scholarly journals Rare Presentation of Pulmonary Alveolar Proteinosis Causing Acute Respiratory Failure

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Ryan R. Kroll ◽  
Sameer Kumar ◽  
Ronald F. Grossman ◽  
Charles Price ◽  
John R. Srigley
Keyword(s):  
Respiratory Failure ◽  
Pulmonary Alveolar Proteinosis ◽  
Periodic Acid ◽  
Rare Condition ◽  
Lung Lavage ◽  
Hypoxemic Respiratory Failure ◽  
Rare Presentation ◽  
Periodic Acid Schiff ◽  
Novel Treatments ◽  
Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare condition characterized by dysfunctional alveolar macrophages, which ineffectively clear surfactant and typically cause mild hypoxemia. Characteristic Computed Tomography findings are septal reticulations superimposed on ground-glass opacities in a crazy paving pattern, with a clear juxtaposition between affected and unaffected parenchyma. While traditionally PAP was diagnosed via biopsy, bronchoalveolar lavage (BAL) is usually sufficient; the fluid appears milky, and on microscopic examination there are foamy macrophages with eosinophilic granules and extracellular hyaline material that is Periodic Acid-Schiff positive. Standard therapy is whole lung lavage (WLL), although novel treatments are under development. The case presented is a 55-year-old woman with six months of progressive dyspnea, who developed hypoxemic respiratory failure requiring mechanical ventilation; she had typical findings of PAP on imaging and BAL. WLL was ultimately successful in restoring adequate oxygenation. Respiratory failure of this magnitude is a rare finding in PAP.

Pulmonary Alveolar Proteinosis as a Terminal Complication in a Case of Myelodysplastic Syndrome with Idic(20q-).

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4864-4864
Author(s):  
Yongquan Xue ◽  
Yongsheng Han ◽  
Tianyu Li ◽  
Suning Chen ◽  
Jun Zhang ◽  
...  
Keyword(s):  
Pulmonary Alveolar Proteinosis ◽  
Conflicts Of Interest ◽  
Periodic Acid ◽  
Chromosome Analysis ◽  
Banding Technique ◽  
Chromosomal Marker ◽  
Fish Analysis ◽  
Periodic Acid Schiff ◽  
Computed Tomographic ◽  
Alveolar Proteinosis

Abstract Abstract 4864 Secondary pulmonary alveolar proteinosis (PAP) is a rare lung disease which was reported in 13 cases of myelodysplastic syndromes (MDS). A dicentric isochromosome of deleted chromosome 20q i.e., idic(20q-) is a newly recognized rare but recurrent cytogenetic anomaly which has been described in thirty cases of MDS. Recently we encounted an interesting MDS patient with idic(20q-) and secondary PAP. At presentation she was a forty-year-old women manifesting pancytopenia and dysplasia involving two cell lineages which were compatible with refractory cytopenia with multilineage desplasia. Chromosome analysis using R-banding technique showed a karyotype of 46,XX,-20,+a small metacentric chromosomal marker. FISH analysis proved this marker to be a dicentric isochromosome of a deleted 20q. Three yaeas after presentation her disease was complicated by secondary PAP which was confirmed by chest computed tomographic scan and the thoracoscopic lung biopsy which revealed the characteristic periodic acid Schiff stain-positive materials filling the alveoli. After prednisolone and intibiotics freatment, her symptoms had temporary improvement. At last she died of respiratory failure with a survival of 45 months. As we know, no MDS patients with idic(20q-) and secondary PAP have been reported in the literature. Thus, this patient is not only the thirty-first MDS case with idic(20q-), but also the first MDS case with idic(20q-) and secondary PAP. Disclosures No relevant conflicts of interest to declare.

scholarly journals Alveolar proteinosis – disease with unpredictable evolution (series of clinical cases)

Pneumologia ◽  
2020 ◽  
Vol 68 (4) ◽  
pp. 183-193
Author(s):  
Anca Macri ◽  
Radu Stoica
Keyword(s):  
Periodic Acid ◽  
Spontaneous Remission ◽  
Lung Lavage ◽  
Periodic Acid Schiff ◽  
Whole Lung Lavage ◽  
Alveolar Surfactant ◽  
Alveolar Proteinosis ◽  
Circulating Autoantibodies ◽  
Turn Over ◽  
Stimulating Factor

