engleski

Osteomyelitis and multiple avascular necrosis of bone (AVN) in a patient with systemic lupus erythematosus (SLE)

Introduction: Camurati-Engelmann disease (CED) is a rare autosomal dominant type of bone disorder, also known as a progressive diaphyseal dysplasia. It is characterized by cortical thickening of the diaphyses of the long bones. We present a sporadic case of CED in 8 year-old girl who was initially suspected to have neuromuscular disorder. At the age of 3 years, she was for the first time admitted to our Department presented with waddling and wide-based gait since she was 16 months old and started to walk. She also suffered symmetrical limb pain and developed muscular weakness. The physical examination revealed muscular hypotrophy of lower extremities and positive Gowers sign. Radiographs showed bilateral and symmetric cortical thickening, affecting the diaphysis of the long bones of the upper and lower limbs, resulting in narrowing of the medullary canal. There were increased density in the skull, ribs and scapulae. Radiolological differential diagnosis was consindered among the metabolic disorders, particularly Gaucher disease, and sclerosing bone dysplasias. Bone scintigraphy showed increased uptake in the both femora, tibiae, fibulae and humeri. EMNG demonstrated the characteristics of myopathy in distal and chronic neurogenic lesion in proximal musculature. The patient underwent a bone biopsy, that showed chronic multifocal osteomyelitis (CMO), and NSAID was started. Since the proposed diagnosis of CMO was not completely consistent with the patient's condition, and because of lack of response to NSAID, she underwent additional radiologic examination. Based on clinical and radiological findings the diagnosis of CED was established. No members of patient’ s family displayed a similar symptomatology. The methylprednisolone, calcium carbonate and vitamin D were introduced in therapy, which resulted in clinical and radiological improvement. Six months after the introduction of corticosteroids, a new bone scintigraphy was performed and showed only a slight increase of 99mTc-methylene disphosphonate uptake in long bones. Now, at the age of 8 years, she is without clinical symptoms but radiographs still show characteristic features for this disease, although less extensive.

systemic lupus erythematosus; child; Camurati-Engelmann disease; diagnosis; treatment

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