Discussion
Diagnosis With Brief Discussion
- Diagnosis
- IgG4-related disease
- Radiologic Findings
- Fig 1. Plain radiograph shows multiple nodular opacities in both lungs.
Fig 2-4. Chest CT images show multiple variably sized, poorly defined nodules and masses with peribronchovascular and subpleural distribution in both lungs.
Fig 5. Multiple mediastinal lymph nodes are also noted.
Fig 6. F-18 FDG PET-CT reveals FDG uptake in the lung lesions and mediastinal lymph nodes.
Fig 7. Contrast-enhanced abdominal CT shows a hypoenhancing ovoid mass in the pancreatic tail.
- Brief Review
- The patient was referred to the oncology department of our institution from an outside hospital with suspected pancreatic cancer. Biopsies of the pancreas and lung nodules were performed, and histopathologic examination confirmed the diagnosis of immunoglobulin G4–related disease (IgG4-RD).
IgG4-RD is a systemic immune-mediated inflammatory disorder that can involve virtually any organ. It most commonly affects the pancreas, bile ducts, salivary glands, lacrimal glands, lungs, kidneys, and retroperitoneum. Thoracic involvement occurs in approximately 40% of patients with IgG4-RD.
The most common imaging findings of thoracic IgG4-RD are mediastinal lymphadenopathy and perilymphangitic interstitial thickening of the lungs.
Pulmonary involvement can be radiologically classified into four major patterns: (1) peribronchovascular and interlobular septal thickening, (2) nodules or mass-like lesions, (3) ground-glass opacities, and (4) alveolar interstitial thickening manifested as diffuse ground-glass opacities, bronchiectasis, and honeycombing.
IgG4-RD may also present as pleuritis with unilateral or bilateral pleural effusion and can involve large- to medium-sized vessels, the coronary arteries, and the pericardium.
IgG4-related pancreatic disease, also known as autoimmune pancreatitis (AIP), typically demonstrates diffuse pancreatic enlargement with loss of the normal lobulated contour (“sausage-shaped” appearance) and minimal adjacent fat stranding on CT. However, approximately 25–40% of patients may show focal enlargement or a mass-like lesion in the pancreas that is iso- to hypoattenuating, making it nearly indistinguishable from pancreatic carcinoma on imaging.
- References
- 1. Kim Y, Choi HY. Immunoglobulin G4-Related Disease in the Thorax: Imaging Findings and Differential Diagnosis. J Korean Soc Radiol. 2021;82(4):826-837.
2. Zheng, Y., Elsayes, K.M., Waranch, C. et al. IgG4-related disease in the abdomen and pelvis: atypical findings, pitfalls, and mimics. Abdom Radiol 2020;45:2485–2499.
- Keywords