Pulmonary alveolar proteinosis in a patient with systemic. Wholelung lavage for pulmonary alveolar proteinosis chest. 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. This results in dyspnea, secondary pulmonary and systemic infection, and in. Pulmonary alveolar proteinosis pap is a syndrome characterized by progressive accumulation of pulmonary surfactant. Pulmonary alveolar proteinosis pulmonary disorders. Pulmonary alveolar proteinosis pap is an uncommon lung disease characterized by an. Pulmonary alveolar proteinosis pap cleveland clinic. Pulmonary alveolar proteinosis pap is a rare lung condition. 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. Lung alveolus proteinosis an overview sciencedirect topics. Pulmonary alveolar proteinosis pap is a diffuse lung disease that results from the accumulation of lipoproteinaceous material. Pulmonary alveolar proteinosis pap is a disease characterized by the deposition of amorphous lipoproteinaceous material in the alveoli secondary to abnormal processing of surfactant by macrophages. Pulmonary alveolar proteinosis pap is a rare lung disease characterized by accumulation of phospholipoproteinaceous.
The diagnosis of pap can be established by the classic milky effluent bronchoalveolar lavage fluid balf. Pulmonary alveolar proteinosis, commonly known as pap, is a rare lung syndrome that occurs in about seven people per million in the general population, affecting both men and women of all ethnicities. Pulmonary alveolar proteinosis pap is a rare condition which is characterised by the abnormal accumulation of proteinaceous material in the alveolar spaces, with resulting impairment in oxygen exchange across the involved alveoli. Pulmonary alveolar proteinosis and tuberculosis in a. 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. Autoimmune pulmonary alveolar proteinosis developed during. A case of pulmonary alveolar proteinosis, which has been manifested by numerous acute episodes of a diffuse pneumonic process is presented. Pulmonary alveolar proteinosis in adults outcomes research. Idiopathic pulmonary alveolar proteinosis pap is a rare disease of unknown cause characterised by the presence of massive quantities of proteinaceous eosinophilic periodic acid schiffpositive material in the alveoli 1, and by an excess of surfactant components in lung. Wholelung lavage often is performed as the first line of treatment for this disease because it is a means to wash out the proteinaceous material from the alveoli and reestablish. Pulmonary alveolar proteinosis is a disease characterized by abnormal accumula tion of surfactant in the alveoli. Acquired pulmonary alveolar proteinosis pap is a syndrome characterized by pulmonary surfactant accumulation occurring in association. Pulmonary alveolar proteinosis is most often idiopathic and occurs in otherwise healthy men and women between 30 and 50 years.
Pulmonary alveolar proteinosis in a kidney transplant. This blocks air from entering alveoli and oxygen from passing through into the blood, which results in a feeling of breathlessness dyspnea. Relationship between diffuse pulmonary fibrosis, alveolar. Abstract pulmonary alveolar proteinosis pap is a rare disease characterized by accumulation of material in the alveoli of the lungs. Pulmonary alveolar proteinosis pap is an orphan lung disease, characterized by intra alveolar accumulation of lipoproteinaceous material of unclear origin, which leads to gas exchange impairment and respiratory symptoms of variable severity. It occurs when surfactant builds up in your lungs and clogs your air sacs, or alveoli. Pulmonary alveolar proteinosis pap is a lung disorder which was first described in 1958 by rosen et al. The clinical course can be variable, ranging from spontaneous.
Pulmonary alveolar proteinosis a crazy presentation of. Thirteen patients are presented and the role of pulmonary lavage in management is illustrated. Plasmapheresis for treatment of pulmonary alveolar proteinosis. 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 treatment by wholelung lavage. Both symptoms and prognosis are variable ranging from spontaneous remission to terminal respira. Pdf alveolar proteinosis in a patient recovering from. Radiographically, pulmonary edema or consolidation is. In 1958, dr rosen was chief of pulmonary and mediastinal pathology at the armed forces institute of pathology. Granulocytemacrophage colonystimulating factor and lung. The authors speculated that the proteinaceous material was produced by the lining cells, which sloughed into. New york, new york in 1958, rosen, castleman and liebow1 reported a series of 27 cases of a pulmonary disease which was characterized by the accumulation of a proteinaceous material in the alveoli.
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. Pulmonary alveolar proteinosis is a rare disease with unknown etiology that is due to an ab. Hereditary pulmonary alveolar proteinosis caused by. Pulmonary function tests measure the lungs ability to breathe and move oxygen. 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. Clinical features frequently observed have been recurrent febrile episodes of pneumonia, paucity. Pulmonary alveolar proteinosis, a case report harold stern, md. Pulmonary alveolar proteinosis associated with a disease. Primary pulmonary alveolar proteinosis pap is a rare syndrome characterized by accumulation of surfactant in the lungs that is presumed.
