Pulmonary alveolar proteinosis, often shortened to PAP, is a rare lung disorder in which lipoprotein material derived from surfactant builds up inside the alveoli, the small air sacs where gas exchange takes place. The accumulated material interferes with normal gas exchange and with the expansion of the lungs, so people affected become short of breath and are more prone to lung infections. Its causes are grouped as primary, meaning autoimmune or hereditary forms, as secondary, arising in the course of several other diseases, and as congenital, which covers a range of usually genetic disorders. In any single patient the most common cause is a primary autoimmune condition. The record here describes the condition as medicine understands it and is not medical advice.
Facts
Classification
ICD-10 ChapterX: Diseases of the Respiratory System 1 Causative Agent
Causative Agent (category) First Described Sources
1. Pulmonary alveolar proteinosis (Wikipedia)
Lead, first sentence
is a rare lung disorder characterized by an abnormal accumulation of surfactant-derived lipoprotein compounds within the alveoli of the lung
Lead, causes
although the most common cause is a primary autoimmune condition in an individual
lead sentence 1
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.
History section
PAP was first described in 1958 by the physicians Samuel Rosen, Benjamin Castleman, and Averill Liebow.
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