PMC9989416_01
(a) Chest X-ray taken in November 2012 during the patient's first hospital visit shows stage II (bilateral hilar lymphadenopathy with pulmonary infiltration) disease in chest X-ray staging. (b) Computed tomography (CT) of the chest shows bilateral hilar and mediastinal lymphadenopathy. (c) The CT scan also shows ground-glass opacities and reticular shadows distributed predominantly in both lower lobes.
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Chest radiography revealed bilateral hilar lymphadenopathy and reticular shadows in the lower lung fields (Figure 1(a)). CT of the chest showed bilateral hilar and mediastinal lymphadenopathy (Figure 1(b)), and ground-glass opacities and reticular shadows distributed predominantly in both lower lobes (Figure 1(c)).
(a) A biopsy specimen obtained from the right B8a and subjected to histopathological analysis shows noncaseating epithelioid granulomas (arrows, x100). (b) A granuloma specimen under x400 magnification. (c) Magnified pictures (x400) of a rectangle within the left upper panel are shown. Alcian blue positive intraluminal fibrosis with mural incorporation is observed (left lower panel).. EMG, elastica masson-goldner; H&E, hematoxylin and eosin staining.
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Histopathological examination of the transbronchial lung biopsy (TBLB) specimens (right B3b, right B8a) revealed noncaseating epithelioid granulomas in the perivascular connective tissue with lymphocyte infiltration, which was consistent with sarcoidosis (Figure 2(a) and (b)). Alcian blue-positive intraluminal fibrosis with mural incorporation was also observed (Figure 2(c)).
A summary of the patient's clinical course.. The decline in forced vital capacity accelerated with the appearance of joint involvement.. anti-CCP antibody, anti-cyclic citrullinated peptide antibody; BAL, bronchoalveolar lavage; PSL, prednisolone; SASP, salazosulfapyridine; TBLB, transbronchial lung biopsy.
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Due to a progressive decline in FVC (>200 mL/year) accompanied by a deterioration of the radiological findings, 25 mg/day oral prednisolone (PSL) was initiated in November 2017 followed by gradual tapering (Figure 3). Gradual progression of dyspnea had been accompanied by a total of 900 mL reduction in FVC during the 4 years until August 2020 (Figure 3). Although home oxygen therapy was introduced in May 2021, FVC was maintained in follow-up pulmonary function tests with successful tapering of PSL to 10 mg/day (Figure 3).
Honeycomb changes gradually developed on a series of computed tomography scans over 5 years of observation.
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Concordantly, honeycomb changes gradually developed on a series of CT scans over 5 years (Figure 4).