Atypical image of pulmonary alveolar proteinosis a case. To date, few cases of secondary pulmonary alveolar proteinosis pap, as a rare complication following lung transplantation, have been reported 26 and the mechanism of pathogenesis is not fully clear. Ct features of pulmonary alveolar proteinosis objective. Autoimmune pulmonary alveolar proteinosis pap is a rare disease marked by an accumulation of surfactant proteins and lipids in the narrow gas exchange pockets of the lung, leading to respiratory failure. Pulmonary alveolar proteinosis is a pathologic entity characterized by intraalveolar surfactant accumulation. Autoimmune pulmonary alveolar proteinosis genetic and. Over ninety percent of people with pap have the autoimmune. Novel insights from an animal model aided the discovery of granulocyte macrophage colony stimulating factor gmcsf antibodies as a pathogenetic mechanism. Wholelung lavage is the most widely accepted therapy for symptomatic pulmonary alveolar proteinosis. Congenital pulmonary alveolar proteinosis is a rare form of respiratory failure. Autoimmune pulmonary alveolar proteinosis apap is a rare lung disease caused by the autoantibody against granulocytemacrophage colony stimulating factor gmcsf.
The acquired form of pulmonary alveolar proteinosis is the most common form, accounting for approximately 90% of cases. To obtain a sample, doctors use a bronchoscope to wash segments of the lung with a saltwater solution and then collect the washings bronchoalveolar lavage. Abnormal intra alveolar accumulation of floccular lipoproteinaceous material derived from surfactant phospholipids and lipoproteins first diagnosed in 1958 may be congenital2% thymic alymphoplasia idiopathic90% secondary510%. Pulmonary alveolar proteinosis msd manual consumer version. Definition pulmonary alveolar proteinosis pap is a rare disease of the lungs. Pulmonary alveolar proteinosis alveolar proteinoses pulmonary. Hematological disorders are the most common underlying conditions of spap, of which 74% of cases demonstrate myelodysplastic syndrome mds.
The combination of a systemic treatment gmcsf and a local treatment wholelung lavage augmenting the action of one another is a promising new approach. Omim entry % 610910 pulmonary alveolar proteinosis. Pulmonary fibrosis on highresolution ct of patients with. The objective of this study was to evaluate the ct findings of pap with a focus on pulmonary fibrosis secondary to pap. Does higher lavage height improve lavage effect for pulmonary. Aug 07, 2018 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. Pulmonary alveolar proteinosis radiology reference article. Pulmonary alveolar proteinosis pap is a rare and progressive lung disease in which protein builds up in the air sacs alveoli of the lungs, making it very difficult for you to breathe.
Pulmonary alveolar proteinosis pap also called alveolar proteinosis, alveolar phospholipidosis, pulmonary alveolar lipoproteinosis, pulmonary alveolar phospholipoproteinosis has been recognized for almost half a century, although descriptions of probable pap cases can. Pulmonary alveolar proteinosis in adults is an acquired primary disorder in more than 90% of. Pulmonary alveolar proteinosis nord national organization. Jan 27, 2017 autoimmune pulmonary alveolar proteinosis apap is a rare autoimmune lung disorder.
Pulmonary alveolar proteinosis pap is a rare disorder comprised of. Alveolar spaces are filled with a proteinaceous phospholipid material that represents components of pulmonary surfactant. The accumulated substances interfere with the normal gas exchange and expansion of the lungs, ultimately leading to difficulty breathing and a predisposition to. For radiologists, the term pulmonary alveolar proteinosis is. There is little or no lung inflammation, and the underlying lung architecture is preserved. Twenty pulmonary alveolar proteinosis patients were randomly allocated into two.
Levine, in goldmans cecil medicine twenty fourth edition, 2012. Is there a cure for pulmonary alveolar proteinosis. The three main causes of pap are autoimmune, congenital, and secondary. To make a definitive diagnosis of pulmonary alveolar proteinosis, doctors examine a sample of the fluid from the alveoli. However, the pathogenesis of pulmonary fibrosis in apap has never been delineated. Pulmonary alveolar proteinosis pap is a parenchymal lung disease in which the primary pathologic process affects the alveolar spaces, not the alveolar walls. Pulmonary alveolar proteinosis pap american thoracic society. Pulmonary alveolar proteinosis pap is a rare lung condition.
