Pulmonary alveolar proteinosis in adults: pathophysiology and clinical approach

Kumar, Anupam; Abdelmalak, Basem; Inoue, Yoshikazu; Culver, Daniel A.

doi:10.1016/s2213-2600(18)30043-2

Cited by 127 publications

(128 citation statements)

References 109 publications

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“…dust-silica, aluminum, titanium, cellulose fibers, flour) or hematologic malignancies (e.g. myeloproliferative diseases, myeloid leukemia, lymphoma) [1]. The symptoms are often vague and the onset is often slowly developing, and thereby delaying the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…Histological examination will show lipoproteinaceous material in the terminal bronchioles with no or little inflammation and a preserved architecture of the alveoli, though often focally thickened septa (Figure 3). Lactate dehydrogenase is elevated in up to 80% of patients with PAP [1]. Pulmonary function tests will typically show a restrictive lung function pattern and a reduced DLCO.…”

Section: Discussionmentioning

confidence: 99%

“…Segmental atelectasis may occur due to obstruction. In chronic cases of PAP focal fibrosis may occur [1].

10.1080/20018525.2018.1552065-F0002Figure 2.A.…”

Section: Investigationsmentioning

confidence: 99%

“…Pulmonary alveolar proteinosis (PAP) is a rare syndrome in which surfactant is accumulated in the alveoli causing respiratory symptoms typically in young to middle-aged people [1]. Both symptoms and prognosis are variable ranging from spontaneous remission to terminal respiratory2 failure [2].…”

Section: Introductionmentioning

confidence: 99%

“…dust-silica and cellulose fibers or hematologic malignancies e.g. myeloproliferative diseases and myeloid leukemia [1]. PAP often debuts with insidious dyspnea at exertion and dry cough [3], which resembles a wide range of respiratory differential diagnoses.…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Segmental atelectasis may occur due to obstruction. In chronic cases of PAP focal fibrosis may occur [1].

10.1080/20018525.2018.1552065-F0002Figure 2.A.…”

Section: Investigationsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Pulmonary alveolar proteinosis - a crazy presentation of dyspnea

Skov

Bendstrup

Davidsen

2018

European Clinical Respiratory Journal

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Autoimmune pulmonary alveolar proteinosis prior to myelodysplastic syndrome

Foo

Chhor

Thien

2020

Respirology Case Reports

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We report the first case of autoimmune pulmonary alveolar proteinosis (PAP) associated with and preceding myelodysplastic syndrome. A 74-yearold female with a history of polymyalgia rheumatica presented with six months history of progressive exertional breathlessness. Examination revealed bilateral chest crackles and exertional desaturation. A diagnosis of autoimmune PAP was made based on the presence of autoantibodies to granulocyte-macrophage colony-stimulating factor and characteristic findings on chest computed tomography, bronchoalveolar lavage, and transbronchial biopsies. Bilateral whole lung lavage was performed with prompt improvement in symptoms. Fourteen months later, she presented with new breathlessness and was diagnosed with myelodysplasia on bone marrow biopsy. No recurrence of alveolar proteinosis was detected. This case highlights the importance of follow-up and screening of patients with autoimmune PAP for haematological conditions.

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Artificial stone‐associated silicosis with concurrent Cryptococcus infection

Shen

Lai

Tsai

et al. 2021

Respirology Case Reports

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Acute silicoproteinosis is a disease that develops in weeks, and lasting for years, after massive exposure to silica dust in relatively closed spaces. It was rare, but the cases have recently increased worldwide due to the development of artificial stone industry. Compared with traditional silicosis, artificial stone‐associated silicosis is more rapidly progressive and lethal. Hence, a correct diagnosis and optimal treatment are crucial. Here, we present the clinical course of a 33‐year‐old artificial stonemason who suffered from acute silicoproteinosis with concurrent Cryptococcus infection resulting in profound respiratory failure. This patient was treated by bronchoscope‐assisted therapeutic segmental lung lavage and antifungal agent, under mechanical ventilator and ECMO support and recovered well. A brief review of acute silicoproteinosis and artificial stone‐associated silicosis is also presented and highlights the new form of industry exposure to silica.

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Pulmonary alveolar proteinosis in adults: pathophysiology and clinical approach

Cited by 127 publications

References 109 publications

Pulmonary alveolar proteinosis - a crazy presentation of dyspnea

Pulmonary alveolar proteinosis - a crazy presentation of dyspnea

Autoimmune pulmonary alveolar proteinosis prior to myelodysplastic syndrome

Artificial stone‐associated silicosis with concurrent Cryptococcus infection

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