Updated Severity and Prognosis Score of Pulmonary Alveolar Proteinosis

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

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.

Study Overview

• Status: Completed
• Conditions: Pulmonary Alveolar Proteinosis
• Intervention / Treatment: Other: Assesssing the severity of pulmonary alveolar proteinosis

Detailed Description

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.

• Study Type: Observational
• Enrollment (Actual): 231

Contacts and Locations

Study Locations

• China
  • Shanghai
    • Shanghai, Shanghai, China, 200433
      • Shanghai Pulmonary Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: N/A
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

The patients had be diagnosed with autoimmune pulmonary alveolar proteinosis. Every patient had a complete medical history, chest HRCT, pulmonary function and arterial blood gas analysis.

Description

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.

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Retrospective

Number of groups / cohorts

3

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Shanghai Pulmonary Hospital; 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; Three severity scores will be used to assess the severity of patients with pulmonary alveolar proteinosis.
Peking Union Medical College Hospital; 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; Three severity scores will be used to assess the severity of patients with pulmonary alveolar proteinosis.
Nanjing Drum Tower Hospital; 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?

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

Collaborators and Investigators

• Sponsor: Shanghai Pulmonary Hospital, Shanghai, China
• Investigators: Principal Investigator: Xinlun Tian, Dr, Peking Union Medical College Hospital; Principal Investigator: Yonglong Xiao, Dr, The Affiliated Nanjing Drum Tower Hospital of Nanjing University Medical School

Study record dates

Study Major Dates

• Study Start (Actual): January 1, 2020
• Primary Completion (Actual): December 31, 2021
• Study Completion (Actual): December 31, 2021

Study Registration Dates

• First Submitted: August 11, 2020
• First Submitted That Met QC Criteria: August 17, 2020
• First Posted (Actual): August 18, 2020

Study Record Updates

• Last Update Posted (Actual): March 9, 2022
• Last Update Submitted That Met QC Criteria: March 5, 2022
• Last Verified: March 1, 2022

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Respiratory Tract Diseases; Lung Diseases; Pulmonary Alveolar Proteinosis
• Other Study ID Numbers: 2020073195

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: Undecided

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No