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AB1139 Chronic recurrent ostemomyelitis in three siblings
  1. A. Uslu Gökceoglu1,
  2. E. Durmaz2,
  3. C.S. Dogan1,
  4. E. Comak1,
  5. M. Koyun1,
  6. S. Akman1
  1. 1Pediatric Nephrology - Rheumatology
  2. 2Pediatric Endocrinology, Akdeniz University, Antalya, Turkey

Abstract

Background Chronic recurrent osteomyelitis(CRMO) is an autoinflammatory disease.The disease is mostly benign,but there is a few case reports with sequela, growth retardation and bone deformities.The disease is characterised by osteolytic and sclerotic bone lesions,multifocal bone involvement,high acute phase reactants, chronic duration and sterile bone biopsy. Three siblings with the diagnosis of CRMO will be presented.

Objectives CASE 1:A girl at 11,5 years old had admitted to our hospital with swelling of mandibula, restriction of multiple joints during 5 years and no ability to walk during 8 months.Biopsy of the lesion at the mandibula was done at a different hospital and there were nonspecific findings in the pathology specimens.She had antibiotic therapies for infection at different times. At admission, she had diffuse mandibular swelling bilaterally, artritis in ankle, knees, elbows and wrists bilaterally.There was sclerotic bone lesions at tibia and diffuse osteoporosis at extremity graphies.There was diffuse activity of mandibula and focal activity at right femur at total body bone scintigraphy.Erytrocyte sedimentation rate(ESR) was 120 mm/hour.Antinuclear antibody tests were negative.She had osteoporosis at bone densitometry.Temporomandibular joint MR and extremity MR findings were related to the findings seen in CRMO.Oral corticosteroid 20mg/day and colchicine treatment with intravenous pamidronate infusion were started. After 3 months of therapy she had begun to walk with support.At that time as she had osteoporosis, corticosteroid dose was reduced to 10 mg/day and azathiopurine was started.After therapy during 6 months, she had begun to walk without any support. With immunosupressive therapy she had pamidronate infusions 5 times in every 3 months. After 1 year of therapy she is in clinical and laboratory remission.

Methods CASE 2:Nine years old brother had myalgia at lower extremities during four years. His physical examination was normal.ESR was 120 mm/hour.He had osteopetrosis and sclerotic bone lesions of femur at lower extremity graphies.The findings of lower extremity MR was related to the findings seen in CRMO.Nonsteroidal antiinflammatory(NSAID) treatment and later corticosteroid 20mg/day and pamidronate therapy were started.After 1.5 months of therapy,he had no complaint and ESR was normal.As he had cushingoid apperance, corticosteroid dose was decreased to 10mg/day and cyclophosphamide was started. With immunosupressive treatment he had pamidronate infusion 5 times in every 3 months.After 3 months of therapy,he is in clinical and laboratory remission.

Results CASE 3:13 years old brother had pain in lower extremity during 6 months.His physical examination was normal.ESR was 90 mm/hour.There was sclerotic bone lesions on X ray of foot.The findings on lower extremity MR were related to the findings in CRMO. NSAID and later corticosteroid therapy(20mg/day) were started.He had cushingoid apperance, so corticosteroid was stopped and azathiopurine was started.After 1 months of therapy he had clinical and laboratory remission.LPIN 2 mutation was negative in three siblings.

Conclusions CRMO should be thought when chronic and sclerotic bone lesions were confirmed.The family members should be assesed for symptoms.As an initial therapy, pamidronat and corticosteroid were effective and as maintanance therapy low dose corticosteroid and azathiopurine were effective.

Disclosure of Interest None Declared

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