Related Experiment Video
Updated: Jan 11, 2026
10:21
Author Spotlight: Exploring the Role of Inflammation in the Co-occurrence of Primary Sjogren's Syndrome and Lung Adenocarcinoma
Published on: September 20, 2024
711
Secondary Pulmonary Alveolar Proteinosis
Jayleigh Lim1, Niamh Boyle1,2, Cormac McCarthy1,2
1Department of Respiratory Medicine, St Vincent's University Hospital, Dublin, Ireland.
Seminars in Respiratory and Critical Care Medicine
|November 10, 2025
Summary
Secondary pulmonary alveolar proteinosis (sPAP) is a rare lung disorder caused by underlying diseases, often hematological. Treatment focuses on the primary condition, as sPAP has a poor prognosis.
Area of Science:
- Pulmonology
- Rare Diseases
- Hematology
Background:
- Pulmonary alveolar proteinosis (PAP) involves surfactant accumulation in alveoli, causing respiratory issues.
- Secondary PAP (sPAP) arises from conditions impairing alveolar macrophages, commonly hematological disorders.
- sPAP represents 4% of PAP cases, affecting adults with a worse prognosis than autoimmune PAP (aPAP).
Purpose of the Study:
- To review the presentation, prognosis, and treatment of secondary pulmonary alveolar proteinosis (sPAP).
Main Methods:
- Review of existing literature on sPAP.
- Analysis of clinical presentation, diagnostic findings, and therapeutic outcomes.
- Comparison of sPAP characteristics with autoimmune PAP (aPAP).
Main Results:
- sPAP presents with nonspecific symptoms, often linked to the underlying disease.
- Diagnosis requires serological, radiological, and bronchoscopic evaluation.
- Radiological findings in sPAP may differ from aPAP, showing diffuse ground glass opacities.
Conclusions:
- The prognosis for sPAP is significantly poorer than aPAP, with a median survival under 20 months.
- Effective treatment for sPAP relies on managing the underlying condition.
- Whole lung lavage may offer benefits in select sPAP cases.
Related Concept Videos
01:20Chronic Obstructive Pulmonary Disease-II: Pathophysiology
4.2K
Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
Chronic Inflammation
4.2K
01:29Pneumonia II: Pathophysiology
2.5K
The pathophysiology of pneumonia involves the following steps:
2.5K
01:30Pulmonary Hypertension: Classification and Pathogenesis
552
Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
There are various classifications for PH, each relating to different underlying causes and also...
552
01:28Pulmonary Tuberculosis II
1.4K
Tuberculosis, or TB, is a bacterial infectious disease caused by Mycobacterium tuberculosis. While its primary impact is on the lungs, leading to pulmonary tuberculosis, it can also affect various other organs, a condition referred to as extrapulmonary tuberculosis.
Here is a detailed explanation of its pathophysiology:
Transmission: The process begins when a person inhales droplet nuclei containing M. tuberculosis. These are typically released into the air when an individual with pulmonary or...
Here is a detailed explanation of its pathophysiology:
Transmission: The process begins when a person inhales droplet nuclei containing M. tuberculosis. These are typically released into the air when an individual with pulmonary or...
1.4K
01:25Pulmonary Function Tests
723
Pulmonary Function Tests (PFTs)
Pulmonary Function Tests are crucial diagnostic tools for assessing respiratory function, particularly in patients with chronic respiratory disorders. They comprehensively evaluate lung volumes, ventilatory function, breathing mechanics, diffusion, and gas exchange. These tests help diagnose pulmonary diseases and play a significant role in monitoring disease progression, evaluating disability, and assessing response to therapy.
PFTs involve using a spirometer, a...
Pulmonary Function Tests are crucial diagnostic tools for assessing respiratory function, particularly in patients with chronic respiratory disorders. They comprehensively evaluate lung volumes, ventilatory function, breathing mechanics, diffusion, and gas exchange. These tests help diagnose pulmonary diseases and play a significant role in monitoring disease progression, evaluating disability, and assessing response to therapy.
PFTs involve using a spirometer, a...
723
01:20COPD: Pathogenesis and Clinical Features
1.7K
Chronic obstructive pulmonary disease (COPD) is a group of lung conditions that progressively worsen over time, including chronic bronchitis and emphysema. This cluster of diseases collectively leads to a gradual and irreversible decline in lung function over time.
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
1.7K