The alveoli are the part of the lungs that contain air. Proteinosis alveolar pulmonar trastornos pulmonares. Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in japan yoshikazu inoue 1, bruce c. Pulmonary alveolar proteinosis pap is characterized by the accumulation of surfactant proteins within the alveolar spaces. It is there that gases between the lungs and the blood are exchanged. Pulmonary alveolar proteinosis in children uptodate. Characteristics of a large cohort of patients with. Pulmonary alveolar proteinosis is a rare disease with varied radiographic and clinical manifestations. During quiescent phases there has been only mild shortness of breath. The changes in the skin and optic fundus were considered to be caused by the polycythemia. Autoimmune pap apap caused by elevated levels of gmcsf autoantibodies gmab is very rarely associated with systemic autoimmune disease. Pulmonary alveolar proteinosis pap is a rare disease characterized by abnormal intraalveolar accumulation of surfactantlike material, 1. Moran, in modern surgical pathology second edition, 2009. Familial pulmonary alveolar proteinosis caused by mutations in.
Alveolar proteinosis was initially described in 1958 by rosen and associates 308 in 27 patients who had filling of the alveoli by a paspositive, lipidrich proteinaceous material. Pulmonary alveolar proteinosis syndrome request pdf. Pulmonary alveolar proteinosis pap is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. Pulmonary alveolar proteinosis what is pulmonary alveolar proteinosis pap. Pdf pulmonary alveolar proteinosis is a rare lung disorder, which was first reported as idiopathic condition in 1958. Several other diseases can simulate pulmonary alveolar proteinosis, including pulmonary edema, alveolar hemorrhage, hypersensitivity pneumonitis, and bronchioalveolar cell carcinoma 1, 2. If ct findings in pulmonary alveolar proteinosis are interpreted in the proper clinical context, a radiologist can often suggest the diagnosis. 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. The symptoms include fever, chest pain, cough and dyspnea. Trapnell2, ryushi tazawa3, toru arai, toshinori takada4, nobuyuki hizawa5, yasunori kasahara 6, koichiro tatsumi, masaaki hojo7, toshio ichiwata8, naohiko tanaka9, etsuro yamaguchi10, ryosuke eda11, kazunori oishi12, yoshiko tsuchihashi, chinatsu kaneko4. Six patients had associated diseases such as nocardiosis, leukemia, and silicosis. Surprisingly, alveolar macrophages require granulocytemacrophage colonystimulating factor gmcsf to perform this function.
The diagnosis was thought to be either the hammanrich syndrome, alveolar cell carcinoma, or alveolar proteinosis, with the first of these considered as most likely. Pulmonary alveolar proteinosis pap is a rare syndrome in which surfactant is accumulated in the alveoli causing respiratory symptoms typically in young to middleaged people 1. Pulmonary alveolar proteinosis pap is a rare disease characterized by the accumulation of surfactantlike lipoproteinaceous material in the distal air spaces. Abstract pulmonary alveolar proteinosis pap, characterized by deposition of intra alveolar pas positive protein and lipid rich material, is a rare cause of progressive respiratory failure. Combinedmodality therapy for pulmonary alveolar proteinosis in a remote setting. Pap is characterized by abnormal accumulation of pulmonary surfactant in the alveolar space, which impairs gas exchange leading to a severe hypoxemia.
Introduction pulmonary alveolar proteinosis is a disorder of obscure and possibly diverse causes. Pulmonary alveolar proteinosis pap is a rare lung disease characterized by. Pulmonary alveolar proteinosis pap is a rare disorder characterized by the accumulation of surfactant lipids and protein in the alveolar spaces, resulting in impairment in gas exchange. Secondary pulmonary alveolar proteinosis develops in patients who are immunosuppressed, usually with corticosteroids. Autoantibodies against gmcsf may cause pulmonary alveolar proteinosis. Combination of extracorporeal membrane oxygenation ecmo. We present a 34yearold woman diagnosed with systemic lupus erythematosus sle in 2001 based on rash malar, arthritis, leukopenia, positive antinuclear antibody. Autoimmune pulmonary alveolar proteinosis wiley online library. Secondary pulmonary alveolar proteinosis pap associated with treatment is relatively frequent1,2. Pulmonary alveolar proteinosis is an uncommon disorder marked by the abnormal accumulation of surfactant within the alveoli. Pulmonary alveolar proteinosis pap was first described in 1958 as an uncommon disorder in adults characterized by the accumulation of lipoproteinaceous material within alveoli. Discussion pap is a rare pulmonary parenchymal disease characterized by the accumulation of lipoproteinaceous material within alveoli, impeding the gas exchange. This year marks the 50th anniversary of its initial description by the eminent pathologists rosen, castleman, and liebow, 2. Pulmonary alveolar proteinosis is a rare condition that occurs when surfactant builds up in your lungs and clogs your air sacs.
Here we report a case of apap manifested during immunosuppressive treatment for polymyositis with interstitial lung disease. Pulmonary alveolar proteinosis associated with dust. Secondary pulmonary alveolar proteinosis in hematologic. Pulmonary surfactant is an insoluble proteinaceous material. Pulmonary alveolar proteinosis european respiratory society.
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