AbstractPulmonary alveolar proteinosis (PAP) is a rare disease, certainly underdiagnosed, characterised by the intra-alveolar accumulation of a milky fluid rich in phospholipids and lipoproteins derived from alveolar surfactant, positive in periodic acid-Schiff staining. The alveolar macrophage plays a major role in the pathogenesis of PAP, and its role in the turn-over of alveolar surfactant is being altered by various mechanisms.More than 90% of cases of PAP are primary autoimmune, characterised by the presence in serum of circulating autoantibodies against granulocyte-macrophages colony-stimulating factor. Other causes of PAP are genetic, secondary to other diseases or to exposure to different agents.The evolution of the disease is unpredictable, from spontaneous remission to progression despite treatment towards pulmonary fibrosis and chronic severe respiratory failure. The gold standard of therapy is the whole lung lavage, other treatments are being still in evaluation.The article presents a few cases that illustrate different patterns in the evolution of PAP.

scholarly journals Secondary pulmonary alveolar proteinosis during corticosteroid therapy for organising pneumonia associated with myelodysplastic syndrome

2019 ◽  
Vol 12 (9) ◽  
pp. e231055 ◽  
Author(s):  
Daiki Inoue ◽  
Satoshi Marumo ◽  
Haruyuki Ishii ◽  
Motonari Fukui
Keyword(s):  
Myelodysplastic Syndrome ◽  
Corticosteroid Therapy ◽  
Amorphous Materials ◽  
Pulmonary Alveolar Proteinosis ◽  
Periodic Acid ◽  
Radiological Findings ◽  
Periodic Acid Schiff ◽  
Gm Csf ◽  
Alveolar Proteinosis ◽  
Secondary Pulmonary Alveolar Proteinosis

Myelodysplastic syndrome (MDS) is frequently complicated by pulmonary disease. Here, we describe secondary pulmonary alveolar proteinosis (sPAP) that developed during corticosteroid therapy for organising pneumonia (OP) associated with MDS. A 75-year-old woman with MDS complained of cough for 2 weeks. Chest CT showed bilateral patchy consolidations with reversed halo sign. Bronchoalveolar lavage (BAL) examination showed remarkably increased cell density with an increased lymphocyte proportion. Abnormal radiological findings improved rapidly on administration of systemic corticosteroid under the diagnosis of OP; however, they relapsed a few weeks later. Transbronchial lung biopsy showed periodic acid-Schiff stain-positive amorphous materials. Autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) in serum and BAL fluid (BALF) were both negative, while GM-CSF level in BALF was elevated. The patient was diagnosed with sPAP. When chest radiological findings show exacerbation during corticosteroid therapy for OP in a patient with MDS, physicians should consider sPAP complication as a differential diagnosis.

scholarly journals Avian pulmonary proteinosis: six cases and a review of the literature

2019 ◽  
Vol 31 (3) ◽  
pp. 378-381 ◽  
Author(s):  
Dayna A. Goldsmith ◽  
Aslı Mete ◽  
Joseph B. Pesavento ◽  
John M. Adaska
Keyword(s):  
Pulmonary Surfactant ◽  
Pulmonary Alveolar Proteinosis ◽  
Incidental Finding ◽  
Periodic Acid ◽  
Review Of The Literature ◽  
Periodic Acid Schiff ◽  
Transmission Electron ◽  
Alveolar Proteinosis ◽  
Lamellar Material ◽  
Electron Lucent

Pulmonary alveolar proteinosis (PAP) is a disease of surfactant clearance in which functional abnormalities in alveolar macrophages lead to accumulation of surfactant within alveoli in mammals. Histologic examination of 6 avian autopsies, including 4 chickens, a turkey, and a cockatiel, revealed accumulation of hypereosinophilic densely arrayed lamellar material in the lungs that was magenta by periodic acid–Schiff stain and diastase resistant. Transmission electron microscopy of the proteinaceous material in 2 cases demonstrated alternating electron-dense and electron-lucent lamellae that formed whorls and had a regular periodicity of 6–14 nm, consistent with pulmonary surfactant. Given the anatomic differences between avian and mammalian lungs, we designated the presented condition “pulmonary proteinosis,” which can be observed as both an incidental finding or, when severe, may be a contributing factor to death through respiratory failure.

