IgG4-Related Disease
Ali Abdel Satir
MBBS,DCP, FRCPath
IgG4-Related Disease
Definition
• Systemic inflammatory condition
- Mass lesions
-Dense lymphocytic & plasma cells, eosino...
IgG4-Related Disease

• History
•
•
•
•

1961-Sarles-Panceatitis + ↑ Ig
1995-Yoshida proposed AIP
2001-Hammano ↑ IgG4 in A...
IgG4-Related Disease

2011-International IgG4related diseases symposium
held in Boston the term
IgG4-related disease
(IgG4...
IgG4-Related Disease
•
•
•
•
•
•
•

Autoimmune pancreatitis
Sclerosing cholangitis
Mikuliz’s disease
Riedel’s thyroiditis
...
IgG4-Related Disease
Diagnostic Criteria *
• Clinical features
• IgG4 ˃ g/dl
1.4
• Radiology
• Histopathology
*Japanese
*B...
IgG4-Related Disease
Japanese CCD criteria *
1-Clinically diffuse / localised swelling
2-Elevated IgG4 ˃ mg / dl
135
3-His...
IgG4-Related Disease
Histopathology
• Lymphoplasmacytic inflammation
• Fibrosis
• Obliterative venulitis
IgG4-Related Disease
IgG4
•
•
•
•
•

Least common of IgG class
3-6% of IgG subclass
IgG subclasses 95% AA homology
Differ ...
IgG4-Related
Kidney Disease
IgG4-Related Kidney Disease
IgG4-RKD
• IgG4-related tubulinterstitial nephritis
• IgG4-related glomerulonephritis
• IgG4-r...
IgG4-Related
Tubulo-Interstitial
Nephritis
IgG4-TIN
IgG4- TIN
•
•
•
•
•

Average age 65 years
70-80 % males
75 % present with renal disease
25% present with renal mass
80% ha...
IgG4-TIN
Laboratory Findings
• 90% have elevated IgG4
• 60-80 % hypocomplementaemia
• 30-50 % peripheral eosinophilia
• 30...
IgG4-TIN
• Plasma cell rich infiltrate
• Tubulitis- mononuclear
• Eosinophils-variable *
• Absent neutros & granuloma
• In...
IgG4-TIN
Immunoflouscence
• 80 % TBM deposits
• IgG, kappa. Lambda & C3
• Corresponding deposits by EM
• Glomeruli negativ...
IgG4-TIN
IgG4-TIN Diagnostic Criteria
• Histology
• Serology
• Other organ involvement
• Radiology
• Exclude: Wegners,
Chu...
IgG4-Related
Glomerulonephritis
IgG4-MGN
IgG4-MGN
•
•
•
•
•
•
•

IgG4-MGN can occur alone
Associated with IgG4-TIN
Patients with IgG4 with proteinuria
IgG4-MGN is ...
IgG4-MGN
•
•
•
•

Granular immunoflouresence
IgG, C3, Kappa & lambda
IgG4 GBM deposits
Phospholipase A2 receptor negative
IgG4-Related
Vascular Disease
Thank you
IgG4 antibodies undergo ‘half-antibody
exchange’
in vivo, resulting in recombined antibodies
comprising
two different bind...
IgG4 related disease
IgG4 related disease
IgG4 related disease
IgG4 related disease
IgG4 related disease
IgG4 related disease
IgG4 related disease
IgG4 related disease
IgG4 related disease
IgG4 related disease
IgG4 related disease
IgG4 related disease
IgG4 related disease
IgG4 related disease
IgG4 related disease
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IgG4 related disease

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IgG4 related disease with special emphasis on kidney disease

