Autoimmune pulmonary alveolar proteinosis: Treatment options in year 2013

Leth, Steffen; Bendstrup, Elisabeth; Vestergaard, Hanne; Hilberg, Ole

doi:10.1111/j.1440-1843.2012.02274.x

Cited by 63 publications

(73 citation statements)

References 79 publications

(167 reference statements)

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“…Lung transplantation and plasmapheresis have been performed and rituximab has been administered in selected cases, but further studies are needed on this issue. The rate of 5-year survival is 74.7%±8.1% in PAP patients (7). Spontaneous progression can be observed in the disease.…”

Section: Pulmonary Alveolar Proteinosismentioning

confidence: 85%

“…Because our patient was asymptomatic and no gas exchange abnormality was observed, we decided to follow up the patient, and TPL was not performed. The use of GM-CSF (inhaler or subcutaneous) in PAP patients is recommended for patients with progressive course of the disease despite TPL treatment (7). Lung transplantation and plasmapheresis have been performed and rituximab has been administered in selected cases, but further studies are needed on this issue.…”

Section: Pulmonary Alveolar Proteinosismentioning

confidence: 99%

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Bilateral Upper Zone Patchy Opacities on the Chest Radiography Taken for Screening in a Patient without Any Smptoms: What Is Your Diagnosis?

Çalışkan¹,

Uyar²,

Demirel³

et al. 2015

Eurasian J Pulmonol

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A 20-year-old male patient who worked as a salesman was referred to the Department of Chest Diseases due to opacities seen in the upper zones of both lungs on the posteroanterior chest radiography taken for screening in the tuberculosis control dispensary. The patient had no complaint, and the physical examination revealed no pathological finding. His arterial blood pressure was 110/70 mmHg, pulse was 88/min, temperature was 36.7°C, and SpO 2 was 97% (oxygen-free). The results of laboratory analyses were found to be as follows: white blood cell count, 8.07×10 3 /µL; eosinophils, 3.46%; hemoglobin, 16.6 g/dL; hematocrit, 47.8%; platelet count, 274,000/µL; complete urine test, normal; sedimentation, 2 mm/hour; C-reactive protein, 1.06 mg/dL; aspartate aminotransferase, 51 U/L; alanine aminotransferase, 78 U/L; and urea, 25 mg/dL. In arterial blood gas, the value of PO 2 was 93.7 mmHg, PCO 2 was 33.7 mmHg, HCO 3 was 21.1 mmoL/L, SaO 2 was 97.9%, and pH was 7.38. The patient's posteroanterior chest radiography revealed diffuse patchy opacities in the upper and middle zones of both lungs (Figure 1). The patient had three negative sputum smear reports for acid-fast bacillus (AFB). There was a restrictive type disorder on the spirometry of the patient, and carbon monoxide diffusing capacity (DLCO) and DLCO/VA was measured as 64% and 98% respectively. High-resolution computed tomography (HRCT) of the chest revealed patchy ground-glass areas with peripheral localization,

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Section: Pulmonary Alveolar Proteinosismentioning

confidence: 85%

Section: Pulmonary Alveolar Proteinosismentioning

confidence: 99%

Bilateral Upper Zone Patchy Opacities on the Chest Radiography Taken for Screening in a Patient without Any Smptoms: What Is Your Diagnosis?

Çalışkan¹,

Uyar²,

Demirel³

et al. 2015

Eurasian J Pulmonol

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“…Secondary PAP is diagnosed based on a history of exposures such as hematologic diseases or inhalation agents and the lack of antibodies, while congenital PAP rests on genetic testing [1]. The standard treatment of autoimmune PAP is WLL, where the excess surfactant is mechanically cleansed from the lungs [6]. Reiterated treatments are often necessary.…”

Section: Discussionmentioning

confidence: 99%

“…Rituximab (an anti-CD 20 B-cell antibody) has been reported with beneficial clinical outcome due to improved DLCO, CT scan, symptoms, and in case reports corresponding a reduced number of GM-CSF antibodies. In the case of progressive terminal respiratory failure despite of attempts with the mentioned treatment modalities, lung transplantation may be the last choice of treatment, but has only been described in a few cases [6]. …”