scholarly journals Pulmonary alveolar proteinosis: a complete response to GM-CSF therapy

Thorax ◽  
2001 ◽  
Vol 56 (8) ◽  
pp. 664-665
Author(s):  
R M Barraclough ◽  
A J Gillies
Keyword(s):  
Pulmonary Alveolar Proteinosis ◽  
Rare Condition ◽  
Complete Response ◽  
Lung Lavage ◽  
Whole Lung Lavage ◽  
Gm Csf ◽  
Macrophage Colony Stimulating Factor ◽  
Alveolar Proteinosis ◽  
Macrophage Colony ◽  
New Treatment

Pulmonary alveolar proteinosis is a rare condition traditionally requiring treatment with whole lung lavage. The case is presented of a young man who obtained complete remission following treatment with granulocyte-macrophage colony stimulating factor, a new treatment option.

scholarly journals Lobar flexible fiberoptic lung lavage: therapeutic benefit in severe respiratory failure in pulmonary alveolar proteinosis and influenza A H1N1 pneumonia

2011 ◽  
Vol 1 (3) ◽  
pp. 53 ◽  
Author(s):  
Antonello Nicolini ◽  
Cornelius Barlascini
Keyword(s):  
Respiratory Failure ◽  
Influenza A ◽  
Pulmonary Alveolar Proteinosis ◽  
Therapeutic Benefit ◽  
Lung Lavage ◽  
Safe Procedure ◽  
Severe Respiratory Failure ◽  
Influenza A H1n1 ◽  
Virus Pneumonia ◽  
Alveolar Proteinosis

Lobar fiberoptic lung lavage is a well-known procedure used in primary pulmonary alveolar proteinosis (PAP); the use of this procedure has increased in the recent years. This procedure has also been used in other pulmonary diseases such as desquamative interstitial pneumonia with good results. We describe a case of extremely severe respiratory failure due to concurrence of PAP and Influenza A H1N1 virus pneumonia which resolved with the help of this procedure. The patient, a 41- year-old woman, needed less mechanical ventilation after undergoing lobar fiberoptic bronchoscopic lavage. Moreover, a rapid and progressive improvement in the computed tomography of the lungs was observed. Flexibile fiberoptic bronchoscopic lobar lavage is a simple, safe procedure used not only in milder disease, but also in particular severe cases in which the physiological derangement of whole lung lavage would not be tolerated by patient or when extra-corporeal membrane oxygenation is not available.

scholarly journals Alveolar proteinosis in Behçet’s disease

2010 ◽  
Vol 5 ◽  
Author(s):  
Cuneyt Tetikkurt ◽  
Seza Tetikkurt ◽  
Imran Ozdemir ◽  
Cigdem Zuhur ◽  
Nihal Bayar
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Pulmonary Alveolar Proteinosis ◽  
Behçet's Disease ◽  
Periodic Acid ◽  
Spontaneous Remission ◽  
Periodic Acid Schiff ◽  
Behcet's Disease ◽  
Gm Csf ◽  
Alveolar Proteinosis

A 51-year-old man with Behçet’s disease complained of fever, dry cough and dyspnea during exertion. Chest CT showed ground glass opacities with interstitial septal thickening in both lungs. Bronchoalveolar lavage (BAL) revealed amorphous and lipoproteinaceous material that was periodic acid-Schiff (PAS) stain positive. Transbronchial biopsy specimen demonstrated PAS positive alveolar eosinophilic material consistent with pulmonary alveolar proteinosis. Serum anti-granulocyte- macrophage colony stimulating factor (GM-CSF) antibody was negative. Recent studies have reported anti-GMCSF not present in the the serum of patients with secondary pulmonary alveolar proteinosis (PAP) but they have not reported so in patients with idiopathic PAP. We report a case of alveolar proteinosis in the setting of Behçet’s disease with spontaneous remission.

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