Updated Severity and Prognosis Score of Pulmonary Alveolar Proteinosis

Study Overview
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Brief Summary
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By updating the chest HRCT scoring criteria of patients with pulmonary alveolar proteinosis, a new and more perfect system for evaluating the severity of alveolar proteinosis will be established.

Detailed Description
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In past, investigators summarized a method that was the severity and prognosis of pulmonary alveolar proteinosis (SPSP) to assess the severity and prognosis of patients with autoimmune pulmonary alveolar proteinosis (auto PAP). The SPSP included five aspects: smoking, symptom, PaO2, chest CT score at specific levels (aortic arch, carina, pulmonary vein confluence, and superior septal level) and DLCO %predicted. But the chest CT score only focused on range of lesions, and inaccurate. So, investigators plan to adopt new score which involves the assessing to range and density. In the new Chest HRCT score, we added the density score. The average density of the lesions at a particular level of the patient's chest HRCT was measured by computer and classified into three degrees: mild (less than -400), moderate (greater than -400 and less than -100) and severe (greater than 100), denoted as 1 ', 2 'and3', respectively. The range score of lung opacity was estimated using a five-point scale: no opacity = 0; opacity involving < 25% of a region of hemithorax = 1; 25-50% = 2; 50-75% = 3; and ≥ 75% = 4. The lesion score for each layer is equal to the density score multiplied by the range score. The chest HRCT score was calculated by summing the lung opacity scores of the four representative regions of each hemithorax, and divided into several level: no opacity = 0; ≤10 = 1; 11 - 20 = 2; 21 - 30 = 3; 31 -40 = 4; > 40 = 5. According to the new chest HRCT score, the SPSP will be updated to SPSP II. In this study, patients with auto PAP from three centers (Shanghai Lung Hospital, Peking Union Medical College Hospital and Nanjing Drum Tower Hospital) were enrolled to evaluate the severity of disease severity score (DSS), SPSP and SPSP II, respectively, to evaluate the pros and cons of different scores and their impact on prognosis. About 30 newly diagnosed auto PAP patients were recruited, and were assess disease severity on the basis on SPSP II scores. According to SPSP II scores, appropriate treatment was used for the patients, who were followed up for 3 months.

Official Title
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Severity of Patients With Pulmonary Alveolar Proteinosis (PAP) Through Disease Severity Score (DSS), Severity and Prognosis Score of PAP (SPSP) and SPSPII

Conditions
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Pulmonary Alveolar Proteinosis

Intervention / Treatment
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* Other: Assesssing the severity of pulmonary alveolar proteinosis


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: The patients had be diagnosed with autoimmune pulmonary alveolar proteinosis through pathology or bronchial alveolar lavage fluid (BALF). The patients are old than 18 year. .Every patient had a complete medical history, chest HRCT, pulmonary function and arterial blood gas analysis. The patients have high level antibody of GM-CSF in serum or BALF. Exclusion Criteria: The patients had a congenital or secondary pulmonary alveolar proteinosis.

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Shanghai Pulmonary Hospital<br>Shanghai Pulmonary Hospital is a hospital specializing in the treatment of lung diseases. Many patients with pulmonary alveolar proteinosis receive treatment in this hospital. | Other: Assesssing the severity of pulmonary alveolar proteinosis<br>* Three severity scores will be used to assess the severity of patients with pulmonary alveolar proteinosis.<br>|
| Peking Union Medical College Hospital<br>Peking Union Medical College Hospital is a famous hospital in China. Many patients with rare pulmonary disease receive treatment in this hospital. | Other: Assesssing the severity of pulmonary alveolar proteinosis<br>* Three severity scores will be used to assess the severity of patients with pulmonary alveolar proteinosis.<br>|
| Nanjing Drum Tower Hospital<br>The Department of Respiratory and Critical Care Medicine of Nanjing Drum Tower Hospital has been focusing on the research of rare pulmonary diseases for many years. | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| The severity and prognosis of pulmonary alveolar proteinosis | The second severity and prognosis score of PAP (SPSP II) involves the range and density of lesion in chest CT of patients | From July 2020 to December 2021 |