CASE HISTORY

A young man now aged 19 years presented, aged 2½ years, with an 8 week history of being unwell with high swinging fevers, a “measles”-type rash, anorexia, and weight loss. He complained throughout this time of a painful right knee, and subsequently, pain in his left wrist and ankle, neck, and elbows. Initial assessment confirmed synovitis in his left ankle, right hip, neck, and some small joints of his hands. He had a quotidian fever pattern, intermittent macular rash, lymphadenopathy but no splenomegaly, and a cardiovascular examination was normal. Inflammatory markers were high (erythrocyte sedimentation rate (ESR) 129 mm/1st h; C reactive protein (CRP) 226 mg/l; platelets 594×10⁹/l—peak 1360×10⁹/l), haemoglobin 92 g/l; white blood cell count 9.6×10⁹/l (neutrophils 6.6, lymphocytes 1.9), and albumin 24 g/l. The differential diagnosis included systemic arthritis, infections, inflammatory bowel disease, and malignancy.

After exclusion of these diseases, a diagnosis of systemic juvenile idiopathic arthritis (SJIA) was made, and treatment was started with high dose non-steroidal anti-inflammatory drugs and oral prednisolone. Many treatments were subsequently used in the following years, including penicillamine, rifampicin with isoniazid, methotrexate (introduced 4 years into his disease and continued for 7 years), ciclosporin in combination with methotrexate, intravenous immunoglobulin; he is currently receiving etanercept (started after 15 years of disease, maximum dose 0.8 mg/kg twice weekly). These treatments have been complemented with intermittent multiple intra-articular steroids. Despite this treatment he continues to have active disease—ESR 42 mm/1st h, CRP 38 mg/l (ESR <20 mm/1st h on only two occasions in 17 years)—and has remained dependent on steroids (average daily dose 5–10 mg prednisolone).

There have been many consequences of a lifetime of inflammatory disease. Growth restriction (130 cm tall at the time of transfer to adult care, with only a 35 cm gain in height …