Paget’s disease of bone

Description

Paget’s disease of bone is a metabolic disorder of the bone, resulting in deformation and pain. It most commonly affects the axial skeleton; pelvis, lumbo-sacral spine, skull, femur and tibia. Diagnostic imaging is a key part of modern day diagnosis, and many cases are in fact incidental findings. On a microscopic level, the disease consists of an increased number of osteoclasts.

Today the disease is well characterised, and treatment with bisphosphonates has revolutionised symptom management and disease progression.

History

First described by James Paget in 1876, the term Padget’s disease was coined in 1889.

Paget published a series of case reports in 1876, describing the chronicity of the disease, which he termed osteitis deformans. His key observations paved way for a further series of case studies in 1882.

In view of the lower limbs, Paget observed:

The left femur and tibia became larger, heavier, and somewhat more curved. Very slowly those of the right limb followed the same course, till they gained very nearly the same size and shape.

Following this he described features of his subject as:

The skull became gradually larger, so that nearly every year, for many years, his hat, and the helmet that he wore as a member of a Yeomanry Corps needed to be enlarged.

The spine very slowly became curved and almost rigid. The whole of the cervical vertebrae and the upper dorsal formed a strong posterior, not angular, curve; and an anterior curve, of similar shape, was formed by the lower dorsal and lumbar vertebrae. The length of the spine thus seemed lessened, and from a height of six feet one inch he sank to about five feet nine inches.

He furthered his study, by employing the skills of his pathologist college (and somewhat ambiguously titled) Mr Butlin, to describe the intricate nature of the bony changes under the microscope.

The whole microscopical architecture of the bone has been altered; the structure appears to have been, almost entirely removed and laid down a fresh on a different plan and in a larger mould.

Controversies

In Paget’s second paper on osteitis deformans, he admits that he was not the first to use the term. He grants credit to a German physician, Professor Czerny. Paget writes:

After the publication of the paper I found that the name ostitis deformans had been given by Prof. Czerny, of Freiburg, to a disease described by him in the ‘Wiener medizinische Wochenschrift,’ September 27th, 1873. It Is mainly, as he says, “Eine locale Malacie des Unterschenkels,” a rather acute inflammation of the lower part of the tibia and fibula, inducing softening and angular bending, and then followed by hardening.

Paget 1882

Associated Persons

• James Paget (1814–1899)
• Vincenz Czerny (1842-1916)

Alternative names

• Osteitis deformans

References

• Czerny V. Eine locale Malacie des Unterschenkels.Wiener medizinische Wochenschrift 1873;23(39): 895-899
• Paget J. On a Form of Chronic Inflammation of Bones (Osteitis Deformans). Medico-Chirurgical Transactions 1877;60:37-64
• Paget J. Additional cases of osteitis deformans, notes on seven cases. Medico-Chirurgical Transactions 1882; 65: 225-36
• Colman E. Sir James Paget: the man and the eponym. Calcif Tissue Int. 2002 May;70(5):430-1. Epub 2002 Apr 19. PMID 11960206