Pulmonary alveolar proteinosis mimicking idiopathic pulmonary fibrosis

Arbiser, Zoya K.; Guidot, David M.; Pine, Jeffrey R.; Giltman, Larry I.; Gal, Anthony A.

doi:10.1053/adpa.2003.50013

Cited by 7 publications

(9 citation statements)

References 14 publications

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“…10---17 In some cases the onset of PAP was clearly established prior to fibrosis, using serial lung biopsies. 15,17 A review by Holbert regarding CT findings in a small group of patients with PAP found substantial fibrosis in 2 cases. 10 Interestingly, a recent paper from Ishii also found that a marked cystic and interstitial appearance might be particularly related to hereditary or secondary forms of PAP.…”

Section: Palavras-chavementioning

confidence: 99%

A novel immunodeficiency syndrome as a rare cause of secondary pulmonary alveolar proteinosis: A diagnosis after 5 decades

Ferreira

Carvalho

Gamboa

2014

Revista Portuguesa de Pneumologia (English Edition)

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Section: Palavras-chavementioning

confidence: 99%

A novel immunodeficiency syndrome as a rare cause of secondary pulmonary alveolar proteinosis: A diagnosis after 5 decades

Ferreira

Carvalho

Gamboa

2014

Revista Portuguesa de Pneumologia (English Edition)

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“…Some investigators concluded that pulmonary fibrosis is secondary to PAP (3,4,(6)(7)(8) and others consider fibrosis a coincidental finding in the natural history of PAP (5). Ohtsuki et al used biopsied or autopsied samples of patients with PAP to show that immunoreactive SP-A localizes in fine granules (FGs) and SP-D localizes in eosinophilic large coarse granules (CGs) (9).…”

Section: F I G U R E 3 a ) T H E F I N E G R A N U L A R Ma T E R Imentioning

confidence: 99%

“…We confirmed the abnormal opacities of the upper lung fields at the age of 11 years and these opacities increased gradually with time. Intra-alveolar accumulation of proteins and phospholipids may induce fibrosis directly or indirectly by altering cytokine expression (6). The proteinaceous material may form crystals when present for a long time, resulting in a granulomatous reaction in the surrounding alveolar wall and fibrosis (7).…”

Section: F I G U R E 3 a ) T H E F I N E G R A N U L A R Ma T E R Imentioning

confidence: 99%

“…Pulmonary cholesterol pneumonitis or cholesterol granuloma is also a rare condition that presents as sheaves of acicular clefts of cholesterol crystals in both the alveolar spaces and the interstitium. PAP typically presents with minimal interstitial inflammation and fibrosis, although patients with long-standing disease may develop some interstitial fibrosis and chronic inflammation (2)(3)(4)(5)(6)(7)(8).…”

Section: Introductionmentioning

confidence: 99%

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A Young Woman with Interstitial Pneumonia and Coexisting Deposition of Surfactant Proteins

et al. 2010

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We report a 23-year-old woman with interstitial inflammation and fibrosis associated with deposition of surfactant proteins in the airways. Biopsy of the lung showed multiple focal lesions containing dilated airways with inflammation and fibrosis. Pale eosinophilic amorphous material, dense eosinophilic bodies and numerous cholesterin clefts were observed in the airways. Surfactant protein A (SP-A) was expressed in the amorphous material. SP-D was identified in the dense eosinophilic bodies and around the acicular clefts. The finding that surfactant depositions were anatomically coincident with the interstitial pneumonia suggests that surfactant depositions were closely related to the interstitial pneumonia.

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“…The pathogenesis of pulmonary fibrosis in aPAP is unknown. It has been hypothesized that the retention of lipoproteinaceous material in the alveoli, silica exposure, and/or superimposed pulmonary infections induces damage to cells lining the alveoli and causes pulmonary fibrosis in aPAP patients [ 5 ]. In rats, the overexpression of GM-CSF in the lung by adenovirus-vector leads to pulmonary fibrosis, suggesting an inconclusive relationship between GM-CSF therapy and pulmonary fibrosis in patients with aPAP [ 8 ].…”

Section: Introductionmentioning

confidence: 99%

Development of microscopic polyangiitis-related pulmonary fibrosis in a patient with autoimmune pulmonary alveolar proteinosis

et al. 2014

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BackgroundAutoimmune pulmonary alveolar proteinosis (aPAP) is a rare lung disease caused by the autoantibody against granulocyte-macrophage colony stimulating factor (GM-CSF). The clinical course of aPAP is variable; in severe cases, patients develop lethal respiratory failure due to pulmonary fibrosis. However, the pathogenesis of pulmonary fibrosis in aPAP has never been delineated.Case presentationHere, we describe a rare case of aPAP that was subsequently complicated by microscopic polyangiitis-related pulmonary fibrosis. The patient was a 75-year-old Japanese man diagnosed with aPAP based on the crazy-paving appearance on high-resolution computed tomography (HRCT), “milky” appearance of broncho-alveolar lavage fluid (BALF), and elevated serum levels of the anti-GM-CSF antibody. The patient was followed-up without aPAP-specific treatment for 3 years. During this period, both hematuria and proteinuria appeared; in addition, serum myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) turned positive and increased markedly. The second BAL performed one year after the diagnosis, showed that the “milky” appearance had resolved. The HRCT showed that fibrotic changes had developed and that the crazy-paving appearance had disappeared. These data suggest an association between pulmonary fibrosis that developed during the natural course of aPAP and ANCA-related systemic vasculitis.ConclusionThis is the first case report that suggests the existence of a pathogenetic relationship between ANCA-associated systemic vasculitis and aPAP-related pulmonary fibrosis. The link between ANCA-associated systemic vasculitis and aPAP-related pulmonary fibrosis requires further investigation.

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Pulmonary alveolar proteinosis mimicking idiopathic pulmonary fibrosis

Cited by 7 publications

References 14 publications

A novel immunodeficiency syndrome as a rare cause of secondary pulmonary alveolar proteinosis: A diagnosis after 5 decades

A novel immunodeficiency syndrome as a rare cause of secondary pulmonary alveolar proteinosis: A diagnosis after 5 decades

A Young Woman with Interstitial Pneumonia and Coexisting Deposition of Surfactant Proteins

Development of microscopic polyangiitis-related pulmonary fibrosis in a patient with autoimmune pulmonary alveolar proteinosis

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