PMC3919965_01
Radiologic findings of IgG4-related disease of lung and pleura. (A, C) Chest X-ray (taken 1 year ago) and computed tomography (taken 6 months ago) demonstrate pleural effusion with patchy ground-glass opacity lesions in the right lung. (D) Subsequent computed tomography reveals newly developed, diffuse patchy lesions with pleural thickening and fissural nodularity in both lung, which radiologically suggests mesothelioma or pleural seeding from the lung cancer. (B) These lesions show hot uptake on positron emission tomography. (E) After steroid therapy, the lesions of both lobes are improved with residual small nodule in the left pleura.
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F1
On the follow-up examinations, the right pleural effusion on chest X-ray (Figure 1A, one year ago) and the patchy lesions of the right lung on computed tomography (Figure 1C, 6 months ago) were found. Multiple nodules and diffuse pleural thickening in the left lower lung field were newly developed 3 months ago (Figure 1D). Positron emission tomography also disclosed hot uptake on these new nodules (Figure 1B, arrows) and no identifiable lesions on the other organs including pancreas, thyroid and salivary gland.
Microscopic findings of IgG4-related disease of lung and pleura (A) Scanned view of the lesion displays pleural thickening and subpleural fibrosis. (B-D, F, G) Infiltrate cells are mainly composed of lymphocytes and plasma cells in the background of dense fibrosis. Dispersed giant cells are also noted in some areas (C, F, and G, H&E stain; B and D, Elastic stain; B, x12.5; C and D, x200; E, x100; F, x40; G, x400). (E) Small blood vessels are infiltrated by lymphoplasma cells without fibrinoid necrosis or granuloma formation. Immunohistochemical stainings for IgG (H) and IgG4 (I) reveal increased ratio of IgG4/IgG positive plasma cells, estimated at about 40% (H and I, x200).
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F2
Microscopically, visceral pleura and alveolar interstitium were thickened with dense cellular infiltration and fibrosis (Figure 2A). Infiltrates were mainly composed of lymphoplasma cells without cytological atypia (Figure 2F, G). Many multinucleated giant cells of foreign-body type were noted near the degenerated elastic layer of the visceral pleura on the inflammatory and fibrotic background (Figure 2B-D). Some blood vessels showed angiocentric infiltration of lymphoplasma cells, but there was no evidence of destructive changes such as neutrophilic infiltration and fibrinoid necrosis in the vessel walls (Figure 2E). Binding Site, Bermingham, UK) revealed increased proportion of IgG4-positive plasma cells, estimated at about 40% (Figure 2H, I). Based on the histological and immunohistochemical findings, IgG4-related disease was confirmed, and increased serum level of IgG4 (over than 201 mg/dL) were subsequently checked.