Localized Pulmonary Alveolar Proteinosis: Two Case Reports

Year 2014, Volume: 31 Issue: 3, 257 - 260, 01.07.2014

Seon Jeong Oh Ji Yung Choo Suk Keu Yeom Ki Yeol Lee Je-hyeong Kim

Abstract

Background: Pulmonary alveolar proteinosis (PAP) is a relatively
rare disease characterized by abnormal accumulation of surfactantlike
material in the alveolar spaces. The classic radiologic findings
of PAP include bilateral, symmetric, diffuse ground-glass opacity
(GGO) or consolidation. The most common computed tomography
(CT) feature of PAP is widespread GGO with thickened interlobular
septa, the so-called crazy-paving pattern, which strongly suggests the
diagnosis.
Case Report: Here, we report the cases of two young male patients
with unusual presentations of PAP. One patient showed localized PAP in the left lower lobe on CT images and the other patient presented
with unilateral PAP involving the right lower lung field and
recurrence in the same area with the same pattern as the initial manifestation.
Conclusion: In conclusion, it is important for radiologiststo be aware
of potential atypical imaging findings of PAP in order to provide a
correct diagnosis. Along these lines, PAP can present as a solitary
nodular lesion or unilateral focal lesion, and can recur in the same
pattern and location

Keywords

CT, localized, pulmonary alveolar proteinosis

References

• 1. Rosen SH, Castleman B, Liebow AA. Pulmonary alveolar proteinosis. N Engl J Med 1958;258:1123-42. [CrossRef]
• 2. Frazier AA, Franks TJ, Cooke EO, Mohammed TL, Pugatch RD, Galvin JR. From the archives of the AFIP: Pulmonary alveolar proteinosis. Radiographics 2008;28:883-99. [CrossRef]
• 3. David MH, Alexander AB, Heber M, Theresa CM, Nestor LM, Jacques R. Fleischner Society: Glossary of terms for thoracic imaging. Radiology 2008;246:697−722. [CrossRef]
• 4. Presneill JJ, Nakata K, Inoue Y, Seymour JF. Pulmonary alveolar proteinosis. Clin Chest Med 2004;25:593-613. [CrossRef]
• 5. Singh G, Katyal SL, Bedrossian CW, Rogers RM. Pulmonary alveolar proteinosis. Staining for surfactant apoprotein in alveolar proteinosis and in conditions simulating it. Chest 1983;83:82-6. [CrossRef]
• 6. Bruce CT, Jeffrey AW, Koh Nakata. Pulmonary Alveolar Proteinosis. N Engl J Med 2003;349:2527-39. [CrossRef]
• 7. Dirksen U, Nishinakamura R, Groneck P, Hattenhorst U, Nogee L, Murray R, et al. Human pulmonary alveolar proteinosis associated with a defect in GM-CSF/IL-3/IL-5 receptor common beta chain expression. J Clin Invest 1997;100:2211-7. [CrossRef]
• 8. HolbertJM,Costello P,LiW,HoffmanRM,RogersRM.CTfeatures of pulmonary alveolar proteinosis.AJR Am J Roentgenol 2001;176:1287-94.[CrossRef]
• 9. Teja K, Cooper PH, Squires JE, Schnatterly PT. Pulmonary alveolar proteinosis in four siblings. N Engl J Med 1981;305:1390-2. [CrossRef]
• 10. Mildenberger E, deMello DE, Lin Z, Kössel H, Hoehn T, Versmold HT. Focal congenital alveolar proteinosis associated with abnormal surfactant protein B messenger RNA. Chest 2001;119:645-7. [CrossRef]
• 11. Boussoffara L, Boudawara N, Knani J, Zantour B, Khouaja I. Protéinose alvéolaire atypique. Rev Pneumol Clin 2011;67:158-62. [CrossRef]
• 12. Prakash UB, Barham SS, Carpenter HA, Dines DE, Marsh HM. Pulmonary alveolar phospholipoproteinosis: Experience with 34 cases and a review. Mayo Clin Proc 1987;62:499-518. [CrossRef]