Sep 26, 2017 alveolar proteinosis paychy distribution of geographic ground glass opacity with septal thickening crazy paving stay safe and healthy. Pulmonary alveolar proteinosis pap is a lung disease characterized by an abnormal intraalveolar accumulation of surfactantderived lipoproteinaceous. Pulmonary alveolar proteinosis pap is a rare pulmonary disease characterised by alveolar. Correlation of histology and radiology findings reveals that groundglass opacities. If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Partner therapeutics receives fda orphan drug designation. Pulmonary alveolar proteinosis pap is a syndrome of reduced gmcsfdependent, macrophagemediated surfactant clearance, dysfunctional foamy alveolar macrophages, alveolar surfactant accumulation, and hypoxemic respiratory failure for which the pathogenetic mechanism is. Recurrent alveolar proteinosis following double lung transplantation. It may result from mutations in surfactant proteins or granulocyte macrophagecolony stimulating factor gmcsf receptor genes, it may be secondary to toxic inhalation or haematological disorders, or it may be autoimmune, with antigmcsf antibodies blocking. Please practice handwashing and social distancing, and check out our resources for adapting to these times.
Furthermore, for some sarscov2 infected patients, ct image of both lungs would present crazypaving pattern, which is a typical characteristic of pulmonary alveolar proteinosis. Autoimmune pulmonary alveolar proteinosis genetic and. Statin as a novel pharmacotherapy of pulmonary alveolar. Pulmonary alveolar proteinosis pap is a rare condition of unknown origin, characterized by the accumulation of surfactantderived phospholipoproteinaceous material in alveolar spaces. The central role for granulocytemacrophage colonystimulating factor gmcsf in surfactant homeostasis has been established in mice lacking the gmcsf gene, which results in murine pulmonary alveolar proteinosis. Most cases affect adults between the ages of 2050 years. Pulmonary alveolar proteinosis pap is a rare, diffuse lung disease that is characterized by the alveolar and interstitial accumulation of a. He had a history of working with indium containing dust in a hydrogen furnace. A threedimensional volumerendering technique pulmo 3d software, vb20. In others, it occurs with lung infection or an immune problem. Murine ipscderived macrophages as a tool for disease modeling of.
It results in restrictive lung function and responds well to therapeutic lavage. Pulmonary alveolar proteinosis pap is a rare lung disease characterized by accumulation of phospholipoproteinaceous material in the alveoli. Whole lung lavage wll is the treatment of choice in symptomatic pap, but transient. Pulmonary alveolar proteinosis pulmonary alveolar proteinosis pap is a lung disease characterized by an abnormal intraalveolar accumulation of surfactantderived lipoproteina. An open lung biopsy showed the typical features of pulmonary alveolar proteinosis. Pulmonary alveolar proteinosis pap is a rare disease characterized by the accumulation of phospholipids and surfactant proteins in the lung. Ct image 1mm section shows airspace opacities in dependent portions of lungs thick arrows. Pulmonary alveolar proteinosis pap was confirmed at necropsy. The accumulated substances interfere with the normal gas exchange and expansion of the lungs, ultimately leading to difficulty breathing and a predisposition to developing lung infections. Her nasopharyngeal swab was positive for covid19 with realtime fluorescence polymerase chain reaction test.
Pulmonary alveolar proteinosis pap is a rare, diffuse lung disease that is characterized by the alveolar and interstitial accumulation of a periodic acidschiff pas stainpositive phospholipoprotein that is derived from surfactant. Pulmonary alveolar proteinosis is an interstitial lung disease that results in accumulation of phospholipoproteinaceous material and should be included as a differential diagnosis for dogs with these clinical and imaging. Pulmonary alveolar proteinosis pap is a rare disease in which a type of protein builds up in the air sacs alveoli of the lungs, making breathing difficult. Papassociated imaging findings are wellcharacterized. Surfactant is a natural substance that lowers surface tension in your lungs and allows you to breathe.
