imaging spectrum of immunoglobulin g4-related disease (igg4-rd) - a pictorial review abstract...
TRANSCRIPT
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 IgG4-related 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)
T2-hypointense soft tissue thickening
involving the nasal septum
and right lateral nasal wall
Extension into the right maxillary
sinus
Sino-nasal Cavity
T1W GADO Heterogeneous enhancement
of lesion with central hypoenhancing regions
Case 115 year old girl with recurrent epistaxis
Sino-nasal cavity15 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 2
Patient with history of left pre-auricular pain, difficulty in mouth opening and intermittent headache in the left temporal region
Thinning and erosion inferior wall of orbit with thickening and sclerosis of anterior wall and floor of maxillary sinus
Ill-defined poorly enhancing soft tissue lesion filling the maxillary sinus and extending into the masticator space
Sino-nasal Cavity Case 3
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
Sino-nasal cavity and palate
Multiple patchy parenchymal and
subpleural opacities in the lungs
Case 4
Central nervous system
Dural thickening with abnormal enhancement, also extending along cranial
nerves IAC
IAC
Enhancing dural thickening with
intense FDG uptake
T1 post Gado
Significant reduction in size of lung lesion post treatment
Biopsy from lung lesion showsDense lymphocytic infiltration on Hand E staining(A) special staining showing IgG4plasma
cells (B)
Intense FDG uptake with SUV of 15 in the right
apical lung parenchymal lesion (white
arrow)
Lung
A B
A B
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
LungLung Cardiovascular Case 1
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
CT of PET
Left main LAD encasement (yellow arrows)
Cardiovascular
PET-CT shows significant FDG uptake (SUV-Upto 5 ) in the homogenous eccentric periaortic soft tissue
F-18 FDG PET-CT
Cardiovascular
Ascending aorta LVOT
Post steroid therapy with marked resolution
Descending thoracic aorta
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
Cardiovascular Case 2
24/09/201419/05/2014
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
Cardiovascular
Case 3
41years old gentleman with fever, chest pain for 8 months, IgG4 levels-1384mg/LRight 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)
Para-vertebral
Pancreas
Enlarged sausage shaped pancreas
Patients with IgG4 related autoimmune pancreatitis
• 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
Left moderate hydroureteronephrosis with reduced parenchymal enhancement
Aorta Iliac arteries
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
SUMMARYPatients 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