Imaging spectrum of
Immunoglobulin G4-related disease
(IgG4-RD) - a pictorial review
Abstract number: IRIA - 1209
Introduction
• Immunoglobulin G4-related disease (IgG4-RD) is
an increasingly recognized immune-mediated
condition
• Swelling of involved organs, lymphoplasmacytic
infiltrate with IgG4-positive plasma cells, fibrosis
with “storiform” pattern and elevated serum
IgG4 are seen
• Different organs in varying combinations can be
involved
Aims & objectives
• This pictorial essay is aimed to depict the spectrum
of imaging findings in patients with proven IgG4related sclerosing disease
•
Materials & methods
• Proven cases of IgG4-related disease were reviewed
and the spectrum of imaging findings are depicted
• Diagnostic criteria include:
(i) Increased serum IgG4 (> 135 mg/dL)
(ii) >45 IgG4-positive cells per high-power field
(iii) IgG4/IgG ratio >30% in involved tissues
Salivary glands
CECT Axial:
Bilateral swelling
of the parotid
glands with
multiple
hypodense lesions
CECT Coronal:
Bilateral swelling
of the parotid
glands
Low-attenuation
lesion incidentally
noted in the thyroid
gland
Orbit
Bilateral symmetric thickening of the extraocular
muscles and lacrimal glands with proptosis
(blue arrows)
Case 1
Sino-nasal Cavity
15 year old girl with recurrent epistaxis
T2-hypointense
soft tissue
thickening
involving the
nasal septum
and right lateral
nasal wall
Extension into the
right maxillary
sinus
T1W GADO
Heterogeneous enhancement
of lesion with central
hypoenhancing regions
Case 2
Sino-nasal cavity
15 year old girl with recurrent right sided nasal bleed
T2 - soft tissue
thickening
involving the nasal
septum and
sphenoid sinus
T1W GADO
Heterogeneous
enhancement of
lesion
Case 3
Sino-nasal Cavity
Patient with history of left pre-auricular pain, difficulty in mouth opening a
intermittent headache in the left temporal region
Ill-defined poorly enhancing soft tissue
lesion filling the maxillary sinus and
extending into the masticator space
Thinning and erosion inferior wall of
orbit with thickening and sclerosis of
anterior wall and floor of maxillary sinus
Sino-nasal cavity and palate
Case 4
Patient with inability to open mouth for the past 6 months
Lytic lesion involving the left
hard palate and adjacent
alveolar process of maxilla
Enhancing soft tissue extending into
the left nasal cavity with destruction of
medial wall of maxillary sinus
Multiple patchy
parenchymal and
subpleural opacities
in the lungs
Central nervous system
Enhancing dural
thickening with
intense FDG uptake
T1 post Gado
IAC
IAC
Dural thickening
with abnormal enhancement,
also extending along cranial
nerves
Lung
Intense FDG
uptake with SUV
of 15 in the right
apical lung
parenchymal
lesion (white
arrow)
A
B
Biopsy from lung lesion shows
Dense lymphocytic infiltration
on Hand E staining(A) special
staining showing IgG4plasma
cells (B)
Significant reduction in size of lung
lesion post treatment
A
B
Lung
Lung
Case 1
Cardiovascular
Isointense sheet like soft tissue
seen surrounding
• Anterior mitral leaflet and
IAS
• LVOT, Aortic root,
Ascending and Arch of
aorta
• Distal thoracic aorta
• Pericardial effusion
Cardiovascular
CT of PET
Homogenous soft tissue
density is seen surrounding
• Anterior mitral
leaflet(black arrow)
• LVOT, Aortic root,
Ascending and Arch of
aorta (white arrows) and
• Distal thoracic aorta (blue
arrow)
• Also note the extension
into root of left subclavian
artery(green arrow)
• Minimal pericardial
effusion (red arrow )
No significant mediastinal
adenopathy
Left main LAD encasement
(yellow arrows)
Cardiovascular
F-18 FDG PET-CT
PET-CT shows significant FDG uptake
(SUV-Upto 5 ) in the homogenous
eccentric periaortic soft tissue
Descending
thoracic aorta
Ascending aorta
LVOT
Post steroid therapy
with marked resolution
Cardiovascular
Case 2
19 years old girl with high blood pressure
Plasma IgG4-1827mg/L
Eccentric periaortic soft tissue with
luminal narrowing of distal thoracic and
proximal abdominal aorta
19/05/2014
24/09/2014
Cardiovascular
55 years old lady
with polyneuritis
cranialis, elevated
serum IgG4-2778
mg/L
Periaortitis
involving
ascending, arch
and great vessels
showing good
response to high
dose steroids and
mycophenolate
Case 3
Para-vertebral
41years old gentleman with fever, chest pain for 8 months, IgG4 levels-1384mg/L
Right posterolateral thoracotomy and open biopsy confirmed IgG4 related disease
•
•
Eccentric homogenous lower thoracic para-vertebral soft tissue with FDG
uptake (SUV-5) (red arrows)
Minimal pericardial thickening and effusion(blue arrow)
Pancreas
Patients with IgG4 related autoimmune pancreatitis
Enlarged sausage
shaped pancreas
•
Bulky featureless pancreas with
hypodense rim suggesting
autoimmune pancreatitis
•
Lobulated presarcal soft tissue
Retroperitoneal
Homogenously
enhancing
soft tissue
encasing aorta,
IVC and
bilateral iliac
arteries
Aorta
Iliac arteries
Left moderate
hydroureteronephrosis
with reduced
parenchymal
enhancement
What should make us suspect IgG4 disease
on imaging?
•
•
•
•
•
Multi-organ involvement
Sheet like infiltrative soft tissue thickening
Hypo to iso-intense on T2W images
Homogeneous sheet like intense enhancement
Peri-aortitis with eccentric wall and peri-aortic soft tissue
thickening
• Excellent response to glucocorticoids
SUMMARY
Patients with IgG4 related disease usually respond well to
corticosteroid therapy; therefore, It is important that radiologists
recognize the condition to establish an early diagnosis and avoid
unnecessary invasive procedures