AB1030 IgG4-RELATED DISEASE: A RETROSPECTIVE CLINICAL CASE SERIES FROM A TERTIARY CARE CENTRE IN INDIA
Authors: M. Gupta, L. Duggal, B. Gurbir Singh, J. Patel, N. Jain, A. K. Grover
Journal: Annals of the Rheumatic Diseases (2020)

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 treatment 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 characteristics Characteristics Number of patients (N=70) Age (years) 41.4 Age group in years, n (%) &gt;50 24 (34.28) &lt;50 46 (65.71) Sex, n (%) Male 36 (51.42) Female 34 (47.22) ESR (mm/hr) 24.37 CRP (mg/dL) 12.31 Serum IgG (mg/dL) 1214.2 Serum IgE (IU/L) 587.2 Type of IgG4 related disease, n (%) Definite 20 (28.57) Probable 23 (32.85) Possible 27 (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 involvement Number (%) Orbital and periorbital 37(52.9) Retroperitoneum 6 (8.5) Laryngotracheal tissue 4 (5.7) Aorta and branches 4 (5.7) Lymph nodes 3(4.2) Paranasal sinus 2 (2.8) Paravertebral tissue 2 (2.8) Pancreas 2 (2.8) Lung and bronchus 2 (2.8) Eye (Scleritis) 1 (1.4) Prevesical Mass 1 (1.4) Ear polyp 1 (1.4) Small Bowel 1 (1.4) Primary sclerosing cholangitis 1 (1.4) Musculoskeletal 1(1.4) Submandibular gland 1(1.4) Central nervous system 1 (1.4) Multisystem involvement 9 (12.86%) Figure 1. Right eye proptosis Figure 2. CT abdomen showing hydronephrosis due to retroperitoneal fibrosis Disclosure of Interests: None declared