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Pulmonary Alveolar Proteinosis in Greece-Türkiye-Cyprus: Answers in a Real-Life Comparison
Spyros A Papiris1, Efsun Gonca Uğur Chousein2, Maria Kallieri3
1Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Respirology (Carlton, Vic.)
|July 15, 2026
Summary
This study compared autoimmune Pulmonary Alveolar Proteinosis (aPAP) patient cohorts from Greece, Türkiye, and Cyprus. Sole iGM-CSF treatment proved non-inferior, but pulmonary fibrosis negatively impacts outcomes.
Area of Science:
- Pulmonology
- Rare Diseases
- Immunology
Background:
- Pulmonary alveolar proteinosis (PAP) is a rare condition characterized by excessive surfactant accumulation in the alveoli.
- Autoimmune PAP (aPAP) is the most common subtype, often associated with anti-GM-CSF antibodies.
Purpose of the Study:
- To compare outcomes of aPAP patients across Greece, Türkiye, and Cyprus over a 20-year period.
- To evaluate the efficacy of different treatment strategies, including whole lung lavage (WLL) and interferon-gamma (i-GM-CSF).
Main Methods:
- Retrospective chart review of 130 PAP patients (39 Greece, 87 Türkiye, 4 Cyprus).
- Analysis of anti-GM-CSF antibody titers in aPAP patients.
- Stratification of aPAP patients by treatment groups: no WLL/i-GM-CSF, WLL alone, i-GM-CSF alone, and both.
Main Results:
- 115 patients had aPAP; 40% were male, with a median age of 40 years at diagnosis.
- Non-survivors exhibited higher rates of cardiovascular disease, long-term oxygen therapy (LTOT), and pulmonary fibrosis.
- Sole i-GM-CSF treatment demonstrated significant improvement in DLCO%, oxygen saturation, and 6-minute walk test (6MWT).
- Pulmonary fibrosis was associated with a diminished treatment response and poorer survival across all therapy groups.
Conclusions:
- This study represents the first real-world comparison of aPAP cohorts from three countries.
- Sole i-GM-CSF treatment is a viable and effective option for aPAP, demonstrating non-inferiority to combination therapies.
- The presence of pulmonary fibrosis is a critical factor negatively influencing treatment efficacy and patient survival in aPAP.
Keywords:
autoantibodies against granulocyte macrophage‐colony stimulating factorautoimmune pulmonary alveolar proteinosisinhaled granulocyte macrophage‐colony stimulating factorwhole lung lavageMore Related Videos
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