From: Proceedings of the 27th European Paediatric Rheumatology Congress (PReS 2021)
Patient age in years [Ref] | Gender | Cranial bones affected | Other parts of the body | ESR (mm/h) | CRP (mg/d) | Bone Biopsy | Imaging | Treatment offered | Country |
---|---|---|---|---|---|---|---|---|---|
10 [our case] | F | Maxillary, Zygomatic & Sphenoid | Radius, Facial nerve | 104 | 7.5 | (Maxilla) Fibrous dysplasia, reactive inflammatory fibro-osseous tissue with abscess formation. (Radius) similar chronic Inflammatory findings | Destructive process of the left maxillary, zygomatic, sphenoid bones, & the clivus. Mixed sclerosis and lucency of the distal right radial metadiaphysis. | NSAID | US |
11 [3] | M | Occipital | sacro-femoral | 41 | 0.4 | Lymphocytic infiltration. | Heterogeneous high intensity area on the right side of the sacrum, osteolytic lesions in the occipital bone with increased signal intensity | NSAID | Japan |
12 [4] | F | Frontal & Sphenoid | Tibia, Femur, Ribs | 44 | NR* | Non-granulomatous active osteomyelitis. | signs of osteomyelitis (destructive, increased signaling, etc). | NSAID | Netherland |
12 [5] | F | Frontal, Zygomatic & Sphenoid | Clavicle | 49 | 3.8 | Osteoblastic/osteoclastic remodeling. Granulomatous tissue rich of lymphocytes, neutrophils, & histiocytes | Area of osteolysis in the clavicle. Rearrangement & thickening of the cranial bones. | NSAID, bisphosphon-ates. | Italy |
19 [6] | M | Neurocranium | NR | 95 | 150 mg/l ** | NR | Osteosclerotic lesions in the cranial bones. | Steroid, colchicine & monthly tocilizumab | UK |
21 [7] | M | Neurocranium | Tibia | 44 | 1.9 | Marrow fibrosis and inflammatory cell invasion. | Thick skull, heterogeneous diploe with outer table erosion appearing as “worm-eaten” spots | NSAID | Japan |