BAL findings in a patient with pulmonary alveolar proteinosis successfully treated with GM-CSF

Schoch, Otto D.; Schanz, Urs; Koller, Moshe; Nakata, Koh; Seymour, John F.; Russi, Erich W.; Boehler, Annette

doi:10.1136/thorax.57.3.277

Cited by 85 publications

(59 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Several reports found favorable response with systemic (subcutaneous) or localized (aerosol) GM-CSF. 10,132,136,142,[182][183][184][185][186][187][188][189][190][191] Prompt improvement with resumption of GM-CSF in patients who relapsed with GM-CSF therapy suggests that disease resolution was not attributable to spontaneous remission and that GM-CSF does have therapeutic activity. 183 Response is generally slow after GM-CSF therapy: the majority of patients show P (A-a)O 2 improvement of Ն 10 mm Hg only after 4 -6 weeks.…”

Section: Exogenous Granulocyte Macrophage Colony Stimulating Factor Tmentioning

confidence: 99%

“…183 Response is generally slow after GM-CSF therapy: the majority of patients show P (A-a)O 2 improvement of Ն 10 mm Hg only after 4 -6 weeks. [181][182][183][184][185][186][187][188][189][190] Despite the slower improvement in oxygenation with GM-CSF, the magnitude of therapeutic effect achievable with GM-CSF or whole-lung lavage may be similar. 184 The observed delay in response is due to the time required for immature precursor cells to be recruited to the lung and stimulated by GM-CSF to differentiate into functional alveolar macrophages.…”

Section: Exogenous Granulocyte Macrophage Colony Stimulating Factor Tmentioning

confidence: 99%

See 1 more Smart Citation

Pulmonary Alveolar Proteinosis

Khan

Agarwal

2011

Respir Care

View full text Add to dashboard Cite

Section: Exogenous Granulocyte Macrophage Colony Stimulating Factor Tmentioning

confidence: 99%

Section: Exogenous Granulocyte Macrophage Colony Stimulating Factor Tmentioning

confidence: 99%

Pulmonary Alveolar Proteinosis

Khan

Agarwal

2011

Respir Care

View full text Add to dashboard Cite

“…A cultivation test of the BALF also did not detect any bacteria or fungi. The titer of anti-GM-CSF antibodies measured by ELISA method (Schoch et al 2002;Uchida et al 2007) was 10.8 µg/mL (cut off value < 1.0 µg/mL, Fig. 1).…”

Section: Case Reportmentioning

confidence: 99%

Elevated Serum Anti-GM-CSF Antibodies before the Onset of Autoimmune Pulmonary Alveolar Proteinosis in a Patient with Sarcoidosis and Systemic Sclerosis

Yamasue

Nureki

Usagawa

et al. 2017

Tohoku J. Exp. Med.

View full text Add to dashboard Cite

Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of periodic acid-schiff stainpositive lipoproteinaceous materials in the alveolar space due to impaired surfactant clearance by alveolar macrophage. Autoimmune PAP is the most common form of PAP, but rarely accompanies collagen disease or sarcoidosis. We report here a rare case of autoimmune PAP preceded by systemic sclerosis and sarcoidosis. A 64-year-old woman was admitted to our hospital for blurred vision, muscle weakness of extremities, Raynaud's phenomenon, and exertional dyspnea. We diagnosed her as having systemic sclerosis complicated with sarcoidosis. Chest computed tomography (CT) and transbronchial lung biopsy showed the findings of pulmonary fibrosis without PAP. We treated her with corticosteroid and intravenous cyclophosphamide therapy, followed by tacrolimus therapy. Thereafter, her symptoms improved except for exertional dyspnea, and she began to complain of productive cough thirteen months after corticosteroid and immunosuppressant therapy. On the second admission, a chest CT scan detected the emergence of crazy-paving pattern in bilateral upper lobes. Bronchoalveolar lavage (BAL) fluid with milky appearance and a lung biopsy specimen revealed acellular periodic acid-schiff stain-positive bodies. The serum titer of anti-granulocyte macrophage colony stimulating factor (GM-CSF) antibodies was elevated on first admission and remained high on second admission. We thus diagnosed her as having autoimmune PAP. Reducing the dose of immunosuppressive agents and repeating the segmental BAL resulted in the improvement of her symptoms and radiological findings. Immunosuppressant therapy may trigger the onset of autoimmune PAP in a subset of patients with systemic sclerosis and/or sarcoidosis.

show abstract

“…The correlation analysis between the significant immunocytes and dyspnea score, score of chest HRCT, lung function index and PaO 2 , indicated that these immunocytes were not associated with the severity of PAP. However, a predominance of CD 4+ cells in some idiopathic PAP cases was reported previously (15). Thus, more studies should be conducted to determine the role of these cells in idiopathic PAP.…”

Section: Discussionmentioning

confidence: 90%

Biochemical index and immunological function in the peripheral blood of patients with idiopathic pulmonary alveolar proteinosis

Bai

Shi

et al. 2013

Biomedical Reports

View full text Add to dashboard Cite

Abstract. Idiopathic pulmonary alveolar proteinosis (PAP) has recently been recognized as a disease of impaired alveolar macrophage function caused by the neutralizing granulocyte-macrophage colony stimulating factor (GM-CSF) autoantibody. However, the change of immunological function and biochemical index in the peripheral blood of patients with idiopathic PAP remains unclear. The clinical data of 29 patients with idiopathic PAP and 30 normal subjects were retrospectively analyzed. Biochemical indices, immunoglobulin and complement of all participants, and immunocytes in 19 patients and 30 normal subjects were evaluated. The peripheral blood of the patients showed a decrease in CD4 + /CD8 + (P<0.05) and the percentage of the CD4 + T lymphocyte and helper T lymphocyte (both P<0.01), whereas an increase was observed in the percentage of the suppressor T lymphocyte (P<0.01) compared to the normal subjects. No significant differences were found in the concentration of IgG, IgA, IgM, C3 and C4 between the two groups. An increase was observed in LDH (P<0.01) and cytokeratin fragment antigen (CYFR) 211 (P<0.01) in the peripheral blood of the patients compared to that of normal subjects. The serum level of LDH was negatively correlated with FVC% Pred (P<0.05), D L CO% Pred (P<0.05), PaO 2 (P<0.05) and positively associated with the dyspnea score (P<0.05) of 29 patients, and positively correlated with the score of high-resolution computed tomography (HRCT) of the chest of 21 patients (P<0.05). The serum level of CYFR211 was negatively correlated with D L CO% Pred (P<0.05) and positively associated with the dyspnea score (P<0.05). Findings of the present study suggest that hypofunction of cellular immunity may exist in the patients with idiopathic PAP. LDH and CYFRA211 may be considered as important indices to monitor the severity of idiopathic PAP.

show abstract

BAL findings in a patient with pulmonary alveolar proteinosis successfully treated with GM-CSF

Cited by 85 publications

References 13 publications

Pulmonary Alveolar Proteinosis

Pulmonary Alveolar Proteinosis

Elevated Serum Anti-GM-CSF Antibodies before the Onset of Autoimmune Pulmonary Alveolar Proteinosis in a Patient with Sarcoidosis and Systemic Sclerosis

Biochemical index and immunological function in the peripheral blood of patients with idiopathic pulmonary alveolar proteinosis

Contact Info

Product

Resources

About