It occurs when surfactant builds up in your lungs and clogs your air sacs, or alveoli. Alveolar proteinosis lung and airway disorders msd. Autoantibodies against gmcsf may cause pulmonary alveolar proteinosis. Smoking is strongly associated with the condition, and in smokers, there is a recognized male predilection m. Since that time, clinicians understanding of this rare lung disease has. Mar 18, 2019 congenital pulmonary alveolar proteinosis is a rare form of respiratory failure that is present from birth. The image is so typical, that together with the characteristic bronchoalveolar lavage examination with presence of periodic acid schiff positive substance is sufficient for. Most cases affect adults between the ages of 2050 years.
Pulmonary alveolar proteinosis is most often idiopathic and occurs in otherwise healthy men and women between 30 and 50 years. Development of microscopic polyangiitisrelated pulmonary. The purpose of this paper is to describe these two cases and to discuss the possible pathogenetic relationship of pulmonary alveolar proteinosis and nocardiosis. Although pap comprises a heterogeneous group of diseases, autoimmune pap repre. However, the impact of spap on the prognosis of underlying mds remains unknown. Pulmonary alveolar proteinosis pap is a rare syndrome resulting from the accumulation of lipoproteinaceous materials in the alveoli and terminal airways due to impairment of surfactant clearance. Pulmonary alveolar proteinosis was diagnosed in an 8monthold cat with chronic tachypnea, failure to thrive and finally respiratory distress. Congenital pulmonary alveolar proteinosis genetic and rare. Pulmonary alveolar proteinosis pap is a rare syndrome resulting from the accumulation of lipoproteinaceous materials in the alveoli and terminal airways due. Pulmonary alveolar proteinosis pap is a rare syndrome characterized by the accumulation of surfactant in alveolar macrophages and alveoli. While the underlying cause of pap is not clearly understoodthe abnormal disease process that causes illness has been identified. Pulmonary alveolar proteinosis pap is an uncommon lung disease that can cause mild to severe respiratory illness. Autoimmune pulmonary alveolar proteinosis genetic and rare. The ct findings of pulmonary fibrosis in patients with pulmonary alveolar proteinosis pap are not yet well defined.
Pulmonary alveolar proteinosis is an uncommon medical condition, entailing the congestion of alveoli with material containing lipoprotein complexes, produced by type ii alveolar epithelial cells. This is the first report of pulmonary alveolar proteinosis in the feline species. Description of additional supplementary files 49k, pdf. Transfer from outside hospital in ards, complaining of 2 month history of progressive dyspnea and productive cough refractory to outpatient therapy. Pulmonary alveolar proteinosis pap is a rare disorder in which excess surfactant accumulates within pulmonary alveoli, causing cough, progressive dyspnea and respiratory insufficiency. Pulmonary alveolar proteinosis statpearls ncbi bookshelf. Pulmonary alveolar proteinosis pap is a rare disease of the lung characterized by the accumulation of surfactantderived lipoproteins within pulmonary alveolar macrophages and alveoli, resulting. Secondary pulmonary alveolar proteinosis predominant in the transplanted lung in patients with idiopathic interstitial pneumonia. Air is breathed in through the nasal passageways, travels through the trachea and bronchi to the lungs. The washings are often opaque or milky because the fluid is rich in protein and fats. Pulmonary alveolar proteinosis is an extremely rare lung disease in animals and humans. Rare secondary forms occur in patients with acute silicosis, pneumocystis jirovecii infection, hematologic cancers, or immunosuppression by drugs and in patients with significant inhalation exposures to aluminum, titanium, cement, and cellulose dusts. Pulmonary alveolar proteinosis a case report by renee m.
