Pulmonary alveolar proteinosis
Journal Title: Postępy Nauk Medycznych - Year 2011, Vol 24, Issue 4
Abstract
Pulmonary alveolar proteinosis (PAP) is an “orphan lung disorder” characterized by abundant accumulation of surfactant-derived phospholipids and protein components in the alveoli and distal airways. PAP was first described in 1958, and for many years the pathogenesis of the disease was unknown. Three clinically and etiologically distinct forms of PAP have been recognized (congenital, secondary and idiopathic), but more then 90% of the cases are idiopathic. Idiopathic PAP is considered to be caused by an autoantibody to the granulocyte-macrophage colony stimulating factor (GM-CSF), which neutralizes GM-CSF and therefore impairs the differentiation of alveolar macrophages. The condition has a variable clinical course from spontaneous resolution to respiratory failure and death due to disease progression or superimposed infection. The diagnosis of PAP may be strongly supported by high-resolution computed tomography (HRCT) of the chest, which reveals diffuse ground-glass opacification superimposed on septal thickening (commonly referred to as “crazy paving”) with geographical distribution.No specific therapy exists for PAP and whole lung lavage remains the standard of care. Exogenous therapy with GM-CSF may improve the lung disease in some patients with PAP but this therapy is still experimental.
Authors and Affiliations
Małgorzata Sobiecka
Keywords
Related Articles
- EP ID EP54159
- DOI -
- Views 134
- Downloads 0
Intramedullary Nailing of Femoral Shaft Fractures – Review of Locking Techniques
Surgical treatment of femoral shaft fractures has been based on locked intramedullary nailing since the introduction of its current principles by Kempf and Grosse. Locked intramedullary nailing is a low-invasive techniqu...
Recurrence AT FSGS in renal transplant
Recurrence of focal segmental glomerular sclerosis in renal transplant recipient usually manifests as massive proteinuria within hours to days after transplantation. Reccurrence of FSGS is recognized to be a disease asso...
COPD in the elderly
COPD is a major public health problem that affects a significant proportion of the elderly population. The prevalence of COPD in individuals 65 years of age and older was recently estimated to be 50% compared with 20% in...
Endovascular treatment of carotid cavernous fistulas
Carotid caveronus fistulas are incorrect connections between carotid arteries and cavernous sinus. Presence of such fistulas results with elevated blood pressure in caveronus sinus and draining vessels. Development of en...
The pancreatojejunostomy after pancreatoduodenectomy. The review of techniques including our own method of the anastomosis
Pancreaticoduodenectomy (PD) is the treatment of choice for resectable periampullary tumors. The most difficult, time-consuming, and complication-prone step in pancreaticoduodenectomy is the pancreaticojejunostomy step....