Clinical Importance of Bronchoalveolar Lavage Fluid and Blood Cytokines, Surfactant Protein D, and Kerbs von Lungren 6 Antigen in Idiopathic Pulmonary Alveolar Proteinosis

Lin, Fang Chi; Chen, Yi Chu; Chang, Shi Chuan

doi:10.4065/83.12.1344

Cited by 31 publications

(22 citation statements)

References 29 publications

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“…marker for not only the disease activity of pulmonary fibrosis (Kohno et al 1989), but also for the monitoring of autoimmune PAP (Inoue et al 2006;Lin et al 2008;Bonella et al 2013). In our case, at the time of the diagnosis of sarcoidosis and systemic sclerosis, the chest CT scan, BAL, and trans-bronchial lung biopsy could not show the existence of autoimmune PAP; however, serum KL-6 and anti-GM-CSF antibodies levels were already highly elevated.…”

Section: Discussionmentioning

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.

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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.

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Section: Discussionmentioning

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.

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show abstract

“…137 High levels of surfactant proteins, KL-6, and lactate dehydrogenase in serum and BALF have been described as evidence of severe disease. 12,[138][139][140][141][142] Several investigators have emphasized the value of these biomarkers in the diagnosis and severity assessment, 27,142 but, due to their low specificity, they are seldom utilized. Similarly, tumor markers, such as squamous-cell carcinoma antigen, carcinoembryonic antigen, carbohydrate antigen 19-9, and cytokeratin 19 fragments, have been used in assessing pulmonary alveolar proteinosis.…”

Section: Biomarkersmentioning

confidence: 99%

Pulmonary Alveolar Proteinosis

Khan

Agarwal

2011

Respir Care

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“…It has also been reported that KL-6 induces chemotaxis of human fibroblasts in vitro, suggesting that it may have a pathological role in fibrosing lung disease [21]. Therefore, measurement of serum KL-6 is now widely accepted as a diagnostic marker for monitoring the activity of interstitial lung diseases, such as idiopathic interstitial pneumonia, and for the long-term management of various interstitial diseases [1,2,[7][8][9][10][11]13].…”

Section: Discussionmentioning

confidence: 99%

“…Several investigators have also reported that KL-6 is a useful serum marker to confirm diagnosis and for long-term management in patients with diffuse pulmonary diseases, particularly interstitial lung diseases. Patients with idiopathic pulmonary fibrosis or non-specific interstitial pneumonia showed significantly elevated KL-6 levels [8][9][10][11][12][13].…”

mentioning

confidence: 99%

Comparison of pulmonary thin section CT findings and serum KL-6 levels in patients with sarcoidosis

Honda¹,

Okada²,

Ando³

et al. 2011

BJR

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Clinical Importance of Bronchoalveolar Lavage Fluid and Blood Cytokines, Surfactant Protein D, and Kerbs von Lungren 6 Antigen in Idiopathic Pulmonary Alveolar Proteinosis

Cited by 31 publications

References 29 publications

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

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

Pulmonary Alveolar Proteinosis

Comparison of pulmonary thin section CT findings and serum KL-6 levels in patients with sarcoidosis

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