M. Gupta, L. Duggal, B. Gurbir Singh, J. Patel, N. Jain, A. K. Grover
2020 Annals of the Rheumatic Diseases  
Background:IgG4-related disease (IgG4-RD) is an often unrecognized, rare fibro-inflammatory condition that can involve various organ systems.Objectives:The aim of this study was to identify the different clinical patterns of this disease in a single centre in North India.Methods:70 patients were diagnosed on the basis of published diagnostic criteria for IgG4-RD. Patients' presenting complaints, epidemiological profiles, laboratory, radiological and histological findings along with the
more » ... and outcomes were collated and analyzed from 2011 – 2019.Results:In a total of 70 patients who were diagnosed with the disease, the ratio of female to male ratio was 1:1. The mean age of patients was 41.4 years. Involvement of orbits and peri-orbital tissues was highest (52.9%) due to large number of referrals from ophthalmology services. 13% of patients had multiple organ involvement. Patients with involvement of retroperitoneal tissues and lymph nodes were 8.5% and 5.7%, respectively. Increased serum IgG4 levels were found in 74.3% of the patients. Histopathological examinations of the affected organs were performed in 44 (62.85%) patients and a diagnosis of possible (38.57), probable (32.85%) and definite (28.57%) IgG4-RD was made. Majority of the patients (94.3%) required immunosuppressive medications along with corticosteroids. Azathioprine was the most commonly used (72.8%) immunosuppressive medication. Rituximab was used in17.1% of the patients, of whom one had multisystem involvement.Conclusion:This study depicts the most common patterns of organ involvement along with the epidemiological, laboratory, histological, radiological data and response to treatment, in IgG4-RD, with a definite ophthalmology referral bias, in a tertiary care centre in North India.References:[1]Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012; 366(6):539-51.[2]Khosroshahi A, CarruthersMN, Deshpande V, Unizony S, Bloch DB, Stone JH. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine 2012; 91(1):57-66.Table 1.Clinical and laboratory characteristicsCharacteristicsNumber of patients (N=70)Age (years)41.4Age group in years, n (%) >5024 (34.28) <5046 (65.71)Sex, n (%) Male36 (51.42) Female34 (47.22) ESR (mm/hr)24.37 CRP (mg/dL)12.31 Serum IgG (mg/dL)1214.2 Serum IgE (IU/L)587.2Type of IgG4 related disease, n (%) Definite20 (28.57) Probable23 (32.85) Possible27 (38.57)Data represented as mean, unless otherwise specified.CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; Ig, immunoglobulin.Table 2.Distribution of patients according to organ involvement.Organ involvementNumber (%)Orbital and periorbital37(52.9)Retroperitoneum6 (8.5)Laryngotracheal tissue4 (5.7)Aorta and branches4 (5.7)Lymph nodes3(4.2)Paranasal sinus2 (2.8)Paravertebral tissue2 (2.8)Pancreas2 (2.8)Lung and bronchus2 (2.8)Eye (Scleritis)1 (1.4)Prevesical Mass1 (1.4)Ear polyp1 (1.4)Small Bowel1 (1.4)Primary sclerosing cholangitis1 (1.4)Musculoskeletal1(1.4)Submandibular gland1(1.4)Central nervous system1 (1.4)Multisystem involvement9 (12.86%)Figure 1.Right eye proptosisFigure 2.CT abdomen showing hydronephrosis due to retroperitoneal fibrosisDisclosure of Interests:None declared
doi:10.1136/annrheumdis-2020-eular.870 fatcat:ntfnapv2cfesxknkj2amz7q63i