Sclerotic bone diseases

Osteosclerosis is a disorder that is by abnormal hardening of bone and an elevation in bone density. It may predominantly affect the medullary portion and/or cortex of the bone. Plain radiographs are a valuable tool for detecting and classifying osteosclerotic disorders. It can manifest in localized or generalized osteosclerosis. Localized osteosclerosis can by Legg–Calvé–Perthes disease, sickle-cell disease, and osteoarthritis among others.

Osteosclerosis can classify in accordance with the causative factor into acquired and hereditary. In the animal kingdom, there also exists a non-pathological form of osteosclerosis; resulting in an unusually solid bone structure with little to no marrow. It is often seen in aquatic vertebrates; especially those living in shallow waters, providing ballast as an adaptation for an aquatic lifestyle. It makes bones heavier, but also more fragile.

Focal and sclerotic bone diseases

The mechanisms and molecular pathways underlying focal and sclerotic bone diseases; rare disorders which have a strong genetic component. This has already led to a clearer characterization of these conditions and, in several cases, has enabled the development of new therapeutic approaches.

The invited reviews featured in this special edition of ‘Calcified Tissue International‘ provide expert commentary and a valuable overview of advances in the knowledge of several rare focal and sclerotic bone diseases including Paget’s disease of bone and related syndromes, fibrous dysplasia of bone and McCune-Albright syndrome; Melorheostosis and Osteopoikilosis, chronic non-bacterial osteomyelitis; as well as Camurati-Engelmann disease.

Although investigations into the molecular and genetic basis of these conditions have not yet yielded results for all the conditions; the advances have overall led to a better understanding of the causes of the particular lesions. In certain cases, like Paget’s disease, this knowledge has vastly improved diagnosis and management of the disorder. In other cases, such as with Camurati-Engelmann disease, it has led to research into a potentially new therapeutic approach.

The case of Fibrogenesis

Finally, in other disorders where a genetic basis is suspected but not yet pinpointed, advances have still made in the management of these disorders, for example in the case of Fibrogenesis Imperfecta Ossium. Professor Stuart Ralston, the special issue editor; “These state-of-the-art reviews by leading experts show the immense progress which has been made in our understanding of rare skeletal diseases.

Indeed, for diseases such as Paget’s, tremendous leaps have made which now mean better management of the disease is possible. In other diseases, these advances mean new avenues for therapeutic intervention are now on the horizon. Although there is still a long way to go, the hope is that with increasing knowledge we will be able to continually improve patient management from diagnosis to treatment. they thank the authors for contributing these outstanding publications.”