Abstract

CASE: 14 Y/M diagnosed with ileo-colonic Crohn disease at 5 Y of age. He was initially treated with oral prednisone (2 mg/kg/day) for 2 weeks and oral mesalamine. The steroids were weaned over a period of 2 months. As he continued to have persistent symptoms, he was started on infliximab infusions. After his third infliximab infusion he developed severe bilateral lower extremity pain and bilateral knee swelling requiring inpatient admission. X ray of bilateral knee joints was normal. The symptoms were attributed to an infusion reaction and he was then switched to mercaptopurine, which he did not tolerate. He was started on adalimumab, but developed antibodies within 2 months of therapy. He was also given 2–3 courses of oral steroids over 1–2 years to induce remission. The dose was 2 mg/kg/day for 1–2 weeks with weaning over 1–2 months. He developed necrosis of the left femoral head when he was 9Y. The diagnosis was based on typical x ray appearance. His BMI was 82 percentile when he developed osteonecrosis and his last course of steroid was >6 months ago. He was on apriso when he developed osteonecrosis. His symptoms improved with physiotherapy over the next 1–2 years. After he failed therapy on Humira, he was started on oral budesonide (6 mg daily) as he continued to have diarrhea and his repeat colonoscopy showed worsening disease. He was on the oral budesonide for 6–7 weeks and developed typical steroid facies—moon face, supraclavicular fat pads and gained 6 lbs in 6 weeks. His blood pressure was normal. The budesonide was quickly weaned and he was switched to subcutaneous methotrexate followed by entyvio due to lack of clinical response on methotrexate. Currently he is in remission on entyvio. He continues to walk with a limp and is not complaining of any limb pain. DISCUSSION: Osteonecrosis of the femoral head (ONFH) is a disabling condition of the hip joint that primarily affects adults (avg: 36–38 Y). Risk factors for ONFH include chronic corticosteroid administration, chronic alcohol ingestion, smoking, and various chronic diseases (renal disease, hematological disease, inflammatory bowel disease (IBD), post organ transplantation, hypertension, and gout). Although ONFH is seen in children with malignancy, post stem cell transplant and sickle cell disease, it is not commonly reported in children with IBD. The exact incidence of ONFH in children on chronic steroids with IBD is unknown. In our patient, we are not sure if the ONFH was from chronic steroid use or underlying IBD. He also developed steroid facies within 6 weeks on oral budesonide which is unusual. Iatrogenic Cushing's syndrome is not expected with budesonide because a “first pass effect” removes the drug from the circulation. Multi center studies are needed in children with IBD looking at serious side effects of all types of systemic steroids as they are frequently prescribed.

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