PMC5329820_01
(A) Chest computed tomography shows an irregularly narrowed bronchus wall with a small polypoid mass attached to the right upper lobar (RUL) bronchus. (B) Peribronchial consolidation with cystic and tubular bronchiectasis, mucoid impactions and multiple centrilobular nodules are seen with patchy ground glass opacities in RUL area. (C) Luminal narrowing is shown due to irregular calcified wall thickening in left upper and lower lobar bronchi. (D) There is associated obstructive pneumonitis with some infiltrative opacities involving lingular segments of left upper lobe and superior segment of left lower lobe.
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Computed tomography (CT) of the chest showed an irregularly narrowed bronchus wall with a small polypoid mass attached to the right upper lobar bronchus, and some infiltrative opacities in both lower lobes (Figure 1).
(A) Bronchoscopy revealed exophytic hypervascular nodular projections accompanied by erythematous inflammation with a white mucosal patch in the right upper lobar bronchial orifice. (B) Two endobronchial nodules are also observed at the orifice of right lower lobe. (C) The lumens of the bronchi are extremely narrowed due to multiple variable sized nodules in the orifice of the lingular segment of the left upper lobe and left lower lobar basal segments.
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Flexible bronchoscopy revealed exophytic hypervascular masses accompanied by erythematous inflammation with a white mucosal patch in the right upper lobar bronchial orifice (Figure 2).
Pathologic findings.. (A) The bronchoscopic tissue shows an amorphous material deposit in endobronchial area (X40). (B) The pinkish material is associated with multinucleated giant cells (X100). (C) The material shows a red color at Congo-red stain (X20). (D) These deposits exhibit a green birefringence under polarized microscopy on Congo red stain (X400).
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These deposits showed a green birefringence under polarized microscopy on Congo red stain, a finding characteristic of immunoglobulin light chain amyloid fibrils (Figure 3).