PMC6955142_01
An axial CT chest image of patient 1 demonstrating long-standing bilateral fibrosis and nodular opacities with honey combing and traction bronchiectasis affecting the left lung.
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HRCT findings were consistent with usual interstitial pneumonia (UIP) according to the American Thoracic Society (ATS) and the European Respiratory Society (ERS) guidelines (Figure 1).
A sural nerve biopsy showing subcutaneous vessels with leukocytoclasis, fibrinoid necrosis, and a perivascular lymphocytic infiltrate (H&E).
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Subsequent nerve conduction studies revealed a severe length-dependent sensorimotor axonal neuropathy, while a sural nerve biopsy demonstrated fibrinoid necrosis and a lymphocytic infiltrate but no evidence of granulomas (Figure 2).
PMC6955142_02
(a) CT pulmonary angiogram of patient 2 reveals bilateral calcified pleural plaques, subpleural fibrosis, paraseptal emphysematous change, and a small region of honey combing in the right lower lobe base laterally. (b) CT chest six months later revealing interval increase in the size of the consolidation, resolution of the right upper lobe spiculated lesions, and stable pulmonary fibrosis.
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Prior to admission, his colonoscopy was normal whilst a CXR and CT pulmonary angiogram (CTPA) revealed several small (up to 8 mm) spiculated lesions in the right upper lobe, bilateral calcified pleural plaques, bilateral subpleural fibrosis, bilateral subpleural emphysematous change, and a small region of honey combing in the right lower lobe base laterally (Figure 3(a)). A CT chest six months later revealed resolution of the spiculated lesions of the right upper lobe, but increase in subpleural fibrosis parenchyma was noted in the previous scan (Figure 3(b)).
Left lung biopsy of patient two showed subpleural proliferative fibroblastic foci and a nodular infiltrate of plasma cells, histiocytes, and multinucleated giant cells.
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There were changes of emphysema with plentiful intra-alveolar pigmented histiocytes (Figure 4).