Osteoarthropathy of the front thorax

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Abstract

This article reviews the data on a unique, rare rheumatologic syndrome – osteoarthropathy of the anterior thorax (OAAT), characterized by inflammation of the bone, joint, and ligament structures that form the skeleton of the anterior thorax. OAAT is part of chronic non-bacterial osteomyelitis, which occurs in adults and children and may be due to genetic mutations. The basis of this disorder is osteitis (focal lesion of the sternum, clavicles, and ribs with a tendency to the destruction of mainly articular parts and development of bone proliferation – hyperostosis), arthritis with possible ankylosis development, as well as enthesitis and ligamentitis with ectopic ossification of affected soft tissues. Many patients also show similar dermatological changes: commonly palmar and plantar pustulosis and sometimes acne inversa (purulent hidradenitis and globular acne). The article describes typical radiological, scintigraphic, and MRI changes in the musculoskeletal system. The classification of chronic non-bacterial osteomyelitis is reviewed. Diseases that may mimic OAAT are listed. Treatments for chronic non-bacterial osteomyelitis, including bisphosphonates and biologics, are reported.

About the authors

Nikolay V. Bunchuk

Academic Clinic of Neurology and Dentistry “Cecil”

Author for correspondence.
Email: nbunchuk@yahoo.com
ORCID iD: 0000-0002-4728-400X

D. Sci. (Med.)

Russian Federation, Moscow

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2. Figure 1. OAPHC in patient N. (39 years old) with SAPHO syndrome: bright inflammation in the region of the sternum and the clavicles and ribs connected to it; pronounced swelling of the subcutaneous tissues and hyperemia of the skin (top photo). Skeletal scintigraphy (99mTc, combined with phosphates): a significant accumulation of radiopharmaceuticals, mainly in the manubrium and body of the sternum, sternoclavicular joints (more in the right) - a bull's head pattern - indicated by an arrow. Focal hyperfixation is also observed in the lateral section of the right clavicle and heads of the humerus (own observation [1]).

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3. Figure 2. Anatomical structures, the defeat of which is typical for OAPK

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4. Figure 3. MRI (T2 STIR mode) of a patient (G., 27 years old) with OAPHC (SAPHO syndrome). Edema of the bone marrow of the handle and body of the sternum, medial sections of the II and III ribs; effusion in the cavity of the manubriosternal joint (own observation).

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5. Figure 4. CT scan of the anterior-upper chest of patient P., 54 years old, with OAPHC (SAPHO syndrome). On the left (coronal plane): erosion of the articulation of the handle with the body of the sternum, as well as the articulation of the handle with the collarbones (on the left, with the expansion of the gap); uneven osteosclerosis of the handle. Right (axial plane): destruction and partial ankylosis of the sternoclavicular joint, expansion (hyperostosis) and restructuring of the bone structure of the medial section of the right clavicle (own observation [1]).

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6. Figure 5. CT scan of the sternum (axial plane) of patient N., 39 years old, with OAPHC (SAPHO syndrome): destruction of the articulation between the II right rib and the sternum, focal ossification of the cartilage of the articulation between the II left rib and the sternum, uneven osteosclerosis and periostitis of the body of the sternum , pronounced swelling of soft tissues anterior to the sternum (own observation).

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7. Figure 6. DILI in patient S., 27 years old, with SAPHO syndrome (own observation).

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8. Figure 7. Clinical classification of CNO in children and adults [7].

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9. Figure 8. CT scan of the chest (sagittal plane) of patient P., 54 years old, with OAPHC (SAPHO syndrome). Changes in the thoracic spine are determined: wedge-shaped deformity and fusion of the bodies of ThVI, ThVII and ThVIII, erosion of the upper endplate of ThVI and the lower endplate of ThVIII, unevenness of the anterior surface of the bodies, numerous areas of bone tissue enlightenment in the bodies of ThVI, ThVII and ThVIII, extensive areas of osteosclerosis in bodies of ThVI and ThVIII, marginal anterior osteophytes. Also noticeable is the deformation of the sternum manubrium with expansion (hyperostosis) and fragmentation of its upper part and zones of enlightenment and sclerosis in the lower part (own observation [1]).

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