The presentation is nonspecific but typically includes dyspnea. Autoantibodies against granulocyte macrophage colony. Pulmonary alveolar proteinosis radiology reference article. Pulmonary alveolar proteinosis is a rare lung disorder characterized by an abnormal accumulation of surfactantderived lipoprotein compounds within the alveoli of the lung. Pulmonary alveolar proteinosis pap, also known as pulmonary alveolar phospholipoproteinosis, is a diffuse lung disease characterized by the accumulation of amorphous, periodic acidschiff paspositive lipoproteinaceous material in the distal air spaces. It occurs when surfactant builds up in your lungs and clogs your air sacs, or alveoli. Patients with pap generally present with subacute indolent symptoms, including progressive dyspnea and cough 3,4. In vivo imaging of pulmonary alveolar proteinosis using. The lung architecture is otherwise normal, and any associated inflammation. In pulmonary alveolar proteinosis pap, a proteinaceous material accumulates in the alveolar air spaces, causing hypoxemia, restrictive lung disease, and dramatic abnormalities on chest radiographs. Pulmonary alveolar proteinosis european respiratory society. A chest ct scan is a major tool in the diagnosis of pap. Widespread interlobular opacities arrowheads are superimposed bilaterally.
Alveolar proteinosis geographic ground glass opacity with. Pulmonary alveolar proteinosis pap is a rare condition characterized by. Rare presentation of pulmonary alveolar proteinosis. In 5 japanese patients, including 3 sibs, with paphg, cho et al. Secondary pulmonary alveolar proteinosis complicating. On imaging, pap is classically associated with the lung crazy paving pattern on ct, although it is a rare cause of this nonspecific finding. The pre to postlavage imaging interval ranged from 2 weeks to 12 months. Pulmonary alveolar proteinosis pap is a syndrome characterized by the accumulation of alveolar surfactant and dysfunction of alveolar macrophages. Pdf in vivo imaging of pulmonary alveolar proteinosis. Radiographic imaging typically reveals bilateral symmetric alveolar. Pulmonary alveolar proteinosis pap is a lung disease characterized by an abnormal intraalveolar accumulation of surfactantderived lipoproteinaceous material. Pulmonary alveolar proteinosis is a rare alveolar filling disease caused by the accumulation of phospholipoproteinaceous material in the alveoli. View full size version of pulmonary alveolar proteinosis. Pulmonary alveolar proteinosis pap is a syndrome of reduced gmcsfdependent, macrophagemediated surfactant clearance, dysfunctional foamy alveolar macrophages, alveolar surfactant accumulation, and hypoxemic respiratory failure for which the pathogenetic mechanism is unknown.
The pulmonary alveolar proteinosis pap program offers comprehensive. Pulmonary alveolar proteinosis pap is a rare lung disorder of unknown etiology characterized by alveolar filling with floccular material that stains positive using the periodic acidschiff pas method and is derived from surfactant phospholipids and protein components see the image. Alveolar proteinosis and phospholipidoses of the lungs. In addition, alveolar macrophages, which might be attacked by sarscov2, are unable to. Pulmonary alveolar proteinosis pap is a rare pulmonary disease characterised by alveolar accumulation of surfactant. Fibred confocal fluorescence microscopy fcfm is a new technology that can provide microscopic imaging of a living tissue through a 1mm flexible fibreoptic miniprobe 1, 2. Pulmonary alveolar proteinosis pap is an exceedingly rare diffuse lung disease. Groundglass opacities are present thin arrows with some sparing of anterior lungs. Pulmonary alveolar proteinosis pap is a syndrome, a set of symptoms and signs not a single disease, in which surfactant in alveoli builds up slowly. Pulmonary alveolar proteinosis pap is a rare lung disease characterized by excessive accumulation of surfactant materials within alveolar spaces. To image the alveolar structures in vivo, the miniprobe is advanced through the working channel of the bronchoscope down to the alveolar ducts and sacs. Case of the week 594 on awardwinning, radiologic teaching site for medical students and those starting out in radiology focusing on chest, gi, cardiac and musculoskeletal diseases containing over 300 powerpoint lectures, quizzes, handout notes, interactive material, most commons lists and pictorial differential diagnoses. However, the impact of spap on the prognosis of underlying mds remains unknown. Pulmonary alveolar proteinosis is a broad group of rare diseases that.
