J Rheum Dis.  2024 Oct;31(4):253-256. 10.4078/jrd.2024.0040.

Osteopoikilosis in a young ankylosing spondylitis patient

• Omelchenko V
• Letyagina E
• Korolev M

• KMID: 2559894
• DOI: http://doi.org/10.4078/jrd.2024.0040

Abstract

Osteopoikilosis (OPK) is a rare benign congenital genetic-mediated sclerosing skeletal disease, characterized by the formation of osteosclerosis foci. OPK is usually clinically asymptomatic, but some patients (15%~20%) may have arthralgia and synovitis. OPK may be associated with rheumatic diseases and might lead to unreasonable over-examination in real clinical practice. Single cases of the OPK together with ankylosing spondylitis (AS) have been described. Here we present a 33-year-old patient diagnosed with AS coexisting with OPK. In the case considered, the combination of AS and OPK accompanied with a high activity of inflammation, peripheral arthritis, a rapid rate of structural progression in axial skeleton, inefficiency of disease-modifying antirheumatic drugs and nonsteroidal anti-inflammatory drugs, a lack of response to anti interleukin-17 and a good response to a tumor necrosis factor inhibitor golimumab. We describe the important points of differential diagnosis associated with the identification of focal changes in bone tissue, especially neoplastic lesion. Foci revealed had typical localization, so, acquaintance of practicing doctors with such rare cases would minimize unnecessary examinations.

Keyword

Osteopoikilosis; Ankylosing spondylitis; Biologic

Figure

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  Figure 1 X-rays of pelvis and spine. (A) X-ray of pelvis bone reveals a stage IV of sacroiliitis and osteosclerotic foci in femurs and pelvis bones. (B~E) X-ray of cervical, thoracic and lumbar spine demonstrates pronounced syndesmophytes and ankylosing without any osteopoikilosis foci in vertebrae.

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  Figure 2 Many osteoclerotic foci are found in X-ray of shoulder (A) and elbow (B) joints. (C) X-ray of hands reveals symmetric osteoclerotic foci. No bone erosions are found. (D) X-ray of feet demonstrates foci in phalanges and metatarsal bones. Many erosions and extra-articular ossification are seen that in accordance with arthritis due to ankylosing spondylitis.

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