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Table 1 Diagnostic algorithm of IgG4-related disease

From: IgG4-related disease: case report and literature review

 

≥2 Pathology features

1 Pathology feature

Numbers of IgG4 + plasma cells

Highly suggestive of IgG4-RD

Probably suggestive of IgG4-RD

Numbers of IgG4 + plasma cells

Highly suggestive of IgG4-RD

Probably suggestive of IgG4-RD

Lacrimal gland

>100

x

 

>100

x

 

Salivary gland

>100

x

 

>100

 

x

Lung (surgical specimen)

>50

x

 

>50

 

x

Lung (biopsy)

>20

 

x

>20

 

x

Pancreas (surgical specimen)

>50

x

 

>50

 

x

Pancreas (biopsy)

>10

x

 

>10

 

x

Bile duct (surgical specimen)

>50

x

 

>50

 

x

Bile duct (biopsy)

>10

 

x

>10

 

x

Liver (surgical specimen)

>50

x

 

>50

 

x

Liver (biopsy)

>10

 

x

>10

 

x

Aorta

>50

x

 

>50

 

x

Retroperitoneum

>30

x

 

>30

 

x

  1. IgG4 +/IgG + plasma cell ration >40 % is a mandatory for histological diagnosis of IgG4-RD