대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

IgG4-related lung diseas

Radiologic Findings

Fig 1. Chest PA shows cavirtary masslike opacity in left hilar area.
Fig 2-3. CT scans shows irregular mass with internal cavitary area and partly spiculated margin in left lower lobe superior segment.

Brief Review

IgG4-related disease is a systemic fibro-inflammatory disease characterized by extensive IgG4-positive plasma cell infiltration of various organs. Patterns of IgG4-related disease involving thoracic organs have been classified as parenchymal, airway, pleural and mediastinal involvements.
Radiologic findings of IgG4-RLD can be categorized into four major features: (i) solid nodular densities; (ii) round-shaped ground- glass opacities; (iii) alveolar interstitial type with bronchiectasis resembling interstitial lung disease and pulmonary fibrosis with honeycombing; and (iv) thickening of the bronchovascular bundles and interlobular septa.
The differential diagnosis of bronchovascular-type lesions includes lymphoproliferative disorders such as multicentric Castleman disease, sarcoidosis, and lymphangitis carcinomatosa. Alveolar interstitial-type lesions radiologically resemble nonspecific interstitial pneumonia. IgG4-related disease seen in pancreas (autoimmune pancreatitis) or kidney shows a variety of imaging features despite the same pathologic change.

Please refer to

Case 723, Case 774, Case 907, Case 1015, Case 1039,

KSTR Monthly Case Conference on 2003 Fall (with McAdams)  Case 4 ,
KSTR Imaging Conference 2014 Summer  Case 5 ,
KSTR Imaging Conference 2016 Spring  Case 9 ,
KSTR imaging conference 2018 Summer  Case 13,

References

1. D. Inoue, Y. Zen, H. Abo,T. Gabata, H. Demachi, T. Kobayashi, et al. Immunoglobulin G4 related lung disease: CT findings with pathologic correlations. Radiology 2009; 251: 260-270.

Keywords

lung, consolidation with cavity, IgG4-related sclerosing disease,