PMC5491814_01
(a) Chest X-ray taken on Day 1 showing bilateral pulmonary infiltrates, especially in the right upper and right lower lung fields. (b) Chest X-ray taken on Day 5 showing progressive worsening of the pulmonary infiltrates, especially in the right upper and lower and left lower lung fields.
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Chest X-ray films showed right-sided predominant bilateral progressive pulmonary infiltrates (Fig. 1). However, during the subsequent four days, the patient's condition continued to deteriorate, with worsening of chest radiograph findings (Fig. 1, 2) and hypoxemia.
High-resolution computed tomography (HRCT) of the chest: (a, b) HRCT of the upper and lower lung fields on Day 1 showing bilateral multiple patchy alveolar opacities with a peripheral predominance. (c) HRCT on Day 14 showing bilateral worsening of alveolar opacities with air bronchograms in the lower lung fields. (d) HRCT of the lower lung fields on Day 34 showed gradual disappearance of the bilateral alveolar opacities with some residual abnormal lung shadows and tortuosity of bronchovascular shadows in the lower lung fields
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High-resolution computed tomography (HRCT) of the chest revealed bilateral and progressive multiple areas of subpleural and peribronchial patchy consolidation with ground glass opacities (Fig. 2). However, during the subsequent four days, the patient's condition continued to deteriorate, with worsening of chest radiograph findings (Fig. 1, 2) and hypoxemia.
The clinical course of the present case between Days 1 and 150. A bronchoscopic examination with a TBLB and BAL was performed on Day 4, and drug therapy of methylprednisolone pulse therapy and intravenous cyclophosphamide was started on Day 8 (dotted line: CRP, solid line: KL-6). KL-6: Krebs von den Lungen-6, IV-CY: intravenous cyclophosphamide, CsA: cyclosporine, PSL: prednisolone, BF: bronchoscopy
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There was no notable increase in the value of Krebs von den Lungen-6 (KL-6) (Table and Fig. 3). On the eighth hospital day, mechanical ventilation and intravenous methylprednisolone treatment (1,000 mg per day for three consecutive days) was started (Fig. 3). She was discharged on hospital day 73 when prednisolone was tapered to 20 mg/day (Fig. 3). She was fine 500 days later and did not develop any symptoms or signs of connective tissue disease (Fig. 3).
A transbronchial lung biopsy was performed for the right upper and lower lobes. (a-d) Tissue from the rtS2b. (a) The tissue showed granulation tissues (Masson bodies) formed in the alveolar ducts and adjacent alveoli. (b) The tissue showed hyalinous thickening of alveolar walls. (c, d) The tissue showed hyalinisation of the central core of the Masson body. (e-h) The tissue taken from the rtS2b. (e) The tissue showed granulation in the alveolar ducts and adjacent alveolar spaces, as well as accumulation of foamy cells in the alveolar spaces (lower centre). Relatively normal alveolar walls were noted in the adjacent area of organizing pneumonia. (f) A higher magnification of the rectangular area of (e); the alveolar duct was obliterated with granulation tissue and the alveolar duct walls were adhesive due to intervening granulation tissue (arrowed). (g) An elastic tissue stain showed granulation tissues and foamy cells in the alveolar ducts and alveolar spaces. (h) A higher magnification of the rectangular area of (g); an alveolar duct was obliterated due to the presence of granulation tissue with adhesion of alveolar duct walls (arrowed). Because of the findings (a-d) and (e-h), we made a histologic diagnosis of fibrosing organizing pneumonia (FOP). The findings of (a-h) showed an organizing pneumonia pattern. However, obiliterative alveolar duct fibrosis was not observed in previously reported cases of steroid-responsive idiopathic BOOP/COP (9, 17, 23, 24). Staining methods: a-f: Hematoxylin and Eosin staining; g, h: Weigert's elastic van Gieson stain. Magnification: a, e, gx4; c, x10; b, d, f, hx40. Bar=1 mm.
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A TBLB obtained two samples from the right upper lobe (rtB2b) and right lower lobe (rtB8b and rtB9b), and the TBLB specimens revealed histologic features of FOP (Fig. 4). One specimen from the right upper lobe showed hyalinisation and fibrosis of the central core of the Masson body, and another sample also from the right upper lobe showed granulation tissues in many terminal air spaces, mainly of the alveolar ducts, with adhesion of the alveolar duct walls due to intervening granulation tissues (obliterative alveolar duct fibrosis) and foamy cells in the alveolar spaces (Fig. 4e-h). None of the four lung specimens showed any malignant cells, granulomas, or significant infiltration of eosinophils or neutrophils (Fig. 4). On the seventh hospital day, a histopathologic diagnosis of FOP was rendered by the lung pathologist of this article (Fig. 4).