Pulmonary alveolar proteinosis acquired

Common Name(s)

Pulmonary alveolar proteinosis acquired

Acquired pulmonary alveolar proteinosis (PAP) is a rare, acquired lung disorder characterized by the accumulation of grainy material consisting mostly of protein and fat (lipoproteinaceous material) in the air sacs of the lungs (alveoli). Most cases affect adults between the ages of 20-50. The symptoms can vary greatly; some individuals may not show symptoms, while others may experience progressive difficulty breathing and shortness of breath upon exertion. Other signs and symptoms may include a dry, chronic cough; fatigue; weight loss; chest pain; and a general feeling of ill health. In rare cases, the coughing up of blood, rounding and swelling of the tips of the fingers, and cyanosis may be present. Most cases occur for no known reason, but some cases may occur secondary to environmental exposures or underlying diseases; some researchers believe it may be an autoimmune disorder. The treatment varies from case to case depending upon the age of the affected individual and severity of the disease. Acquired PAP differs from congenital PAP, an extremely rare form of PAP that occurs in some newborns.
 

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Condition Specific Organizations

Following organizations serve the condition "Pulmonary alveolar proteinosis acquired" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Pulmonary alveolar proteinosis acquired" returned 3 free, full-text research articles on human participants. First 3 results:

Whole-lung lavage: a successful treatment for restoring acinar ventilation distribution in primary acquired pulmonary alveolar proteinosis.
 

Author(s): E Vanderhelst, S Hanon, S Verbanck, D Schuermans, K Wissing, F Bonella, W Vincken

Journal: Respiration. 2012 ;84(1):70-4.

 

A 51-year-old active smoker with primary acquired pulmonary alveolar proteinosis (PAP) diagnosed by biopsy and anti-GM-CSF antibodies was treated safely with whole-lung lavage (WLL). This resulted in a rapid improvement of symptoms and arterial blood oxygenation, but not of standard ...

Last Updated: 4 Jul 2012

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Acquired pulmonary alveolar proteinosis after umbilical cord blood transplantation for acute myeloid leukemia.
 

Author(s): Akira Tomonari, Naoki Shirafuji, Tohru Iseki, Jun Ooi, Hitomi Nagayama, Atsuko Masunaga, Arinobu Tojo, Kenzaburo Tani, Shigetaka Asano

Journal: Am. J. Hematol.. 2002 Jun;70(2):154-7.

 

Pulmonary alveolar proteinosis (PAP) is a heterogeneous disease that occasionally develops with hematological malignancy. However, PAP in association with hematopoietic stem cell transplantation is quite rare. Here we present the first report of a patient who developed PAP after cord ...

Last Updated: 11 Jul 2002

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Attenuated hematopoietic response to granulocyte-macrophage colony-stimulating factor in patients with acquired pulmonary alveolar proteinosis.
 

Author(s): J F Seymour, C G Begley, U Dirksen, J J Presneill, N A Nicola, P E Moore, O D Schoch, P van Asperen, B Roth, S Burdach, A R Dunn

Journal: Blood. 1998 Oct;92(8):2657-67.

 

The pathogenesis of acquired pulmonary alveolar proteinosis (PAP), a rare lung disease characterized by excessive surfactant accumulation within the alveolar space, remains obscure. Gene-targeted mice lacking the hematopoietic growth factor granulocyte-macrophage colony-stimulating ...

Last Updated: 9 Nov 1998

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Pulmonary alveolar proteinosis acquired" returned 0 free, full-text review articles on human participants.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Pioglitazone Therapy of Autoimmune Pulmonary Alveolar Proteinosis Autoimmune Pulmonary Alveolar Proteinosis
 

Status: Recruiting

Condition Summary: Autoimmune Pulmonary Alveolar Proteinosis

 

Last Updated: 11 Oct 2017

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Evaluation of a Single Dose of Inhaled Sargramostim in Patients With Autoimmune Pulmonary Alveolar Proteinosis
 

Status: Recruiting

Condition Summary: Autoimmune Pulmonary Alveolar Proteinosis

 

Last Updated: 11 Oct 2017

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Efficacy and Safety of Inhaled Molgramostim (rhGM-CSF) in Autoimmune Pulmonary Alveolar Proteinosis (aPAP)
 

Status: Recruiting

Condition Summary: Autoimmune Pulmonary Alveolar Proteinosis

 

Last Updated: 16 Oct 2017

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