Inhaled gmcsf therapy for autoimmune pulmonary alveolar. Secondary pulmonary alveolar proteinosis complicating. Pulmonary alveolar proteinosis pap is a rare lung disorder characterized by an abnormal accumulation of surfactant derived lipoprotein compounds within the alveoli of the lung. The congregation of the protein in the alveolar space leads to difficulty in breathing, impaired pulmonary immunity, and susceptibility to both opportunistic and acquired pulmonary. It is the most common form 90% of the cases of pulmonary alveolar proteinosis pap. Pulmonary alveolar proteinosis, a rare lung disease, is caused by a buildup of material in the air sacs. Pulmonary alveolar proteinosis pap was first described in 1958 by samuel h. Pulmonary alveolar proteinosis definition of pulmonary. Pulmonary alveolar proteinosis an overview sciencedirect. Statin as a novel pharmacotherapy of pulmonary alveolar. It can manifest as an autoimmune, hereditary or secondary medical condition. Pulmonary alveolar proteinosis pulmonary disorders. Correction of gmcsf deficiency with exogenous gmcsf is an alternative therapy. 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 pulmonary disorder caused by a congregation of excessive lipoproteinaceous material in the alveolar spaces due to impaired surfactant metabolism. Nocardiosis and pulmonary alveolar proteinosis annals of. Omim entry % 610910 pulmonary alveolar proteinosis, acquired. Pulmonary alveolar proteinosis information mount sinai. A search of the pathology files was rewarded by the finding of another instance in which pulmonary alveolar proteinosis was associated with nocardiosis. This investigation describes the ct features of pulmonary alveolar proteinosis in a large group of patients. Pulmonary alveolar proteinosis pap also called alveolar proteinosis, alveolar phospholipidosis, pulmonary alveolar lipoproteinosis, pulmonary alveolar phospholipoproteinosis has been recognized for almost half a century, although descriptions of probable pap cases can be found in the earlier medical literature. Surprisingly, alveolar macrophages require granulocytemacrophage colonystimulating factor gmcsf to perform this function. Pulmonary alveolar proteinosis is a rare alveolar filling disease caused by the accumulation of phospholipoproteinaceous material in the alveoli. The diagnosis was based on the milky appearance of the. Proteinose alveolar pulmonar pap proteinose alveolar pulmonar e uma doenca rara na qual um liquido rico em proteinas surfactante enchem os alveolos prejudicando as trocas gasosas.
Pulmonary alveolar proteinosis pap is a rare disease characterised by. Since 1983, more than 50,000 lung transplants have been performed worldwide. Autoimmune pulmonary alveolar proteinosis apap is a rare autoimmune lung. Pulmonary alveolar proteinosis pap cleveland clinic. Secondary pulmonary alveolar proteinosis predominant in the. 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. Usually, it appears as a crazypaving pattern on highresolution computed tomography.
It happens most often in people in the age range of 30 to 60 years. 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. Dec 18, 2019 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. Pap is a lung condition that is caused by a buildup of proteins and other substances in the air sacs of the lungs, called the alveoli. Pulmonary alveolar proteinosis is a rare but potentially treatable disease, characterized by impaired surfactant metabolism that leads to accumulation in the alveoli of proteinaceous material rich in surfactant protein and its component. Scattered groundglass opacities, right greater than left, with interlobular septal thickening. Pulmonary disease certification examination blueprint purpose of the exam the exam is designed to evaluate the knowledge, diagnostic reasoning, and clinical judgment skills expected of the certified pulmonologist in the broad domain of the discipline. Secondary pulmonary alveolar proteinosis spap is a very rare lung disorder comprising approximately 10% of cases of acquired pap. It typically appears in younger patients, ages 2050, and more often in men than in women. Proteinose alveolar pulmonar wikipedia, a enciclopedia livre.
Pulmonary alveolar proteinosis is a very rare interstitial lung disease caused by abnormal intraalveolar surfactant accumulation. Unsuspected pulmonary alveolar proteinosis in a patient with. Description in this disease, also called alveolar proteinosis or phospholipidosis, gas exchange in the lungs is progressively impaired by the accumulation of phospholipids, compounds widely found in other living cells of the body. Pulmonary alveolar proteinosis pap is a syndrome characterized by the accumulation of surfactant in alveolar macrophages and alveoli resulting in respiratory insuf. Pulmonary alveolar proteinosis clinical presentation. Secondary pulmonary alveolar proteinosis in hematologic. 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. Pulmonary alveolar proteinosis is rare and usually presents in young and middleaged adults 2050 years of age 6,7.
Pdf pulmonary alveolar proteinosis is a rare but potentially treatable disease, characterized by impaired surfactant metabolism that leads to. Pulmonary alveolar proteinosis linkedin slideshare. A recent report suggested that manual ventilation during the second half of the drainage cycle might aid surfactant clearance,97 but this was a single report of an. 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. Secondary pulmonary alveolar proteinosis predominant in. This blocks air from entering alveoli and oxygen from passing through into the blood, which results in a feeling of breathlessness dyspnea. Congenital pulmonary alveolar proteinosis genetic and. Pulmonary alveolar proteinosis pulmonary disorders merck. Novel insights from an animal model aided the discovery of granulocyte macrophage colony stimulating factor gmcsf antibodies as a pathogenetic mechanism in. If you have problems viewing pdf files, download the. Pulmonary alveolar proteinosis constrictive bronchiolitis idiopathic and toxic exposure. Initiallythe goal of treatment for people with pap is symptom relief. Wed like to understand how you use our websites in order to improve them. Pulmonary alveolar proteinosis alveolar proteinoses.
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