Pulmonary alveolar proteinosis: another autoimmune disease associated with sarcoidosis?

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Eda Burcu Boerner
Ulrich Costabel
Thomas Ernst Wessendorf
Dirk Theegarten
Martin Hetzel
Marjolein Drent
Francesco Bonella


Pulmonary alveolar proteinosis (PAP), sarcoidosis, whole lung lavage


Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of surfactant phospholipids and lipoproteins within the alveoli.  Here we report on a female patient who was diagnosed with autoimmune PAP and successfully treated with whole lung lavage (WLL). 15 months after PAP diagnosis the patient developed marked fatigue. Additional tests revealed the diagnosis of sarcoidosis. We can only speculate that PAP and sarcoidosis in our patient are linked to each other based on the fact that other autoimmune disorders have also been associated with sarcoidosis.


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