PMC8349009_01
Radiological and bronchoscopic findings. Chest CT on admission showed subpleural consolidation and GGOs predominantly in the lower lobes accompanying traction bronchiectasis and marked volume loss (A, B). Bronchoscopy showed a white ulcerative lesion on the membranous portion of the trachea (C). Axial short-tau inversion recovery image of the thigh MRI showed a high signal in the fascia of the bilateral femoral flexor muscles (white arrowheads) (D). Three months after the start of treatment, the consolidation, GGOs, tracheal ulcer, and fasciitis improved (E-H).
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Chest computed tomography (CT) showed an increase in subpleural consolidation and ground-glass opacities (GGOs) predominantly in the lower lobes accompanying traction bronchiectasis and marked volume loss (Fig. 1A and B). Bronchoscopic observation of the lumen revealed a white ulcerative lesion on the membranous portion of the trachea (Fig. 1C). Thigh magnetic resonance imaging (MRI) showed a high signal in the fascia of the bilateral femoral flexor muscles, but no high signal within the muscle was evident (Fig. 1D).
Pathological findings. The tracheal ulcer showed marked squamous metaplasia without findings of malignancy from granulomatous diseases at low power (A), and mild lymphocytes, plasma cells, and neutrophilic infiltration in the tracheal mucosa at high power (hematoxylin and eosin stain) (B). Transbronchial cryobiopsy specimens obtained from the left lower lobe of the lung showed severe alveolar fibrosis with alveolar collapse (hematoxylin and eosin stain) (C) and elastic fibres with a high degree of contraction from the alveolar region to the interstitial region (Elastica van Gieson stain) (D).
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Pathological evaluation of the tracheal ulcer showed squamous metaplasia suggestive of a post-ulceration occurrence without findings of malignancy from granulomatous diseases (Fig. 2A and B). Transbronchial lung cryobiopsy specimens obtained from the left lower lobe of the lung showed marked alveolar fibrosis with severe alveolar collapse (Fig. 2C and D). Although mild infiltration with lymphocytes and plasma cells were observed in some alveolar areas and non-specific interstitial pneumonia was presumed to be the basal histological pattern, a sufficient evaluation of the overall picture could not be achieved and the pathological diagnosis of unclassifiable interstitial pneumonia was finally made (Fig. 2C and D). Evaluation by CT, MRI, and bronchoscopy performed 3 months after the start of treatment showed that consolidation, GGOs, fasciitis, and tracheal ulcer had all improved (Fig. 2E-G).