SAT0504\u2005IGG4 RELATED DISEASE IN CHILDREN: A SINGLE CENTRE EXPERIENCE FROM NORTH-WEST INDIA

Abstract

Background: Immunoglobulin G4 related disease (IgG4RD) is a mutisystemic diorder characterized by elevated serum IgG4 levels and infiltration of IgG4 positive plasma cells accompanied by fibrosis. It is mostly considered a disease of adults and elderly people. There is paucity of literature on pediatric IgGRD. A recent systematic review has found only 25 pediatric cases. Objectives: To report broad patterns of organ involvement in IgG4RD in children and also to create awareness among treating pediatricians about this new entity. Methods: The study is based on a review of the hospital records of children with IgG4RD at tertiary centre from North-West India. Diagnosis was based on clinical features, IgG4 levels and characteristic histopathology findings. Results: Six patients had IgG4RD. Pt-1; 10-year-old boy presented with fever for 3 months and significant hepatomegaly. Investigations showed anemia, thrombocytopenia, elevated erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP). Ultrasonography (USG) revealed a hepatic mass (10x5x6 cm) that was confirmed on computed tomography. Liver biopsy showed increased plasma cells (>50 IgG4 positive plasma cells/HPF) and storiform fibrosis suggestive of IgG4 related hepatic mass. Serum IgG4 level was 420 mg/dl (N: 6-28). Pt-2; 12-year-old girl presented with an abdominal lump. Upper gastrointestinal endoscopy showed an intragastric mass with exophytic component. Histopathology of abdominal mass was consistent with IgG4RD. Serum IgG4 level was >170 mg/dl (N: 6-28). Pt-3; 21-year-old male symptomatic since age of 14 years with recurrent erythematous swellings over dorsum of the left hand, forearm and chest. Investigations showed anemia, elevated ESR, CRP, and hypergammaglobulinemia. IgG4 levels were 211 mg/dl (N: 7-57). Histopathology from left hand showed lymphoplasmacytic infiltrates, storiform fibrosis, obliterative phlebitis and increased IgG4 plasma cells (>50/HPF). Pt-4; 18-year-old girl presented with fever and weight loss. She had anemia, thrombocytosis, elevated ESR and CRP. USG abdomen showed omental thickening. Serum IgG4 level was 215 mg/dl (N: 7-57). Peritoneal and omental biopsy showed fibrosing stage of IgG4RD. Pt-5; 7-year-old girl presented with fever, oliguria, and anasarca. Investigations revealed anemia, elevated CRP, ESR, deranged renal functions and nephrotic proteinuria. Renal biopsy showed plasma cell infiltrate, storiform fibrosis, and 10-16 IgG4 plasma cells/HPF; consistent with IgG4 related tubulointerstitial nephritis. Serum IgG4 was 68 mg/dl (N: 7-26). Pt-6; 14-year-old, boy presented with painful protrusion of right eye for 8 months. MRI orbit showed bulky and enhancing extra-ocular muscles of the right eye with lacrimal gland involvement. Serum IgG4 was 119 mg/dl (N: 7-26). Histopathology of mass showed extensive fibrosis, obliterative phlebitis and lymphoplasmacytic cells infiltrate with IgG4 plasma cells >30/HPF, consistent with IgG4RD. All patients showed good clinical response to oral prednisolone (1-2 mg/kg/day and subsequently tapered). Four patients required maintenance therapy with azathioprine. Conclusion: We report 6 cases of pediatric IgG4RD with varied organ involvement and clinical manifestations. Some of the cases presented as space-occupying lesions that can sometimes be confused with neoplastic lesions. It is important to suspect these disorders early and start immunosuppressive therapy promptly to halt end-organ damage due to fibrosis. References [1] Karim F, Loeffen J, Bramer W, et al. IgG4-related disease: a systematic review of this unrecognized disease in pediatrics. Pediatr Rheumatol Online J. 2016;14(1):18 Acknowledgement: None Disclosure of Interests: None declared