Section: Discussionmentioning

confidence: 99%

Pulmonary alveolar proteinosis - a crazy presentation of dyspnea

Skov

Bendstrup

Davidsen

2018

European Clinical Respiratory Journal

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This case report demonstrates 44-year old man, presenting with recurring clinical pneumonias during a period of over 1 year. The patient was clinically affected with, i.a., weight loss, finger clubbing and severely reduced diffusion capacity. Repetitive chest x-rays showed bilateral and consolidating infiltrates, and a high-resolution computed tomography of the thorax (HRCT) exposed ground glass opacities superimposed on a reticular pattern as the so-called ‘crazy paving’ pattern. A bronchoscopic alveolar lavage revealed alveolar proteinaceous material compatible with the diagnosis pulmonary alveolar proteinosis (PAP). PAP is a rare syndrome where surfactant is accumulated in the alveoli, causing respiratory disease in typically young to middle-aged patients with male predominance. Both symptoms and prognosis are variable, and range from spontaneous remission to terminal respiratory failure. The standard treatment is whole lung lavage, where surfactant is mechanically rinsed from the lungs. The lack of specific clinical symptoms makes it easy to overlook the diagnosis, as supported by this case report. It serves as a reminder, that the findings of a crazy paving pattern on HRCT in young adults should alert of this rare disease, and advises on the further examinations required to make the diagnosis.

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“…However, not all patients respond to this treatment. Based on the current literature, a stepwise treatment plan is suggested starting with WLL, continuing to inhaled GM-CSF, and then to rituximab if the former treatment regimes are unsuccessful (Leth et al, 2013;Yamamoto et al, 2008). Some authors think that the whole-lung lavage is a safe and effective palliative procedure in pulmonary alveolar proteinosis and in the treatment of patients with pulmonary disease, such as cystic fibrosis or asthma, in which filling of the lung acini by liquid or solid material impairs oxygenation of the pulmonary capillary blood (Lippmann et al, 1977).…”

Section: Whole Lung Lavagementioning

confidence: 99%

Whole lung lavage therapy: Treatment for lung injury caused by paraquat poisoning

Kan¹,

Jian²,

Zhang³

et al. 2014

J. Toxicol. Environ. Health Sci.

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Paraquat poisoning is characterized by multi-organ failure and pulmonary fibrosis with respiratory failure, resulting in high mortality and morbidity. To serious paraquat patients, the effectiveness of conventional treatments is unsuccessful. Whole lung lavage is a technique that was developed in the 1960s with the purpose of removing lipoproteinaceous material that accumulates in the bronchi of patients with alveolar proteinosis, leading to clinical and functional improvement. Pneumoconioses are characterized as irreversible, progressive respiratory diseases. No effective therapy exists to prevent progression of these diseases. Whole lung lavage might limit the rate of disease progression through the removal of dust, inflammatory cells, and cytokines. Whole lung lavage is also used successfully to treat other lung diseases such as endogenous lipoid pneumonia and mineral oil lipoid pneumonia. Paraquat poisoning could not be controlled by only one method and combined therapies are needed. So, we hypothesized that whole lung lavage will provide a new therapy of acute lung injury caused by paraquat. On the base of conventional therapy for paraquat poisoning, whole lung lavage could be considered in the early time of poisoning and then followed by glucocorticoid for patients with moderate to severe paraquat poisoning.

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Autoimmune pulmonary alveolar proteinosis: Treatment options in year 2013

Cited by 63 publications

References 79 publications

Bilateral Upper Zone Patchy Opacities on the Chest Radiography Taken for Screening in a Patient without Any Smptoms: What Is Your Diagnosis?

Bilateral Upper Zone Patchy Opacities on the Chest Radiography Taken for Screening in a Patient without Any Smptoms: What Is Your Diagnosis?

Pulmonary alveolar proteinosis - a crazy presentation of dyspnea

Whole lung lavage therapy: Treatment for lung injury caused by paraquat poisoning

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