igg4 rd pdf - esnr.org
TRANSCRIPT
University of Illinois at Chicago
IgG4 RD and pseudotumours
of the neck
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● Tumefactive inflammatory lesions • Fibroblastic proliferation • Inflammatory infiltrates
INFLAMMATORY PSEUDOTUMOR
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● Ig-G4 RD● Inflammatory myofibroblastic tumor● Inflammatory Pseudotumor● Granulomatous disorders● Histiocytic disorders
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● Systemic, inflammatory disorder ● Can affect any/ multiple organs
○ “black crow flying through the dark night”
IgG4 related disease
IgG4-RD
PancreatitisSclerosing cholangitis
Sialadenitis
Dacryoadenitis
Tubulointerstnephritis
Aortitis
Lung disease
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Marco Lanzillotta et al. BMJ 2020;369:bmj.m1067
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Histopathology
● Dense lymphoplasmacytic infiltrations
• IgG4-positive plasma cells
● Fibrosis (storiform)
● Obliterative phlebitis
Dx• 2/3• IgG4+ cells
• >40% IgG cells
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IgG4 related disease
• ↑ serum IgG4 (>135 mg/dL): • Supportive, but ! in up to 40%• Not essential for dx
• ↑ serum and tissue IgG4: not specific• in sarcoidosis, GPA
• Dx requires immunostaining for IgG4+ cells • ↑ numbers of IgG4 positive plasma cells • ↑ IgG4 / IgG ratio (>40 %)
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● Middle aged/ elderly, ♂ > ♀
H&N: Asian, younger, ♂ = ♀
● subacute onset and indolent course
● Mild weight loss, fatigue. No fever
IgG4-RD- Clinical features
● painless salivary gland swelling
● painless neck masses/ proptosis
● multiorgan involvement/ limited disease
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• Orbits: IgG4-ROD *• lack typical histopath features
• Salivary glands
• Neck: • LN
• Soft tissues
• Mucosal surfaces
IgG4-RD / Head and Neck
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IgG4- related sialadenitis
• Mikulicz’s disease
• Küttner’s pseudotumor
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Mikulicz’s disease
• >2006 : IgG4 RD
• Painless swelling of lacr glands, parotid, submand & subling glands
US• bilateral hypoechoic nodules
• ↑ Doppler • preserved glandular parenchyma
around the lesions• no duct dilatation
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Mikulicz’s disease
CT/MR
• Homog, enlarged glands
Gland function usually preserved
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Akifum i Fujita, Osam u Sakai, M argaret N. Chapm an, and Hideharu Sugim oto
RadioGraphics 2012 32:7, 1945-1958
IgG4-related Disease of the Head and Neck: CT and MR Imaging Manifestations
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Sjögren’s syndrome
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Focal chronic sclerosing sialadenitis
• Küttner’s pseudotumor (2005/ IgG4 RD)
• Painless, firm palpable mass
• Slow growing (months to years)
• Submand >> parotid • US: hypoechoic lesion
• CT/MR: enlarged gland/ focal enh
Imaging of IgG4-related disease of the head and neckClinical Radiology 73 (2018) 106e120
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IgG4-related lymphadenopathy
• 80% of patients systemic LAP on imaging
• Presenting sx in < 8%
• ↓ fibrosis ⇢ IgG4 dx difficult on the basis of LN pathology alone.
• avid on FDG PET-CT scans
• CT/MR: non specific; enlarged, homog enh
• no central necrosis or calcification
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IgG4-related mucosal inflammation
• Pharynx, hypopharynx & lx
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IgG4-related thyroiditis
• Riedel’s thyroiditis
• Hashimoto’s thyroiditis (fibrosing subtype)
RT:
• diffusely enlarged gl, with a homogeneous texture
• Loss of perithyr fat planes
Sholosh B, Borhani AA.
Thyroid ultrasound part 1: technique and diffuse
disease. Radiol Clin North Am. 2011
May;49(3):391-416,
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Imaging findings in head and neck IgG4-related disease: a
pictorial essay
Poster: ECR 2018 / C-3058
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● Steroids○ Can be steroid-unresponsive, ↑ relapse rate
● Rituximab
IgG4 Tx
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Castleman / Angiofollicular LNH● Unicentric/ multicentric
● Children & 30-40s / 50-60s
● Exuberant clinical presentation
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INFLAMMATORY MYOFIBROBLASTICTUMOR
• Children • Variable mix of inflammatory cells and fibroblasts/myofibrobl• neoplastic
anaplastic lymphoma kinase (ALK) gene rearrangement
○ Infiltrative, fibrous, bone erosion!
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Inflammatory pseudotumor
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Granulomatous disorders
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• Granulomatosis with polyangiitis (GPA)(Wegener granulomatosis)
• necrotizing granulomatous inflammation & vasculitis of small / medium-sized bl vessels
• characterized by ■ leukocytoclastic vasculitis ■ with geographic necrosis ■ surrounded by palisaded histiocytes
Granulomatosis with polyangiitis
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● Antineutrophil cytoplasmic autoAb (ANCA)-assoc vasculitides
● ↑ ANCA important
• !¼ of patients with limited GPA⇢ Final dx: bx
• but diagnostic pathological triad only 16% of bx• presence of vasculitis• necrosis • granulomatous inflammation
○ Dx can be difficult
GPA
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● M/F: 1:1
● more common in the 40s &50s
• present at any age
● H&N involvement in 72-99% of cases
● ENT manifestations are the first symptom of the disease in 63-72%
GPA- H&N
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● Can be the initial presenting finding
● Ill defined, infiltrative, with decreased T2 signal
GPA/ Skull base & soft tissues
55y old woman presenting with left otalgia
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● Vascular involvement
GPA/ Soft tissues
52 y old male with sinonasal GPA, presenting with neck pain
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● Subglottic stenosis : most comm and sev presentation (+ up to 20% of cases)
● More common in pediatric group
● Due to long-term mucosal inflam resulting in fibrosis
GPA/ Laryngotracheal involvement
32 y old woman presenting with stridor
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GPA/ Sinonasal involvement
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● Non-caseating granulomatous inflm
● 10x ↑ in African descents
● 30s & 60-70s
● ACE ↑
Sarcoidosis
● can involve any region
" “The Great Mimicker”
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● LN: +○ Post○ Nontender○ Bx !
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Sarcoidosis
● Parotid & lacrimal gl
○ Heerfordt's syndrome
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● Chr granulomatous inflm
● Children & young adults
● Bartonella henselae
● Fever only in 1/3
● Neck LAP > 80%
Cat scratch
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Histiocytosis
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● Sinus histiocytosis with massive lymphadenopathy
● Children (fever, ↑ ESR)
● Painless LAP, fever, extraconal orbital mass, dural based masses
Rosai Dorfman
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● Eosinophilic hyperplastic lymphogranuloma,● Rare benign inflammatory
● LAP (near mand angle & post-auricular), enlarged salivary gl
● Increased IgE● Males 20-40s
Kimura
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● extracellular deposition of fibrillar proteins
● Loc/ sys
● IN H&N usually presents as localized disease
● Lx > LN
● Low T2
● Calcifications
Amyloidosis
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● Clinic !● Look at the corners!● If something looks odd, start thinking.● Follow-up your difficult cases.
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THANK YOU!
@burceozgen
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