Bone Abstracts

Background: Chronic rheumatic conditions including juvenile idiopathic arthritis (JIA) lead to the deterioration of bone mineral metabolism and confers risk of fragility fractures. Although contemporary medical therapies have limited skeletal complications in JIA, some rare cases of severe osteoporosis are still reported in these patients. Systemic glucocorticoid (GCS), long-term inflammation, and disease-related immobility are responsible to skeletal damage in JIA.

Presenting problem: We discuss a case of a 15-year-old boy treated for 13 years due to systemic onset JIA. Diagnosed at 2nd year of life, henceforward he presented with severe polyarticular involvement (>30 joints affected), limited range of movement, short stature, and muscle atrophy. The immunosuppressant therapies included methotrexate, cyclosporine, and TNF-alpha inhibitors. The patient required continuous treatment with oral and/or intravenous GCS, and subsequently developed glucocorticoid-dependence with apparent clinical manifestations of GCS adversity. Both clinical and densitometric signs of osteoporosis were found. The DXA scan showed: Lumbar BMD Z-score=−4.3; Femoral BMD Z-score=−5.7. Despite pamidronate therapy, evident vertebral compression fractures Th4, Th7, Th9 and L1 were found on MRI scans. The patient sustained a sudden, unexpected critical spinal cord compression resulting in lower body palsy, diplegia, severe neurological damage, and neurogenic urinary bladder.

Clinical management: Due to the rapid progression of the neurological damage, and serious sequence of the process, the patient was referred to prompt orthopedic and neurosurgical emergency. He was then successfully treated using a unique high-risk surgery in a specialized centre. A fast-track procedure was performed as a matter of urgency: the spinal cord decompression was effectively carried out, accompanied by a large posterior spinal fusion Th2-Th12.

Discussion: This is a report of an exceptional orthopedic surgery performed on the osteoporotic bone in the spine. The above case demonstrates that the risk of life threatening osteoporosis should still be considered during the chronic course of juvenile arthritis in pediatric patients, and, furthermore, a multidisciplinary comprehensive approach is necessary. The glucocorticoid-induced osteoporosis is one of the most serious complications of the rheumatic diseases in children, and should always be taken into account, even in the era of modern treatment.

Disclosure: The authors declared no competing interests.