Abstract
Pulmonary alveolar proteinosis (PAP), lymphangioleyomiomatosis (LAM) and amyloidosis are three unrelated diseases of rare occurrence, with characteristic histopathological features. A pattern of alveolar filling with granular pink material accumulation is characteristic of PAP. This material can be recognized in lung biopsies, but also in bronchial lavage fluid. PAP is clinically related to the abnormal clearance of alveolar surfactant, most commonly due to the disruption of the granulocyte macrophagecolony stimulating factor signalling pathway. Whole lung lavage is the treatment of choice. LAM is characterized by cystic lung degeneration and interstitial proliferation of LAM cells, which express both melanocyte and smooth muscle cell markers, has a typical cystic pattern on CT scan, can be associated clinically with abdominal angiomyolipomas and limphangioleiomyomas, and occurs in female patients, either in isolation or as a manifestation of tuberous sclerosis. Sex hormone manipulation is the therapy of choice in this otherwise progressive disease. Diffuse interstitial or perivascular amyloid deposits in the lung can form in the context of systemic amyloidosis, usually associated with myeloma or monoclonal gammopathy, and less often with chronic inflammatory diseases. Nodular amyloid deposits, in contrast, are not associated with systemic lung disease, and present instrumentally as a coin lesion or lung mass. Isolated tracheobronchial amyloidosis is another rare form that is not related to systemic disease. In all conditions, amyloid has a typical waxy, amorphous, slightly eosinophilic stain, stains red with Congo red and presents a characteristic apple-green birefringence under polarized light, which is essential for diagnosis.
| Original language | English |
|---|---|
| Pages (from-to) | 547-556 |
| Number of pages | 10 |
| Journal | Pathologica |
| Volume | 102 |
| Issue number | 6 |
| Publication status | Published - Dec 2010 |
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Keywords
- Lymphangioleiomyomatosis
- Polmonary amyloidosis
- Pulmonary alveolar proteinosis
ASJC Scopus subject areas
- Pathology and Forensic Medicine
Cite this
Rare diffuse diseases of the lung : Pulmonary alveolar proteinosis, lymphangioleiomyomatosis, amyloidosis. / Morbini, P.; Guddo, F.; Contini, P.; Luisetti, M.; Schiavina, M.; Zompatori, M.
In: Pathologica, Vol. 102, No. 6, 12.2010, p. 547-556.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Rare diffuse diseases of the lung
T2 - Pulmonary alveolar proteinosis, lymphangioleiomyomatosis, amyloidosis
AU - Morbini, P.
AU - Guddo, F.
AU - Contini, P.
AU - Luisetti, M.
AU - Schiavina, M.
AU - Zompatori, M.
PY - 2010/12
Y1 - 2010/12
N2 - Pulmonary alveolar proteinosis (PAP), lymphangioleyomiomatosis (LAM) and amyloidosis are three unrelated diseases of rare occurrence, with characteristic histopathological features. A pattern of alveolar filling with granular pink material accumulation is characteristic of PAP. This material can be recognized in lung biopsies, but also in bronchial lavage fluid. PAP is clinically related to the abnormal clearance of alveolar surfactant, most commonly due to the disruption of the granulocyte macrophagecolony stimulating factor signalling pathway. Whole lung lavage is the treatment of choice. LAM is characterized by cystic lung degeneration and interstitial proliferation of LAM cells, which express both melanocyte and smooth muscle cell markers, has a typical cystic pattern on CT scan, can be associated clinically with abdominal angiomyolipomas and limphangioleiomyomas, and occurs in female patients, either in isolation or as a manifestation of tuberous sclerosis. Sex hormone manipulation is the therapy of choice in this otherwise progressive disease. Diffuse interstitial or perivascular amyloid deposits in the lung can form in the context of systemic amyloidosis, usually associated with myeloma or monoclonal gammopathy, and less often with chronic inflammatory diseases. Nodular amyloid deposits, in contrast, are not associated with systemic lung disease, and present instrumentally as a coin lesion or lung mass. Isolated tracheobronchial amyloidosis is another rare form that is not related to systemic disease. In all conditions, amyloid has a typical waxy, amorphous, slightly eosinophilic stain, stains red with Congo red and presents a characteristic apple-green birefringence under polarized light, which is essential for diagnosis.
AB - Pulmonary alveolar proteinosis (PAP), lymphangioleyomiomatosis (LAM) and amyloidosis are three unrelated diseases of rare occurrence, with characteristic histopathological features. A pattern of alveolar filling with granular pink material accumulation is characteristic of PAP. This material can be recognized in lung biopsies, but also in bronchial lavage fluid. PAP is clinically related to the abnormal clearance of alveolar surfactant, most commonly due to the disruption of the granulocyte macrophagecolony stimulating factor signalling pathway. Whole lung lavage is the treatment of choice. LAM is characterized by cystic lung degeneration and interstitial proliferation of LAM cells, which express both melanocyte and smooth muscle cell markers, has a typical cystic pattern on CT scan, can be associated clinically with abdominal angiomyolipomas and limphangioleiomyomas, and occurs in female patients, either in isolation or as a manifestation of tuberous sclerosis. Sex hormone manipulation is the therapy of choice in this otherwise progressive disease. Diffuse interstitial or perivascular amyloid deposits in the lung can form in the context of systemic amyloidosis, usually associated with myeloma or monoclonal gammopathy, and less often with chronic inflammatory diseases. Nodular amyloid deposits, in contrast, are not associated with systemic lung disease, and present instrumentally as a coin lesion or lung mass. Isolated tracheobronchial amyloidosis is another rare form that is not related to systemic disease. In all conditions, amyloid has a typical waxy, amorphous, slightly eosinophilic stain, stains red with Congo red and presents a characteristic apple-green birefringence under polarized light, which is essential for diagnosis.
KW - Lymphangioleiomyomatosis
KW - Polmonary amyloidosis
KW - Pulmonary alveolar proteinosis
UR - http://www.scopus.com/inward/record.url?scp=79953659123&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=79953659123&partnerID=8YFLogxK
M3 - Article
C2 - 21428118
AN - SCOPUS:79953659123
VL - 102
SP - 547
EP - 556
JO - Pathologica
JF - Pathologica
SN - 0031-2983
IS - 6
ER -