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Response to: ‘Eosinophilic granulomatosis with polyangiitis can manifest lacrimal and salivary glands swelling by granulomatous inflammation: a potential mimicker of IgG4-related disease’ by Akiyama et al
  1. Augusto Vaglio1,2,
  2. Federica Maritati3,
  3. Jochen Zwerina4
  1. 1Department of Biomedical Experimental and Clinical Sciences “Mario Serio”, University of Florence, Firenze, Italy
  2. 2Nephrology Unit, Meyer Children’s Hospital, Firenze, Italy
  3. 3Nephrology, Dialysis and Transplant Unit, University Hospital "Ospedali Riuniti" of Ancona, Ancona, Italy
  4. 4Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of Österreichische Gesundheitskasse (ÖGK) and Research Funds of the Austrian Workers Compensation Board (AUVA),1st Medical Department, Hanusch Hospital, Wien, Austria
  1. Correspondence to Dr Augusto Vaglio, University of Florence, Firenze 50139, Italy; augusto.vaglio{at}

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In their insightful correspondence, Akiyama et al1 focus on a peculiar feature of eosinophilic granulomatosis with polyangiitis (EGPA), that is, the involvement of salivary and lacrimal glands, which mimics the Mikulicz disease, a common manifestation of IgG4-related disease (IgG4-RD). Given that EGPA shows high serum IgG4 levels (particularly in its active phases),2 and that IgG4-RD often features allergy, eosinophilia and respiratory tract involvement, the differential diagnosis between these two disorders may be challenging. The observation that patients with EGPA can present with Mikulicz-like salivary and lacrimal gland involvement enhances the difficulty in the differential diagnosis with …

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