Summary and discussion
PAP is a rare disease characterized by interalveolar accumulation of
surfactant impairing gas exchange and resulting in progressive
respiratory insufficiency. [1] PAP has three distinct clinical
forms: primary, secondary and congenital [1,2]. Primary or
idiopathic PAP accounts for 90% of PAP case. Secondary PAP occurs as a
consequence of various underlying diseases [5]. Congenital PAP is a
heterogenous collection of disorders caused by homozygous mutation of
the genes encoding surfactant protein (SP)-B, SP-C and the ABCA3
transporter or by the absence of granulocyte/macrophage
colony-stimulating factor (GM-CSF) receptor [6].
The prevalence of PAP has recently been determined to be 6.87 ± 0.33 per
million in the general population and it affects men and women similarly
[7]. Autoimmune PAP represented 89.9% of cases and had a minimum
incidence and prevalence of 0.49 and 6.2 per million, respectively
[8].
Autoimmune PAP generally presents as progressive dyspnea of insidious
onset unless secondary infection is also present, in which case fever,
cough and rarely hemoptysis may also be seen. Chest CT typically shows
bilateral patchy or diffuse air-space consolidation or hazy ground-glass
opacity similar to pulmonary edema but without other features of left
heart failure. WLL is current gold standard of care, however, GM-CSF
augmentation strategies are also used in autoimmune PAP [9].
Management of PAP patients infected with COVID-19 is challenging. The
present case demonstrates that WLL may benefit the patient as it not
only removes the excessive surfactant, but also decreases the viral
load.