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IgG4 related disease

  1. 1. IgG4-Related Disease Ali Abdel Satir MBBS,DCP, FRCPath
  2. 2. IgG4-Related Disease Definition • Systemic inflammatory condition - Mass lesions -Dense lymphocytic & plasma cells, eosino -IgG4 positive plasma cells -Storiform fibrosis -Plebitis
  3. 3. IgG4-Related Disease • History • • • • 1961-Sarles-Panceatitis + ↑ Ig 1995-Yoshida proposed AIP 2001-Hammano ↑ IgG4 in AIP 2003 -Kamisawa systemic disease
  4. 4. IgG4-Related Disease 2011-International IgG4related diseases symposium held in Boston the term IgG4-related disease (IgG4-RD) was proposed
  5. 5. IgG4-Related Disease • • • • • • • Autoimmune pancreatitis Sclerosing cholangitis Mikuliz’s disease Riedel’s thyroiditis Retroperitoneal fibrosis Orbital pseudotumour Idiopathic hypocomplementaemic tubulointerstitial nephritis
  6. 6. IgG4-Related Disease Diagnostic Criteria * • Clinical features • IgG4 ˃ g/dl 1.4 • Radiology • Histopathology *Japanese *Boston consensus histo criteria
  7. 7. IgG4-Related Disease Japanese CCD criteria * 1-Clinically diffuse / localised swelling 2-Elevated IgG4 ˃ mg / dl 135 3-Histopathology -marked lympho & plasma cell + fibrosis -infiltration by IgG4 + plasma cells ** Definite: 1+2+3, Probable: 1+3 Poss: 1+2 *Japanese comprehensive clinical diagnostic criteria ** IgG4+/ IgG+ ˃ 40% & 10 IgG4 + cells /HPF
  8. 8. IgG4-Related Disease Histopathology • Lymphoplasmacytic inflammation • Fibrosis • Obliterative venulitis
  9. 9. IgG4-Related Disease IgG4 • • • • • Least common of IgG class 3-6% of IgG subclass IgG subclasses 95% AA homology Differ in the hinge region IgG4 plays role in allergy
  10. 10. IgG4-Related Kidney Disease
  11. 11. IgG4-Related Kidney Disease IgG4-RKD • IgG4-related tubulinterstitial nephritis • IgG4-related glomerulonephritis • IgG4-related plasma cell arteritis • IgG4-related ureteral pseudotumour
  12. 12. IgG4-Related Tubulo-Interstitial Nephritis IgG4-TIN
  13. 13. IgG4- TIN • • • • • Average age 65 years 70-80 % males 75 % present with renal disease 25% present with renal mass 80% have other organs involved * *Panceas, liver,salivary, lacrimal glands
  14. 14. IgG4-TIN Laboratory Findings • 90% have elevated IgG4 • 60-80 % hypocomplementaemia • 30-50 % peripheral eosinophilia • 30 % ANA positive (low titer)
  15. 15. IgG4-TIN • Plasma cell rich infiltrate • Tubulitis- mononuclear • Eosinophils-variable * • Absent neutros & granuloma • Interstitial fibrosis-storiform *Drug induced allergic TIN
  16. 16. IgG4-TIN Immunoflouscence • 80 % TBM deposits • IgG, kappa. Lambda & C3 • Corresponding deposits by EM • Glomeruli negative • IgG4 positive plasma cells * * Moderate 11-30 / HPF, Marked ˃ 30
  17. 17. IgG4-TIN IgG4-TIN Diagnostic Criteria • Histology • Serology • Other organ involvement • Radiology • Exclude: Wegners, Churg-Strauss,Myeloma
  18. 18. IgG4-Related Glomerulonephritis IgG4-MGN
  19. 19. IgG4-MGN • • • • • • • IgG4-MGN can occur alone Associated with IgG4-TIN Patients with IgG4 with proteinuria IgG4-MGN is similar to idiopathic Thick capillary loops Subepithelial deposits-trichrome GBM spikes on silver stain
  20. 20. IgG4-MGN • • • • Granular immunoflouresence IgG, C3, Kappa & lambda IgG4 GBM deposits Phospholipase A2 receptor negative
  21. 21. IgG4-Related Vascular Disease
  22. 22. Thank you
  23. 23. IgG4 antibodies undergo ‘half-antibody exchange’ in vivo, resulting in recombined antibodies comprising two different binding specificities. IgG4 does not activate complement pathways and has reduced effector function relative to other IgG subtypes. IgG4 production is driven in part by Th2 cytokines. The role of IgG4 antibodies in the pathogenesis of IgG4-RSD remains unclear at this time.
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