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