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LIFE-THREATENING COMPLICATIONS OF SULPHASALAZINE THERAPY IN PAEDIATRIC INFLAMMATORY BOWEL DISEASE
CY Ooi, S Hussey, M Zachos
GI/Nutrition Division, Hospital for Sick Children
BACKGROUND: Sulphasalazine (SASP) is an established first-line therapy for paediatric inflammatory bowel disease (IBD). Dose-dependent adverse effects are not uncommon. Here we highlight two rare, potentially life-threatening complications of SASP occurring within weeks of starting therapy – haemophagocytic lymphohistiocytosis (HLH) and the drug rash with eosinophilia and systemic symptoms syndrome (DRESS).
CASE 1: A 14-year-old boy was commenced on SASP following diagnosis with ulcerative colitis. He presented 3 weeks later with a high fever, diffuse rash, lymphadenopathy, conjunctivitis and hepatosplenomegaly. SASP was stopped but within days of admission he had developed bloody diarrhea. He also had significant polyuria and polydypsia. Serial laboratory investigations revealed a marked hepatitis, synthetic dysfunction (including coagulopathy) and marked leukocytosis with neutrophilia, monocytosis and eosinophilia. Renal function tests were consistent with tubulo-interstitial disease. Subsequent investigations were consistent with secondary HLH (highly elevated ferritin and lactate dehydrogenase, hypertriglyceridemia, hypofibrinogenemia, anaemia and thrombocytopenia). The boy was treated with high dose methlyprednisolone, intravenous immunoglobulin and cyclosporine. His course was further complicated by a pulmonary embolism which required anticoagulation. His hepatic and renal dysfunction resolved and he remains well on low dose prednisone maintenance therapy one year later.
CASE 2: A 10-year-old girl with steroid dependent colitis (IBD-unclassified) developed fever and a rash 3 weeks after starting SASP. Her medication was immediately stopped but she subsequently developed bloody diarrhoea and abdominal pain. She had cervical adenopathy but no hepatosplenomegaly. Laboratory tests showed leukocytosis, eosinophilia, atypical lymphocytosis, hepatitis, synthetic liver dysfunction and abnormalities consistent with tubulo-interstitial nephritis. She was treated with high dose methylprednisolone and the rash, bloody diarrhoea and laboratory abnormalities subsequently resolved.
DISCUSSION: HLH and DRESS syndrome are rare complications of SASP therapy but cause serious clinical instability and potentially life-threatening, mutli-organ involvement. Clinicians must remain vigilant for such early complications of SASP, especially in patients who are systemically unwell.