case_images: PMC11810437_01
case_images: CT chest axial slices from initial presentation through to definitive management with whole lung lavage. (A) initial presentation with cryptococcal pneumonia with predominant left lower lobe consolidative change. (B) two weeks after initial presentation with progressive bilateral ground glass changes and early interlobular septal thickening which correlated with high CMV titre in BAL fluid. (C) Six weeks after initial presentation with progressive, confluent bilateral ground glass change and interlobular septal thickening in keeping with crazy paving pattern. (D) Four days post-whole lung lavage with marked radiographic improvement.
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case_images: Computed tomography (CT) chest identified asymptomatic left lower lobe consolidation (Figure 1A). CT chest demonstrated progressive consolidation in the upper lobes and widespread ground glass change (Figure 1B). A repeat CT chest demonstrated progressive ground glass opacities with inter-lobular septal thickening, the classical crazy-paving pattern (Figure 1C). Right lung was selected for lavage first due to the greater degree of infiltrate seen on CT chest (Figure 1C). 1-L aliquots of warmed normal saline were instilled from a 50 cm height above the thorax via passive instillation and both afferent and efferent limbs were clamped. The patient had rapid response to WLL, transitioning from HFNC to maintaining SpO2 95% on an FiO2 of 21% post-ECMO decannulation (Corresponding CT:Figure 1D).
case_images: Flow cytometry histogram plot of CD14+ monocytes with either unstimulated (unstim), stimulated (stim) with GM-CSF 10 ng/mL, stained with phosphoSTAT5, in the presence of either patient serum (Pt, replicates) or healthy control (HC) serum (single replicate of two separate subjects), demonstrating complete neutralisation of GM-CSF by patient serum.
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case_images: Expression of pSTAT5 relative to positive and negative controls was measured to determine the presence of GM-CSF antibodies, confirming the diagnosis of autoimmune-PAP (Figure 2).
case_images: Whole lung lavage fluid specimens. Jug filled via efferent limb and aliquoted into pots with excess fluid discarded. This allows visual estimation of sediment volume and analysis of colour and clarity of lavage fluid. (A) sequential lavage samples from left lung (front) and right lung (back) from 1st litre (left) to final litre (right). (B) juxtaposes initial fluid sample (bottom) with final fluid sample (top). Red arrow indicates sediment: Fluid interface.
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case_images: The efferent limb was unclamped, and drainage volume, colour and sediment were recorded (Figure 3).
case_images: CT chest axial slices. (A) 8-weeks post-bilateral whole lung lavage demonstrating recurrence of bilateral ground glass opacities. (B) 5-months post-right lung lavage with sustained radiographic resolution of ground glass changes bilaterally.
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case_images: Left lung lavage was performed 3 months later due to recurrent radiographic ground glass opacities (Figure 4A) and symptomatic dyspnoea. PAP has since remitted on follow up CT chest after a further 5 months (Figure 4B).