Marco Lanzillotta1,2, Corrado Campochiaro1,2, Matteo Trimarchi2,3, Gianluigi Arrigoni4, Simonetta Gerevini5, Raffaella Milani6, Enrica Bozzolo1, Matteo Biafora2,3, Elena Venturini2,5, Maria Pia Cicalese7,8, John H Stone9, Maria Grazia Sabbadini1,2, Emanuel Della-Torre1,2. 1. a Unit of Medicine and Clinical Immunology, Ospedale San Raffaele , Milan , Italy. 2. b Unit of Medicine and Clinical Immunology, Università Vita-Salute San Raffaele , Milan , Italy. 3. c Department of Otorhinolaryngology , IRCCS San Raffaele Scientific Institute , Milan , Italy. 4. d Pathology Unit, IRCCS San Raffaele Scientific Institute , Milan , Italy. 5. e Neuroradiology Unit, IRCCS San Raffaele Scientific Institute , Milan , Italy. 6. f Immunohematology and Transfusion Medicine Unit , IRCCS San Raffaele Scientific Institute , Milan , Italy. 7. g San Raffaele Telethon Institute for Gene Therapy (TIGET) , Milan , Italy. 8. h Pediatric Immunohematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Scientific Institute , Milan , Italy , and. 9. i Division of Rheumatology, Allergy, and Immunology , Massachusetts General Hospital, Harvard Medical School , Boston , MA , USA.
Abstract
OBJECTIVE: A series of destructive and tumefactive lesions of the midline structures have been recently added to the spectrum of IgG4-related disease (IgG4-RD). We examined the clinical, serological, endoscopic, radiological, and histological features that might be of utility in distinguishing IgG4-RD from other forms of inflammatory conditions with the potential to involve the sinonasal area and the oral cavity. METHODS: We studied 11 consecutive patients with erosive and/or tumefactive lesions of the midline structures referred to our tertiary care center. All patients underwent serum IgG4 measurement, flow cytometry for circulating plasmablast counts, nasal endoscopy, radiological studies, and histological evaluation of tissue specimens. The histological studies included immunostaining studies to assess the number of IgG4 + plasma cells/HPF for calculation of the IgG4+/IgG + plasma cell ratio. RESULTS: Five patients with granulomatosis with polyangiitis (GPA), three with cocaine-induced midline destructive lesions (CIMDL), and three with IgG4-RD were studied. We found no clinical, endoscopic, or radiological findings specific for IgG4-RD. Increased serum IgG4 and plasmablasts levels were not specific for IgG4-RD. Rather, all 11 patients had elevated blood plasmablast concentrations, and several patients with GPA and CIMDL had elevated serum IgG4 levels. Storiform fibrosis and an IgG4+/IgG + plasma cell ratio >20% on histological examination, however, were observed only in patients with IgG4-RD. CONCLUSIONS: Histological examination of bioptic samples from the sinonasal area and oral cavity represents the mainstay for the diagnosis of IgG4-RD involvement of the midline structures.
OBJECTIVE: A series of destructive and tumefactive lesions of the midline structures have been recently added to the spectrum of IgG4-related disease (IgG4-RD). We examined the clinical, serological, endoscopic, radiological, and histological features that might be of utility in distinguishing IgG4-RD from other forms of inflammatory conditions with the potential to involve the sinonasal area and the oral cavity. METHODS: We studied 11 consecutive patients with erosive and/or tumefactive lesions of the midline structures referred to our tertiary care center. All patients underwent serum IgG4 measurement, flow cytometry for circulating plasmablast counts, nasal endoscopy, radiological studies, and histological evaluation of tissue specimens. The histological studies included immunostaining studies to assess the number of IgG4 + plasma cells/HPF for calculation of the IgG4+/IgG + plasma cell ratio. RESULTS: Five patients with granulomatosis with polyangiitis (GPA), three with cocaine-induced midline destructive lesions (CIMDL), and three with IgG4-RD were studied. We found no clinical, endoscopic, or radiological findings specific for IgG4-RD. Increased serum IgG4 and plasmablasts levels were not specific for IgG4-RD. Rather, all 11 patients had elevated blood plasmablast concentrations, and several patients with GPA and CIMDL had elevated serum IgG4 levels. Storiform fibrosis and an IgG4+/IgG + plasma cell ratio >20% on histological examination, however, were observed only in patients with IgG4-RD. CONCLUSIONS: Histological examination of bioptic samples from the sinonasal area and oral cavity represents the mainstay for the diagnosis of IgG4-RD involvement of the midline structures.
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