Direct evidence that GM-CSF inhalation improves lung clearance in pulmonary alveolar proteinosis

Ohashi, Kazumasa; Sato, Susumu; Takada, Toshinori; Arai, Toru; Nei, Takahito; Kasahara, Yasunori; Motoi, Natsuki; Hojo, Masayuki; Urano, Shinya; Ishii, Haruyuki; Yokoba, Masanori; Eda, Ryosuke; Nakayama, Hideaki; Nasuhara, Yasuyuki; Tsuchihashi, Yoshiko; Kaneko, Chinatsu; Kanazawa, Hiroko; Ebina, Masahito; Yamaguchi, Etsuro; Kirchner, Jacqueline; Inoue, Yoshikazu; Nakata, Koh; Tazawa, Ryushi

doi:10.1016/j.rmed.2011.10.019

Cited by 31 publications

(22 citation statements)

References 24 publications

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“…The 2-year-old LPI patient in their report had large amounts of cholesterol, cholesterol crystals and lipid-laden macrophages in the airways, and the authors suggested that therapies aiming to decrease the amount of cholesterol in the airways might be beneficial instead. On the other hand, in the study by Ohashi and colleagues, the GM-CSF inhalation therapy improved lung clearance and decreased the amount of protein in BAL fluid in a patient with autoimmune alveolar proteinosis (Ohashi et al 2012). Furthermore, in 2014, Yu and colleagues reported a patient with autoimmune PAP treated successfully with a combination of whole lung lavage and inhaled GM-CSF .…”

Section: Discussionmentioning

confidence: 99%

Inhaled Sargramostim Induces Resolution of Pulmonary Alveolar Proteinosis in Lysinuric Protein Intolerance

et al. 2016

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Pulmonary alveolar proteinosis (PAP) is a potentially fatal complication of lysinuric protein intolerance (LPI), an inherited disorder of cationic amino acid transport. The patients often present with mild respiratory symptoms, which may rapidly progress to acute respiratory failure responding poorly to conventional treatment with steroids and bronchoalveolar lavations (BALs). The pathogenesis of PAP in LPI is still largely unclear. In previous studies, we have shown disturbances in the function and activity of alveolar macrophages of these patients, suggesting that increasing the activity and the number of macrophages by recombinant human GM-CSF (rhuGM-CSF) might be beneficial in this patient group.Two LPI patients with complicated PAP were treated with experimental inhaled rhuGM-CSF (sargramostim) after poor response to maximal conventional therapy.

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Section: Discussionmentioning

confidence: 99%

Inhaled Sargramostim Induces Resolution of Pulmonary Alveolar Proteinosis in Lysinuric Protein Intolerance

et al. 2016

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“…Several trials have examined the efficacy of either inhaled or subcutaneous GM-CSF in patients with PAP with anti-GM-CSF antibodies. [565][566][567] Therapy does not seem to affect autoantibody titers, and both routes of administration were effective, possibly by complexing with (consuming) the autoantibody. Long-term administration appears to be both effective and safe.…”

Section: Defects Of the Respiratory Burst Chronic Granulomatous Disementioning

confidence: 99%

Practice parameter for the diagnosis and management of primary immunodeficiency

Bernstein¹,

Blessing-Moore²,

Khan³

et al. 2015

Journal of Allergy and Clinical Immunology

572

494

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The American Academy of Allergy, Asthma & Immunology (AAAAI) and the American College of Allergy, Asthma & Immunology (ACAAI) have jointly accepted responsibility for establishing the "Practice parameter for the diagnosis and management of primary immunodeficiency." This is a complete and comprehensive document at the current time. The medical environment is a changing environment, and not all recommendations will be appropriate for all patients. Because this document incorporated the efforts of many participants, no single individual, including those who served on the Joint Task Force, is authorized to provide an official AAAAI or ACAAI interpretation of these practice parameters. Any request for information about or an interpretation of these practice parameters by the AAAAI or ACAAI should be directed to the Executive Offices of the AAAAI, the ACAAI, and the Joint Council of Allergy, Asthma & Immunology. These parameters are not designed for use by pharmaceutical companies in drug promotion.

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“…In cases of acquired PAP, definitive treatment rests on addressing the underlying disorder or avoiding the suspected environmental agent (1). Recently, studies have demonstrated that GM-CSF (inhalation or subcutaneous) can result in improvement in some patients with idiopathic PAP (12,13). Other options focus on reducing the amount of autoantibodies via B-cell depletion therapy (rituximab) or removal of autoantibodies (plasmapheresis); however, the exact role of these modalities remains to be defined (14,15).…”

Section: Abstract: Alveolar Infiltrates; Crazy Paving; Gm-csf; Pulmomentioning

confidence: 99%

Pulmonary alveolar proteinosis

Vasei¹,

Modaresi²,

Safavi³

2019

Postgraduate Medical Journal

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Learning objectives• To recognize the various presentations and appropriate management of pulmonary alveolar proteinosis (PAP). • To understand the various acquired causes of PAP. Pre-test• What is the etiology of PAP?• What are the various differential diagnoses associated with the 'crazy-paving' pattern that need to be considered and ruled out before making a diagnosis of PAP? CASE PRESENTATIONA 32-year-old Caucasian man without a significant medical history presented with a two-month history of progressively worsening dyspnea on exertion. He denied any cough, hemoptysis, chest pain, lower extremity swelling, fevers, chills, night sweats, weight change, new rashes, arthralgias or myalgias. He had no exotic travel or sick contacts. He was a 10 pack-year smoker and occasionally consumed alcohol. He denied any illicit drug use. He worked in construction, and his hobbies included woodworking, in which he was chronically exposed to cement and saw dust. Before his visit to the Mayo Clinic (Minnesota, USA), he was treated by other providers with various courses of antibiotics and prednisone without relief. A review of systems was otherwise unrevealing. On physical examination, the patient's vital signs were as follows: temperature 36.6°C; blood pressure 96/70 mmHg; pulse rate 80 beats/min; respiratory rate 18 breaths/min; and oxygen saturation 88% on room air. Most notably, his lung examination demonstrated scattered inspiratory crackles without any rales, rhonchi or wheezes. The cardiac, vascular, abdominal, lymphatic and integument examinations were all unremarkable.

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Direct evidence that GM-CSF inhalation improves lung clearance in pulmonary alveolar proteinosis

Abstract: GM-CSF inhalation decreased the concentration of total protein and SP-A in BALF, and increase interleukin-17 and cancer antigen-125 in improved lung of autoimmune pulmonary alveolar proteinosis.

Cited by 31 publications

References 24 publications

Inhaled Sargramostim Induces Resolution of Pulmonary Alveolar Proteinosis in Lysinuric Protein Intolerance

Inhaled Sargramostim Induces Resolution of Pulmonary Alveolar Proteinosis in Lysinuric Protein Intolerance

Practice parameter for the diagnosis and management of primary immunodeficiency

Pulmonary alveolar proteinosis

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