM-CSF therapy. We present a case report of primary idiopathic PAP treated with bilateral whole lung lavage. A 50 year old female presented with history of progressive breathlessness and recurrent lower respiratory tract infection. There were bilateral basal ne crepitations on auscultation and she was maintaining saturation of 70% at room air. Serial chest radiographs showed persistent bilateral alveolar opacities. HRCT thorax showed crazy paving pattern involving both lungs. BAL uid showed lipoproteinaceous material which was PAS stain positive. Patient was subsequently treated with bilateral WLLfollowing which there was radiological and clinical improvement

scholarly journals Pulmonary Alveolar Proteinosis in Setting of Inhaled Toxin Exposure and Chronic Substance Abuse

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Meirui Li ◽  
Salem Alowami ◽  
Miranda Schell ◽  
Clive Davis ◽  
Asghar Naqvi
Keyword(s):  
Risk Factors ◽  
Alveolar Macrophage ◽  
Pulmonary Alveolar Proteinosis ◽  
Anabolic Steroids ◽  
Diagnostic Process ◽  
Gm Csf ◽  
Macrophage Dysfunction ◽  
Macrophage Colony Stimulating Factor ◽  
Alveolar Proteinosis ◽  
Macrophage Colony

Pulmonary alveolar proteinosis (PAP) is a rare lung disorder in which defects in alveolar macrophage maturation or function lead to the accumulation of proteinaceous surfactant in alveolar space, resulting in impaired gas exchange and hypoxemia. PAP is categorized into three types: hereditary, autoimmune, and secondary. We report a case of secondary PAP in a 47-year-old man, whose risk factors include occupational exposure to inhaled toxins, especially aluminum dust, the use of anabolic steroids, and alcohol abuse, which in mice leads to alveolar macrophage dysfunction through a zinc-dependent mechanism that inhibits granulocyte macrophage-colony stimulating factor (GM-CSF) receptor signalling. Although the rarity and vague clinical presentation of PAP can pose diagnostic challenges, clinician awareness of PAP risk factors may facilitate the diagnostic process and lead to more prompt treatment.

scholarly journals Pulmonary alveolar proteinosis: from classification to therapy

Breathe ◽  
2020 ◽  
Vol 16 (2) ◽  
pp. 200018
Author(s):  
Elena Salvaterra ◽  
Ilaria Campo
Keyword(s):  
Disease Severity ◽  
Pulmonary Alveolar Proteinosis ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Premature Death ◽  
Lung Lavage ◽  
List Type ◽  
Gm Csf ◽  
Surfactant Production ◽  
Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare respiratory syndrome characterised by the accumulation of surfactant lipoproteins within the alveoli. According to various pathogenetic mechanisms and aetiologies, PAP is classified as primary, secondary or congenital. Primary PAP is led by a granulocyte–macrophage colony-stimulating factor (GM-CSF) signalling disruption; the autoimmune form is driven by the presence of anti GM-CSF autoantibodies and represents 90% of all the PAP cases; and the hereditary form is the result of mutations in genes encoding GM-CSF receptor. Secondary PAP is associated with various diseases causing a reduction in function and/or number of alveolar macrophages. Congenital PAP emerges as a consequence of corrupted surfactant production, due to mutations in surfactant proteins or lipid transporter, or mutations affecting lung development. The clinical manifestations are various, ranging from insidious onset to acute or progressive respiratory failure, including premature death within the first days of life in neonates with congenital surfactant production disorders. The diagnostic workup includes clinical and radiological assessment (respiratory function test, high-resolution chest computed tomography), laboratory tests (anti-GM-CSF autoantibodies dosage, GM-CSF serum level and GM-CSF signalling test), and genetic tests. Whole-lung lavage is the current gold standard of care of PAP; however, the therapeutic approach depends on the pathogenic form and disease severity, including GM-CSF augmentation strategies in autoimmune PAP and other promising new treatments.Educational aimsTo update knowledge about a rare respiratory syndrome, pulmonary alveolar proteinosis, in order to promote early diagnosis and correct management.To highlight recent treatment options based on pathogenesis and disease severity.

A combination therapy of whole lung lavage and GM-CSF inhalation in pulmonary alveolar proteinosis

2008 ◽  
Vol 43 (8) ◽  
pp. 828-830 ◽  
Author(s):  
Hajime Yamamoto ◽  
Etsuro Yamaguchi ◽  
Hiroatsu Agata ◽  
Nobuhisa Kandatsu ◽  
Toru Komatsu ◽  
...  
Keyword(s):  
Combination Therapy ◽  
Pulmonary Alveolar Proteinosis ◽  
Lung Lavage ◽  
Whole Lung Lavage ◽  
Gm Csf ◽  
Alveolar Proteinosis
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