P15 A Juvenile idiopathic arthritis mimic

Abstract Case report - Introduction Juvenile idiopathic arthritis (JIA) is a diagnosis of exclusion. In paediatric rheumatology clinic, children can present with signs and symptoms that don’t quite fit under the umbrella JIA. When these cases it important we take thorough history examination, investigate as appropriate, continual assessment to check progress review response treatment. This case highlights importance this practise, in turn enabling correct be reached. allows for more appropriate treatment choices specific counselling child family regards condition expected prognosis. description A 7-year-old girl was referred 3-week inability close her hands properly, pain swelling feet. There had been no preceding illness, trauma, or tick bites. associated fever, rash, mouth ulcers eye symptoms. She complained central abdominal constipation. Her appetite weight were stable. No significant PMH. FHx brother On assessment, systems exam unremarkable. skin soft erythema. It did not appear thickened shiny, but there bilateral pitting oedema just below both knees. musculoskeletal notable dorsal hands, restriction wrists. pain, fingers, elbows, knees, ankles toes. neck shoulder movements also restricted. Limited summary investigation An USS performed affected joints which showed poly-articular synovitis tenosynovitis. documented fasciitis. treated three-days IV methylprednisolone (30mg/kg) discharged on weaning course prednisolone. reviewed 3-weeks later. functioning better reported on-going arms looked thicker. The appeared thickened. unable make claw fully extend fingers. wrists restricted visible wrist flexor tendons. improved. inflammatory markers normal. proceeded have an MRI right hand/wrist confirmed eosinophilic fasciitis Discussion Following initial found tenosynovitis, eosinophilia hypo-albuminaemia. However, clinical picture felt typical differential at time could evolving connective tissue disease; however, autoimmune screen negative. Also, initially abnormal fascial features seen imaging, other organ dysfunction pointing towards condition. Once infection risk malignancy excluded consultation infectious disease haematology teams, agreed required. Pulse first choice plan oral steroids. little returned review, progressed include non-tender, tightness thickening forearms, ongoing multiple pain. view eosinophilia, low albumin rising ALT she discussed gastroenterology team who repeated OGD. slightly enlarged spleen. OGD macroscopically Microscopically some submucosal duodenal foamy macrophages, significance uncertain. remained suspicious driven findings, so hand requested. rim high signal around tendons along planes forearm extensor compartments, consistent muscle biopsy obtained chronic inflammation fibrosis fascia. These suggested radiological commenced subcutaneous methotrexate. Key learning points Eosinophilic faciitis (EF), known Shulman syndrome after physician who, 1974 disorder medical literature, rare disorder. characterised by infiltrate fascia consisting lymphocytes, macrophages plasma cells, eosinophils sometimes present. 2- 15- fold; dermis epidermis unaffected. precede, coexist follow localised scleroderma. Clinically painful progressive induration – “peau d’orange” appearance. paediatrics, girl, findings are minimal absent presentation. Paediatric EF tends involve extremities, often paucity data EF. childhood onset higher frequency joint involvement. many develop persistent cutaneous permanent disability. Over 30% visceral involvement, such mesenteric lymphadenopathy, hepatosplenomegaly pericardial effusion. Early recognition timely initiation improves likelihood good Risk factors extensive (3—4 extremities trunk involvement) and/or younger age onset. standardised guidelines childhood-onset given severe, rapidly articular expert should combination therapy i.e., corticosteroids If fails, literature suggests instigation alternative DMARDS, biologics JAK inhibitors. Summary learning/discussion include;