Table 2:

Radiographic findings in IgG4-related disease

Organ systemTypical findings
Pancreas (58)CT: focal (more common) or diffuse pancreatic enlargement with delayed enhancement and a low-density “halo”; pancreatic atrophy is uncommon
Cholangiography: diffuse irregular narrowing of the pancreatic duct
Salivary glandCT: swelling is often bilateral and preferentially involves the submandibular glands
US: (59) multiple hypoechoic lesions in affected glands
MRI: (60) homogenous enhancement in hypointense or isointense T2-weighted imaging
Orbits (61)CT: involvement of any surrounding structures including lacrimal glands, nerves, extraocular muscles and maxillary and frontal bony structures
Lungs (9)CT: four major categories of findings (solid nodular masses, localized ground glass opacities, diffuse ground glass opacities associated with honeycomb lung and bronchovascular thickening); mediastinal lymphadenopathy is common Diffuse tracheal inflammation and subglottic stenoses may also be seen
Arterial system (62)CT: adventitial sclerosing inflammation characterized by diffuse wall thickening and late-phase enhancement
Retroperitoneum (63)CT: perivascular fibrosis concentrates around the aorta, iliac vessels and vena cava. Occasionally, fibrotic disease will dominate perirectal and retrovesicular spaces. Associated lymphadenopathy and aortitis is common.
Kidneys (64)CT: abnormalities noted in 70% of patients with renal disease include bilateral diffuse enlargement, solitary nodules and atrophy
MRI: low-density lesions with T2-weighting hypointensity, with progressive enhancement pattern
Biliary treeCholangiography: stricturing disease is difficult to differentiate from other causes of sclerosing cholangitis. Common bile duct thickness of > 2.5 mm and continuous strictures can be suggestive of IgG4-RD rather than PSC; (65) 4 patterns of stricturing disease have been described, but the clinical relevance of the different types is not obvious (66)
Cholangioscopy: direct mucosal visualization may give diagnostic clues, although this requires thorough validation (67)
Meninges and brain (8)CT: diffuse dural thickening or perineural masses as large as 3 cm in diameter; pituitary lesions require MRI
  • Note: CT = computed tomography, IgG4-RD = immunoglobulin G4–related disease, MRI = magnetic resonance imaging, PSC = primary sclerosing cholangitis, US = ultrasonography.