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Rapidly Progressive Pulmonary Alveolar Proteinosis Following Cryptococcal Infection: Case Report and Literature
Samuel Cartmel Brookes1,2, Alexandra Sadler1, Alexander Troelnikov3,4,5
1Department of Thoracic Medicine Royal Adelaide Hospital Adelaide South Australia Australia.
Abstract:
Pulmonary alveolar proteinosis (PAP) is a rare disease caused by accumulation of sediment within alveoli. Cryptococcosis a fungal infection typically presenting with central nervous system (CNS) and pulmonary disease. Granulocyte-macrophage colony-stimulating factor antibodies are associated with PAP and elevated risk of cryptococcosis. The usual interval from cryptococcal infection to the onset of PAP spans several years. Here, we describe a case of a 24-year-old Aboriginal Australian woman with no prior medical history, who presented with seizures from CNS cryptococcosis, and subsequently developed rapidly progressive hypoxic respiratory failure secondary to autoimmune-PAP within weeks of initial presentation. The rate and degree of respiratory failure necessitated urgent bilateral whole lung lavage (WLL) whilst on venovenous-extracorporeal membrane oxygenation. Our report hopes to increase recognition of PAP in the Australian population, document the utility and risks of bilateral WLL in the critically unwell patient and provide an updated literature review of PAP and cryptococcal infection.
Insights
This case study highlights a rare instance of autoimmune pulmonary alveolar proteinosis (PAP) rapidly developing after cryptococcal infection in a young Aboriginal Australian woman. It emphasizes the need for prompt recognition and intervention for this severe respiratory condition.
Area of Science:
- Pulmonary Medicine
- Infectious Diseases
- Immunology
Background:
- Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by surfactant accumulation in alveoli.
- Cryptococcosis is a fungal infection often affecting the central nervous system and lungs.
- Autoimmune PAP is linked to granulocyte-macrophage colony-stimulating factor antibodies, also associated with increased cryptococcosis risk.
Purpose of the Study:
- To report a unique case of rapidly progressive autoimmune PAP following CNS cryptococcosis.
- To underscore the importance of recognizing PAP in the Australian population.
- To review the management and risks of bilateral whole lung lavage (WLL) in critically ill patients.
Main Methods:
- Case report of a 24-year-old Aboriginal Australian woman.
- Description of clinical presentation, diagnostic workup, and treatment.
- Literature review on PAP and cryptococcal infections.
Main Results:
- The patient presented with seizures due to CNS cryptococcosis, followed by rapid hypoxic respiratory failure from autoimmune PAP within weeks.
- Urgent bilateral whole lung lavage (WLL) was performed while on venovenous-extracorporeal membrane oxygenation.
- The case illustrates an unusually short interval between cryptococcal infection and PAP onset.
Conclusions:
- Autoimmune PAP can develop rapidly after cryptococcosis, presenting a diagnostic and therapeutic challenge.
- Bilateral WLL can be a critical intervention for severe PAP in ECMO-supported patients, despite associated risks.
- Increased awareness of PAP, particularly in specific populations like Aboriginal Australians, is crucial.
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