Granulomatosis with polyangiitis (GPA) or Wegener’s 
granulomatosis is a systemic necrotizing vasculitis with small 
sized vessel involvement and granulomatous inflammation of 
ENT and/or Lung and/or Kidney that is associated with ANCA 
positivity. 
Upon the clinical/laboratory/imaging judgement of an 
expert Rheumatologist in cooperation with an expert 
otolaryngologist and an expert infectious disease specialist the 
diagnosis of GPA can be established. 
There have been three criteria for GPA including: 
• The 1990 ACR classification criteria for Wegener’s 
granulomatosis 
• The EMA diagnostic criteria of systemic GPA 
• Iran criteria for early diagnosis of GPA 
The 1990 ACR criteria is not sensitive enough and it can not 
detect the cases of GPA in early stages. 
The EMA criteria has been made for diagnosis of 
systemic GPA in the absence of biopsy and its sensitivity is too 
much low. 
Iran criteria for early diagnosis of GPA is highly 
sensitive but there is a lag period of about a few months between 
the initial presentation of disease and the time of confirmation of 
it. 
You have to know that with a little changes within Iran 
criteria a new criteria is created by the author of this letter called 
2017 ACR/EMA revised criteria by which the diagnosis of GPA 
can be established too earlier with a lag period of a few weeks 
only. 
 
Keywords: GPA; 1990 ACR Criteria; EMA Criteria; Iran 
Criteria; 2017 ACR/EMA Revised Criteria