Relationship Between Diffuse Pulmonary Fibrosis, Alveolar Proteinosis, and Granulocyte-Macrophage Colony Stimulating Factor Autoantibodies

Luisetti, Maurizio; Bruno, Pierdonato; Kadija, Zamir; Suzuki, Takuji; Raffa, Salvatore; Torrisi, Maria Rosaria; Campo, Ilaria; Mariani, Francesca; Pozzi, Ernesto; Trapnell, Bruce C.; Mariotta, Salvatore

doi:10.4187/respcare.01054

Cited by 11 publications

(8 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In these cases, as in ours, the features of PAP disappeared as pulmonary fibrosis progressed [5, 7]. As Luisetti et al mentioned, it is not possible for us to exclude that some subjects diagnosed with diffuse fibrotic lung disease actually represented the end-stage evolution of a previous pulmonary alveolar proteinosis process [7]. If a patient’s BALF or HRCT does not show a typical PAP appearance at the time of admission, serum GM-CSF autoantibody is not usually measured.…”

Section: Case Presentationsupporting

confidence: 58%

See 1 more Smart Citation

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

et al. 2014

View full text Add to dashboard Cite

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.

show abstract

Section: Case Presentationsupporting

confidence: 58%

“…In the contemporaneous cohort of 223 Japanese aPAP patients, 3 patients (1.4%) were complicated by severe respiratory failure due to pulmonary fibrosis [2]. And so far, there have been, at least, 5 case reports, which highly suggested the pathogenetic relationship between aPAP and pulmonary fibrosis [3–7]. …”

Section: Introductionmentioning

confidence: 99%

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

et al. 2014

View full text Add to dashboard Cite

show abstract

“…[1] Three types of PAP have been described: idiopathic, secondary, and congenital. [4,5] The causes of secondary PAP include hematologic diseases such as MDS and multiple myeloma, inhalation exposure to dust, and infectious disease. [6] Congenital PAP is caused by gene mutations of SP-B or C or the GM-CSF receptor [2].…”

Section: Discussionmentioning

confidence: 99%

A Case of Autoimmune Pulmonary Alveolar Proteinosis with Pulmonary Fibrosis and Asbestosis-Like Features

et al. 2019

View full text Add to dashboard Cite

Pulmonary alveolar proteinosis (PAP) is a lung disease characterized by accumulation of lipid-rich proteinaceous materials that stain positive with periodic acid-Schiff (PAS) in the pulmonary alveolar space [1,2]. Although autoimmune PAP, pulmonary asbestosis, and idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP) are independent pulmonary disorders, cases of secondary PAP caused by asbestosis or PAP-leading pulmonary fibrosis may occur occasionally. Differential diagnosis among these diseases is important but difficult. Here we report a case of autoimmune PAP with pulmonary asbestosis-like features including asbestos bodies in the lung tissue and pleural thickening on high-resolution computed tomography (HRCT), and a histologically possible UIP pattern. CASE REPORT A 78-year-old man visited a local hospital in May 2016 because of persistent productive cough and ground-glass opacities (GGOs) were observed on a chest radiograph and HRCT at a nearby hospital in May 2016. The productive cough eventually resolved, but the chest radiograph and HRCT showed expansion

show abstract

“…Les sites infectés étaient majoritairement pulmonaires (75 à 86 % des cas), mais des abcès cérébraux étaient rapportés lors des nocardioses (19 % des cas) ; une dissémination ganglionnaire, hépatique, médullaire, céré-brale ou oculaire était rencontrée dans 25 % des infections à mycobactéries, et la moitié des infections fongiques était disséminée [37]. Des cas de fibrose pulmonaire secondaire ont également été décrits [38,39].…”

Section: éVolution Et Complicationsunclassified

La protéinose alvéolaire pulmonaire

Jouneau

Kerjouan

Briens

et al. 2014

Revue des Maladies Respiratoires

View full text Add to dashboard Cite

Alveolar proteinosis (AP) is a rare disease characterized by alveolar accumulation of surfactant components, which impairs gas exchange. AP is classified into three groups: auto-immune AP defined by the presence of plasma autoantibodies anti-GM-CSF, the most frequent form (90% of all AP); secondary AP, mainly occurring as a consequence of haematological diseases, or following on from toxic inhalation or infections, and genetic AP, which affects almost exclusively children. AP diagnosis is suspected where chest CT-scan demonstrates interstitial lung disease with a crazy paving aspect; and confirmed by bronchoalveolar lavage, which has a milky appearance and contains periodic acid Schiff positive proteinaceous alveolar deposits. The use of surgical lung biopsy to confirm AP is less frequent nowadays. In this context, positive antibodies against GM-CSF indicates an auto-immune etiology of the AP. Concerning management, whole lung lavage is the gold standard therapy. In refractory AP, new treatments are available such as subcutaneous or inhaled GM-CSF supplementation, or rituximab infusions. The clinical course is unpredictable. Spontaneous improvement or even cure can occur, and the 5-year actuarial survival is 95%. The most frequent complications are infectious etiology.

show abstract

Relationship Between Diffuse Pulmonary Fibrosis, Alveolar Proteinosis, and Granulocyte-Macrophage Colony Stimulating Factor Autoantibodies

Cited by 11 publications

References 10 publications

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

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

A Case of Autoimmune Pulmonary Alveolar Proteinosis with Pulmonary Fibrosis and Asbestosis-Like Features

La protéinose alvéolaire pulmonaire

Contact Info

Product

Resources

About