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Case Reports
. 2018 Jan 1;57(1):43-48.
doi: 10.2169/internalmedicine.9026-17. Epub 2017 Oct 16.

IgG4-related Lung Pseudotumor and Pleural Inflammation with Autoimmune Hepatitis

Kazunori Nagashima  1 , Itsuki Sano  2 , Tomoe Kobayashi  1 , Kazunori Eto  1 , Kosuke Nagai  1 , Ryusuke Ninomiya  3 , Akira Suzuki  4   5 , Yoshihiro Oohata  3 , Kouhei Konishi  1 , Tsuyoshi Nakano  3 , Fumiyasu Yamamoto  1
Affiliations
Case Reports

IgG4-related Lung Pseudotumor and Pleural Inflammation with Autoimmune Hepatitis

Kazunori Nagashima et al. Intern Med. .

Abstract

A 63-year-old man was admitted to our department following a secondary medical examination. Blood tests showed high levels of liver enzymes, IgG, IgG4, and antinuclear antibody. Computed tomography showed tumors in the bilateral lower lobes of the lungs and pleural thickening. After pleural and liver biopsy procedures, he was conclusively diagnosed with IgG4-related lung pseudotumor and pleural inflammation with autoimmune hepatitis. We started treatment with prednisolone 40 mg/day, and chest radiograph and blood tests showed signs of improvement. This was a rare case that suggested an association between IgG4-related disease and autoimmune hepatitis.

Keywords: IgG4; autoimmune hepatitis; lung pseudotumor.

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Figures

Figure 1.
Figure 1.
Imaging findings before treatment. (a) Chest radiograph reveals consolidation in the right lower lung field and blunted right costophrenic sulcus. (b) (c) CT reveals 35-mm tumors at S10 of both lungs, bilateral thickening of the pleura, and right-sided pleural effusion (b: mediastinal window; c: lung window). (d), (e) PET-CT reveals increased the FDG uptake in the lung tumor and pleura (SUVmax: 5.87).
Figure 2.
Figure 2.
Pathologic findings of the right pleura and liver (a-c: pleura; d, e: liver). (a) The right visceral pleura has lymphoplasmacytic infiltration and marked fibrosis comprising mostly lymphocytes and plasma cells [Hematoxylin and Eosin (H&E) staining, ×100]. (b) (c) IgG4 immunostaining shows an IgG4/IgG-positive plasma cell ratio of ≥40% and ≥10 IgG4-positive plasma cells per HPF, which is more clearly shown by double immunostaining with IgG4 and IgG [b: IgG4 immunostaining, ×400; c: Double immunostaining with IgG4 (brown) and IgG (red), ×400]. (d) A liver biopsy reveals periportal inflammatory cell infiltrate and interface hepatitis comprising mostly plasma cells. No rosette formation, fat deposition, fibrosis, or inflammation is observed around the bile duct (H&E staining, ×100). (e) IgG4 immunostaining reveals ≤9 per HPF IgG4-positive plasma cells (IgG4 immunostaining, ×400).
Figure 3.
Figure 3.
Imaging findings at one month after treatment. (a) Chest radiograph reveals the resolution of the consolidation in the right lower lung field and the blunted right costophrenic sulcus. (b) CT imaging reveals tumor shrinkage and reduced bilateral thickening of the pleura and pleural effusion.
Figure 4.
Figure 4.
Time course of treatment.
See this image and copyright information in PMC

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