Pulmonary alveolar proteinosis

Widespread airspace consolidation and ground glass opacities 

Abbreviations. HRCT, high-resolution computed tomography PAP, puhnonaiy alveolar proteinosis. 

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Pulmonary alveolar proteinosis Surfactant protein C (SP-C) Local... 

Pulmonary alveolar proteinosis Lung surfactant protein C Local... 

In pulmonary alveolar proteinosis, the alveoli of the lung fill with an excess of a glycoprotein. The reaction may occur in response to dust or other irritants. 

A patient with acute myeloid leukemia developed pulmonary alveolar proteinosis during the period of leukopenia following an unrelated umbilical cord blood transplantation (49). 

Tomonari A, Shirafuji N, Iseki T, Ooi J, Nagayama H, Masunaga A, Tojo A, Tani K, Asano S. Acquired pulmonary alveolar proteinosis after umbilical cord blood transplantation for acute myeloid leukemia. Am J Hematol 2002 70(2) 154-7. 

Immunomodulatory effects of isotretinoin in the treatment of facial acne (40 mg/day for 4 weeks) were blamed for a recurrence of pulmonary alveolar proteinosis in a 16-year-old girl, in whom it had been in spontaneous remission for 2 years (107). 

Khurshid 1, Seymour IF, Nakata K, Downie GH. Recurrent manifestations of idiopathic pulmonary alveolar proteinosis after isotretinoin (Accutane) treatment. Chest 2001 120(Suppl) 335. 

Lung surfactant protein C Res 9-22 (WWVVVLVVVVIV) (deMeUo and Lin 2001 Hosia et al. 2002 Schmitt-Bemard et al. 2000 Seymour and PresneiU 2002 Trapnell et al. 2003) Pulmonary alveolar proteinosis... 

Seymour JF, Presneill JJ (2002) Pulmonary alveolar proteinosis progress in the first 44 years. Am J Respir Crit Care Med 166 215-235... 

Tracz SM, Abedini A, Driscoll M, Raleigh DP (2004) Role of aromatic interactions in amyloid formation by peptides derived from human Amylin. Biochemistry 43 15901-15908 Trapnell BC, Whitsett JA, Nakata K (2003) Pulmonary alveolar proteinosis. N Engl J Med 349 2527-2539 Tsuii S (1999) Dentatombral-pallidoluysian atrophy (DRPLA) clinical features and molecular genetics. Adv... 

Respiratory Pulmonary alveolar proteinosis is a rare complication of treatment with sirolimus but has been reported in a 49-year-old female kidney transplant recipient [126 ]. A biopsy showed intact lung parenchyma and macrophages among alveoli filled with PAS-positive eosinophilic material which also stained positive for... 

Kadikoy H, Paolini M, Achkar K, Suki W, Gaber AO, Anwar N, Jeroudi A, Barrios R, Abdellatif A. Pulmonary alveolar proteinosis in a kidney transplant a rare complication of sirolimus. Nephrol Dial Transplant 2010 25(8) 2795-8. 

Respiratory Bronchiolitis-Associated Interstitial Lung Disease 341 Desquamative Interstitial Pneumonia 341 Lymphoid Interstitial Pneumonia 342 Acute Interstitial Pneumonia 342 Sarcoidosis 343 Miscellaneous Rare Forms of Interstitial Lung Disease of Unknown Etiology 345 Pulmonary Langerhans Cell Histiocytosis 345 Lymphangioleiomyomatosis 345 Eosinophilic Pneumonia 346 Pulmonary Alveolar Proteinosis 348 Pulmonary Microlithiasis 348... 

Ben-Dov I, Kishinevski Y etal. (1999) Pulmonary alveolar proteinosis in Israel ethnic clustering. Isr Med Assoc J 1 75-78... 

Figure 14 HRCT image demonstrating thickened interlobular septa against a background of ground glass opacities or a crazy paving pattern in a patient with PAP. Abbreviations HRCT, high-resolution computed tomography PAP, pulmonary alveolar proteinosis.

Shah PL, Hansell D, Lawson PR, et al. Pulmonary alveolar proteinosis clinical aspects and current concepts on pathogenesis. Thorax 2000 55(l) 67-77. 

Godwin JD, Muller NL, Takasugi JE. Pulmonary alveolar proteinosis CT findings. Radiology 1988 169(3) 609-613. 

Murch CR, Carr DH. Computed tomography appearances of pulmonary alveolar proteinosis. Clin Radiol 1989 40(3) 240-243. 

Lee KN, Levin DL, Webb WR, et al. Pulmonary alveolar proteinosis high-resolution CT, chest radiographic, and functional correlations. Chest 1997 lll(4) 989-995. 

Newell JD, Underwood GH Jr., Russo DJ, et al. Computed tomographic appearance of pulmonary alveolar proteinosis in adults. J Comput Tomogr 1984 8(l) 21-29. 

Acute pulmonary edema Diffuse alveolar hemorrhage Periengraftment respiratory distress syndrome Bronchiolitis obliterans syndrome Bronchiolitis obliterans organizing pneumonia Idiopathic pulmonary syndrome Delayed pulmonary toxicity syndrome Pulmonary cytolytic thrombi Pulmonary veno-occlusive disease Progressive pulmonary fibrosis Pulmonary hypertension Hepatopuhnonary S3mdrome Pulmonary alveolar proteinosis Eosinophilic pneumonia... 

Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by the accumulation of surfactant-like material in the alveolar spaces, with resultant impairment in gas exchange (1-8). Since the sentinel description of PAP by Rosen and colleagues in 1958 (9), fewer than 500 cases have been reported in the literature (5). 

Table 1 Salient Features of Pulmonary Alveolar Proteinosis (PAP)...

Figure 5 (A) PA chest radiograph from a patient with pulmonary alveolar proteinosis (PAP) shows extensive alveolar opacification involving all lung fields. The patient was severely hypoxemic. (B) PA chest radiograph from the same patient 4 weeks following bilateral whole lung lavage shows marked, albeit partial, clearing.

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