autoimmune pulmonary alveolar proteinosis
MONDO:0012579Mondo
Findings
No curated finding names autoimmune pulmonary alveolar proteinosis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of a lipoproteinaceous substance in the distal air spaces which positively stains with periodic acid-Schiff (PAS).
Definition from the Mondo Disease Ontology (MONDO:0012579), read 2026-09-29. CC BY 4.0.
Features
26 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Anti-granulocyte-macrophage colony stimulating factor antibody positivityHPOHP:0020050
- 12 of 12 reported patients
- HypoxemiaHPOHP:0012418
- 1 of 1 reported patient
- Frequent (30% to 79% of cases)
- Restrictive ventilatory defectHPOHP:0002091
- 1 of 1 reported patient
- Frequent (30% to 79% of cases)
- Intraalveolar phospholipid accumulationHPOHP:0006517
- Very frequent (80% to 99% of cases)
- Abnormal circulating protein concentrationHPOHP:0010876
- Frequent (30% to 79% of cases)
- Abnormality of the upper respiratory tractHPOHP:0002087
- Frequent (30% to 79% of cases)
- Autoimmune antibody positivityHPOHP:0030057
- Frequent (30% to 79% of cases)
- ClubbingHPOHP:0001217
- Very rare (1% to 4% of cases)
- Frequent (30% to 79% of cases)
- CyanosisHPOHP:0000961
- Frequent (30% to 79% of cases)
- Decreased DLCOHPOHP:0045051
- Frequent (30% to 79% of cases)
- DyspneaHPOHP:0002094
- Frequent (30% to 79% of cases)
- Foam cellsHPOHP:0003651
- Frequent (30% to 79% of cases)
Show the remaining 14
- Increased circulating lactate dehydrogenase concentrationHPOHP:0025435
- Frequent (30% to 79% of cases)
- CoughHPOHP:0012735
- Occasional (5% to 29% of cases)
- CracklesHPOHP:0030830
- Occasional (5% to 29% of cases)
- Crazy paving patternHPOHP:0025391
- Occasional (5% to 29% of cases)
- Chest painHPOHP:0100749
- Very rare (1% to 4% of cases)
- FatigueHPOHP:0012378
- Very rare (1% to 4% of cases)
Where it sits
Other names
5 names
Resolves to: autoimmune pulmonary alveolar proteinosis
- Also called
- APAPautoimmune PAPidiopathic PAPidiopathic pulmonary alveolar proteinosisiPAP