J Lung Cancer.  2010 Dec;9(2):103-105. 10.6058/jlc.2010.9.2.103.

IgG4-Related Lung Disease Presenting as a Consolidative Mass: A Case Report

• Park HY
• Han J
• Kang G
• Yi CA
• Chung MP

• KMID: 2199917
• DOI: http://doi.org/10.6058/jlc.2010.9.2.103

Abstract

Immunoglobulin G4 (IgG4)-related sclerosing disease involving the lung is a rare condition, and this is characterized by an elevated serum IgG4 level, fibrotic inflammation with numerous IgG4-positive plasma cells and a response to steroid therapy. We present here a case of pulmonary IgG4-related disease in a 75-year-old man who presented with cough and yellowish sputum for the previous 3 months. The chest images showed a consolidative mass in the right lower lobe that suggested mucinous bronchioloalveolar carcinoma. The wedge resected specimen revealed an ill-defined gray-tan, firm lesion. Microscopically, the lesion showed a diffuse lymphoplasmacytic infiltration with irregular fibrosis in the alveolar interstitium and bronchovascular bundles. There were numerous IgG4-positve plasma cells and these cells were diffusely distributed. The serum IgG4 level was elevated on the postoperative check-up (249 mg/dL). After corticosteroid therapy for 7 months, the patient's symptoms and radiologic abnormalities were improved.

Keyword

Autoimmune disease; IgG4; Interstitial lung disease; Plasma cells; Lung neoplasms

MeSH Terms

Adenocarcinoma, Bronchiolo-Alveolar
Aged
Autoimmune Diseases
Cough
Fibrosis
Humans
Immunoglobulin G
Immunoglobulins
Inflammation
Lung
Lung Diseases
Lung Diseases, Interstitial
Lung Neoplasms
Mucins
Plasma Cells
Sputum
Thorax
Immunoglobulin G
Immunoglobulins
Mucins

Figure

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  Fig. 1. (A) The chest X-ray shows parenchymal consolidation at the right costophrenic angle with pleural effusion. (B, C) The chest computed tomography scans reveal air space consolidation and ground-glass attenuations.

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  Fig. 2. (A, B) Scanning views of the VATS wedge resected specimen show a subpleural patchy mononuclear cells infiltration. (C) The low power view shows a dense lymphoid cell infiltration with fibrosis in the alveolar interstitium and bronchovascular bundles (H&E satin, ×40). (D) The lesion is composed of mature plasma cells and some lymphocytes (H&E stain, ×200). (E) Phlebitis is also present in the interlobular septal vein (H&E stain, ×100). (F) Many plasma cells are positive for IgG4 (×400).

Reference

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