FRI0587 IGG4-RELATED DISEASE IN ITALY: RESULTS FROM A MONOCENTRIC COHORT OF 150 PATIENTS (2013–2018)

Abstract

Background IgG4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory disease characterized by fibrotic lesions and elevated IgG4 serum level. Epidemiological and clinical data in our country have not been precisely assessed yet Objectives The aim of this work is to present the data from the largest monocentric Italian IgG4-RD cohort. Methods We conducted a retrospective analysis searching for IgG4-RD patients referred to our tertiary care centre between 2013 and 2018. Diagnosis were made according to the “Comprehensive Diagnostic Criteria 2011”, and patients were clustered on the basis of a definite, probable and possible, diagnosis. Each patient underwent clinical, serological, and radiological evaluation according to the organ involvement. IgG4-RD activity was measured with IgG4-RD Responder Index (IgG4-RD RI). All the data are reported in median and interquartile range. Results 150 patients were included in this work (111 male, 39 female, M:F ratio 2.8:1). 75 cases received a definite IgG4-RD diagnosis, while 6 patients and 49 patients were diagnosed as probable and possible IgG4-RD, respectively. Median age at diagnosis was 60 (SD 13) years old. The most involved organ was the pancreas (66 cases; 44%), followed by lymph nodes (26 cases; 17%), biliary tree (25 cases; 17%), salivary glands (21 cases; 14%), aorta and retroperitoneum (12 cases; 8%), meninges (9 cases; 6%), nasal sinuses and nasal septum (5 cases; 3%). 18 patients (12%) had a positive history of malignancies that occurred ten years earlier or after IgG4-RD diagnosis. At baseline, IgG4 level was elevated in 92 patients (61%), while eosinophil count and IgE level were elevated in 35 (23%) and 52 (35%) patients, respectively. Circulating plasmablast were measured in 88 and elevated in 66 (1859 cell/mL, 600-3950). Median IgG4-RD RI at diagnosis was 6 (6-9). 55 patients were treated with glucocorticoids alone, whereas immunomodulator (Mycophenolate mofetil, azathioprine, cyclophosphamide) were associated in 31 cases. Rituximab was administered in 32 patients. 15 patients underwent surgical excision of the fibrotic lesion. 15 patients were candidate to a watchful waiting strategy, due to low disease burden. Conclusion Our study reports clinical and epidemiological data from the largest monocentric cohort of patients with IgG4-RD in Italy. Our data reflect those reported in other international cohorts of IgG4-RD patients.