Journal Article

246 A Case of Primary Pulmonary IgG4 Disease

Elham Yousefi and Maksim Agaronov

in American Journal of Clinical Pathology

Published on behalf of American Society for Clinical Pathology

Volume 149, issue suppl_1, pages S105-S105
Published in print January 2018 | ISSN: 0002-9173
Published online January 2018 | e-ISSN: 1943-7722 | DOI:
246 A Case of Primary Pulmonary IgG4 Disease

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A 66-year-old woman with a history of invasive lobular carcinoma of the right breast, post mastectomy and chemoradiation therapy, underwent chest CT scan as part of the follow-up for her cancer treatment. A solitary subpleural pulmonary nodule measuring 1.5 cm in greatest dimension was identified in the right upper lobe that was not present in the previous chest CT scan. A wedge resection was performed.


A wedge resection of the right upper lobe was received in the pathology department. Sectioning of the specimen revealed a 1.2 x 1 x 0.5 cm tan-pink firm rubbery nodule in the subpleura. Histologic evaluation of the lesion revealed a well-circumscribed non-encapsulated nodule composed of dilated blood vessels with sclerotic stroma showing marked lymphoplasmacytic infiltration. The vessels show areas with obliterative phlebitis. Immunohistochemical evaluation for AE1/AE3, CD138, kappa/lambda, and IgG/IgG4 was performed.


The lesion was diffusely negative for cytokeratin stain and consisted of polyclonal plasma cells predominantly of IgG4 type (IgG4:IgG ratio >50%). The number of IgG4 plasma cells is 50–60/hpf. This lesion was diagnosed as IgG4-related disease of the lungs.


IgG4 related pulmonary disease is a rare and difficult diagnosis mimicking malignancy on radiology as manifested by our case, which demonstrates a primary pulmonary IgG4 disease with no systematic manifestations that was resected with a suspicion of metastatic breast carcinoma.

Journal Article.  0 words. 

Subjects: Medical Skills ; Pathology

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