A combination therapy of whole lung lavage and GM‐CSF inhalation in pulmonary alveolar proteinosis

Yamamoto, H; Yamaguchi, Etsuro; Agata, Hiroatsu; Kandatsu, Nobuhisa; Komatsu, Toru; Kawai, S; Baba, Kenji; Awaya, Tomonari; Nishikomori, Ryuta; Tsurusawa, Masahito; Nakata, Koh

doi:10.1002/ppul.20856

Cited by 40 publications

(30 citation statements)

References 7 publications

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“…In this respect, antecedent lung lavage may be useful before GM-CSF therapy to further increase efficacy. Such an approach was successful in a 9-year-old girl with severe autoimmune PAP (24). In the present study, A-aDO 2 did not fully normalize during the treatment in many patients, implying the presence of residual disease.…”

Section: Discussionmentioning

confidence: 67%

Inhaled Granulocyte/Macrophage–Colony Stimulating Factor as Therapy for Pulmonary Alveolar Proteinosis

Tazawa¹,

Trapnell²,

Inoue³

et al. 2010

Am J Respir Crit Care Med

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192

153

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Rationale: Inhaled granulocyte/macrophage-colony stimulating factor (GM-CSF) is a promising therapy for pulmonary alveolar proteinosis (PAP) but has not been adequately studied. Objectives: To evaluate safety and efficacy of inhaled GM-CSF in patients with unremitting or progressive PAP. Methods: We conducted a national, multicenter, self-controlled, phase II trial at nine pulmonary centers throughout Japan. Patients who had lung biopsy or cytology findings diagnostic of PAP, an elevated serum GM-CSF antibody level, and a Pa O 2 of less than 75 mm Hg entered a 12-week observation period. Those who improved (i.e., alveolar-arterial oxygen difference [A-aDO 2 ] decreased by 10 mm Hg) during observation were excluded. The rest entered sequential periods of high-dose therapy (250 mg Days 1-8, none Days 9-14; 3 six cycles; 12 wk); low-dose therapy (125 mg Days 1-4, none Days 5-14; 3 six cycles; 12 wk), and follow-up (52 wk). Measurements and Main Results: Fifty patients with PAP were enrolled in the study. During observation, nine improved and two withdrew; all of these were excluded. Of 35 patients completing the high-and low-dose therapy, 24 improved, resulting in an overall response rate of 62% (24/39; intention-to-treat analysis) and reduction in A-aDO 2 of 12.3 mm Hg (95% confidence interval, 8.4-16.2; n 5 35, P , 0.001). No serious adverse events occurred, and serum GM-CSF autoantibody levels were unchanged. A treatment-emergent correlation occurred between A-aDO 2 and diffusing capacity of the lung, and high-resolution CT revealed improvement of ground-glass opacity. Twenty-nine of 35 patients remained stable without further therapy for 1 year. Conclusions: Inhaled GM-CSF therapy is safe, effective, and provides a sustained therapeutic effect in autoimmune PAP.

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Section: Discussionmentioning

confidence: 67%

Inhaled Granulocyte/Macrophage–Colony Stimulating Factor as Therapy for Pulmonary Alveolar Proteinosis

Tazawa¹,

Trapnell²,

Inoue³

et al. 2010

Am J Respir Crit Care Med

Self Cite

192

153

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“…WLLs are performed in disease states associated with alveolar filling by abnormal material, with the most frequent being pulmonary alveolar proteinosis [11,[17][18][19][20][21][22][23][24][25]. In addition, WLLs have been used in children with metabolic disorders such as lysinuric protein intolerance [26,27], Niemann-Pick disease [28][29][30] or lipoid pneumonia [31][32][33][34][35][36][37].…”

Section: Resultsmentioning

confidence: 99%

ERS statement: interventional bronchoscopy in children

et al. 2017

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Paediatric airway endoscopy is accepted as a diagnostic and therapeutic procedure, with an expanding number of indications and applications in children. The aim of this European Respiratory Society task force was to produce a statement on interventional bronchoscopy in children, describing the evidence available at present and current clinical practice, and identifying areas deserving further investigation. The multidisciplinary task force panel performed a systematic review of the literature, focusing on whole lung lavage, transbronchial and endobronchial biopsy, transbronchial needle aspiration with endobronchial ultrasound, foreign body extraction, balloon dilation and occlusion, laser-assisted procedures, usage of airway stents, microdebriders, cryotherapy, endoscopic intubation, application of drugs and other liquids, and caregiver perspectives. There is a scarcity of published evidence in this field, and in many cases the task force had to resort to the collective clinical experience of the committee to develop this statement. The highlighted gaps in knowledge underline the need for further research and serve as a call to paediatric bronchoscopists to work together in multicentre collaborations, for the benefit of children with airway disorders.

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“…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%

Pulmonary Alveolar Proteinosis

Khan

Agarwal

2011

Respir Care

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A combination therapy of whole lung lavage and GM‐CSF inhalation in pulmonary alveolar proteinosis

Cited by 40 publications

References 7 publications

Inhaled Granulocyte/Macrophage–Colony Stimulating Factor as Therapy for Pulmonary Alveolar Proteinosis

Inhaled Granulocyte/Macrophage–Colony Stimulating Factor as Therapy for Pulmonary Alveolar Proteinosis

ERS statement: interventional bronchoscopy in children

Pulmonary Alveolar Proteinosis

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