American journal of respiratory cell and molecular biology. It is the most common form 90% of the cases of pulmonary alveolar proteinosis pap. Pulmonary alveolar proteinosis pap is a rare lung condition. Pulmonary alveolar proteinosis navigation for this section. Pulmonary alveolar proteinosis pap is a rare disease of unknown etiology that is characterized by impaired surfactant metabolism, which leads to the accumulation of proteinaceous periodic acidschiffpositive material in the alveolar space 1,2. The data were acquired with a matrix of 512 512 and submillimeter slice thicknesses.
Pulmonary alveolar proteinosis pap is a rare disease of unknown origin. In vivo imaging of pulmonary alveolar proteinosis using confocal endomicroscopy article pdf available in european respiratory journal 362. Developed by renowned radiologists in each specialty, statdx provides comprehensive decision support you can rely on pulmonary alveolar proteinosis. Autoimmune pulmonary alveolar proteinosis apap is a rare autoimmune lung disorder. These conditions are the human lung disease known as pulmonary alveolar proteinosis, the lipoproteinosis that arises in the lungs of rats during acute silicosis, and the phospholipidoses induced by numerous cationic amphiphilic therapeutic agents. Siemens healthineers, forchheim, germany highlighted the distribution of pulmonary opacities, which was useful to determine the extension of the disease fig 2, movie supplement. Although not specific, highresolution computed tomography shows a characteristic diffuse groundglass attenuation with superimposed interlobular septal thickening and intralobular lines which is called crazy paving figure 1. The variant in the first family was found by wholeexome sequencing. Pulmonary alveolar proteinosis pap, also known as pulmonary alveolar. A 52 year old man presented with dyspnea, and was found to have bilateral airspace disease on chest xray. Pulmonary alveolar proteinosis pap is a rare lung disorder of unknown etiology characterized by alveolar filling with floccular material that stains positive using the periodic acidschiff pas method and is derived from surfactant phospholipids and protein components see the image below.
Histopathologic changes and sarscov2 immunostaining in. Hematological disorders are the most common underlying conditions of spap, of which 74% of cases demonstrate myelodysplastic syndrome mds. Crazy paving in pulmonary alveolar proteinosis nejm. Three pulmonary disease conditions result from the accumulation of phospholipids in the lung.
Secondary pulmonary alveolar proteinosis spap is a very rare lung disorder comprising approximately 10% of cases of acquired pap. From developing new therapies that treat and prevent disease to helping people in. Alveolar proteinosis paychy distribution of geographic. Pulmonary alveolar proteinosis cancer therapy advisor.
Wholelung lavage for pulmonary alveolar proteinosis chest. The three main types of pap are congenital, acquired, and secondary. This blocks air from entering alveoli and oxygen from passing through into the blood, which results in a feeling of breathlessness dyspnea. This investigation describes the ct features of pulmonary alveolar proteinosis in a large group of patients. There is little or no lung inflammation, and the underlying lung architecture is preserved. Levine, in goldmans cecil medicine twenty fourth edition, 2012. Complications are unusual, but of the ones that do occur, infection, particularly with nocardia asteroides, and fibrosis are the most common. Apr 8, 2019 alveolar proteinosis geographic ground glass opacity with septal thickening crazy paving stay safe and healthy. The causes of pap may be grouped into primary and secondary causes, although the most common cause is a primary autoimmune condition. Pulmonary alveolar proteinosis radiology reference. Autoimmune alveolar proteinosis is the most frequent form of pap, representing 90% of cases. Pulmonary alveolar proteinosis is an interstitial lung disease that results in accumulation of phospholipoproteinaceous material and. Treatment and prognosis of pulmonary alveolar proteinosis in adults.
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