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Case Reports
. 2013 Mar-Apr;20(2):87-9.
doi: 10.1155/2013/619453.

Immunoglobulin G4-related disease mimicking asthma

Hiroshi Sekiguchi  1 , Ryohei Horie, Timothy R Aksamit, Eunhee S Yi, Jay H Ryu
Affiliations
Case Reports

Immunoglobulin G4-related disease mimicking asthma

Hiroshi Sekiguchi et al. Can Respir J. 2013 Mar-Apr.

Abstract
in English, French

Immunoglobulin (Ig) G4-related disease (also known as 'IgG4-related sclerosing disease', 'IgG4-related systemic disease' or 'hyper-IgG4-disease') is a recently recognized systemic fibroinflammatory disease associated with IgG4-positive plasma cells in tissue lesions. IgG4-related disease was initially described as autoimmune pancreatitis, but it is now known to affect virtually any organ. The authors describe a patient presenting with multi-organ manifestations, including airway inflammation mimicking asthma, pulmonary parenchymal infiltrates, intrathoracic lymphadenopathy, submandibular gland swelling and a kidney mass.

La maladie liée aux immunoglobulines (Ig) G4 (aussi appelée maladie sclérosante liée aux IgG4 ou syndrome d’hyper-IgG4) est une maladie fibro-inflammatoire systémique récemment reconnue qui s’associe à des cellules plasmatiques positives aux IgG4 contenues dans les lésions tissulaires. La maladie liée aux IgG4 a d’abord été décrite comme une pancréatite auto-immune, mais on sait maintenant qu’elle peut toucher à peu près n’importe quel organe. Les auteurs décrivent le cas d’un patient atteint de manifestations multiorganiques, y compris une inflammation des voies aériennes imitant l’asthme, des infiltrats pulmonaires parenchymateux, une lymphadénopathie intrathoracique, un œdème des glandes sous-maxillaires et une masse rénale.

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Figures

Figure 1)
Figure 1)
Computed tomography (CT). A Neck CT demonstrating enlarged submandibular glands. B Chest CT demonstrating hilar and mediastinal lymphadenopathy. C Chest CT demonstrating a 2.5 cm spiculated infiltrate in the left upper lobe. D Abdominal CT demonstrating a 2.5 cm low-attenuation mass in the left kidney
Figure 2)
Figure 2)
Microscopic examination. A Submandibular gland biopsy demonstrating the features of chronic sialadenitis (hematoxylin and eosin stain, original magnification ×400). B Increased immunoglobulin (Ig) G4-positive plasma cells within the chronic inflammatory infiltrate in submandibular gland biopsy (IgG4 immunostain, original magnification ×400). C Bronchoscopic biopsy demonstrating chronic inflammatory infiltrate and thickened basement membrane in bronchial mucosa (hematoxylin and eosin stain, original magnification ×200). D Increased IgG4-positive plasma cells within the inflammatory infiltrate in bronchial mucosa (IgG4 immunostain, original magnification ×200)
See this image and copyright information in PMC

References

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