When did the first Paget's disease support group start?
The first organised local support group began in 1990, in Manchester, and supported many patients, carers and family members as they shared their experiences and knowledge of the condition. Alf passed away aged 77, on the 8th April 1984. He had suffered for at least 27 years with Paget’s disease.
What are the first line treatments for Paget's disease?
First line treatments for Paget's disease include medications, surgery, joint replacement, diet and exercise. While there is no cure for Paget’s disease, treatment can help ease the discomfort associated with the disorder.
How is Paget’s disease of the bone treated?
Medication: Many medications can treat Paget’s disease of the bone. The most common type is bisphosphonates. The most effective bisphosphonate is zoledronate (Reclast®). Often, one dose of this drug can result in long-lasting remission of the disease but it can’t correct bone deformities.
What is the pathophysiology of Paget disease?
Two hypotheses are proposed as to the pathogenesis: one states that Paget cells are derived from breast parenchyma or ducts and then spread to epithelium of the nipple; the other believes that Paget cells are derived from the flat epithelial cells that line the opening of the nipple [4].
What is the history of Paget's disease?
Paget's disease of bone is a chronic disorder of unknown origin. It was described for the first time by Sir James Paget in 1877. It was initially referred to as osteitis deformans, as it was considered to be chronic inflammation of the bone.
What is the first-line treatment for Paget disease?
Bisphosphonates are first-line therapy for Paget's disease, and the advent of the new bisphosphonates permits a dramatic improvement in treatment. The optimal treatment regimen should obtain normalisation or quasi-normalisation of markers of bone remodelling.
When was Paget's disease discovered?
Paget's disease of bone (PDB), which is also known as osteitis deformans, was first described by Sir James Paget more than 130 years ago [Paget, 1877].
Are there any new treatments for Paget's disease?
There is widespread agreement that a single intravenous injection (5 mg) of zoledronate (also known as zoledronic acid) is the first-line therapy for Paget's disease 18, 19.
What is the life expectancy of someone with Paget's disease?
In that study, the British General Practice Research Database identified 2465 patients diagnosed with Paget's disease of bone from 1988 to 1999; retrospective review indicated that 5-yr survival was 67% in patients with Paget's disease compared with 72% in control patients.
Is Paget's disease an autoimmune disease?
Paget's disease of bone is a chronic autoimmune inflammatory disease that leads to an increase in bone reabsorption. While depression is extensively described in Paget's disease, affecting up to 50% of patients, the prevalence of manic episodes is unknown.
What is the most common age for females to be diagnosed with Paget's disease?
Paget's disease of the breast occurs most often after age 50. Most people with this diagnosis also have underlying ductal breast cancer, either in situ — meaning in its original place — or, less commonly, invasive breast cancer. Only rarely is Paget's disease of the breast confined to the nipple itself.
Is Paget's disease genetic or environmental?
The cause of Paget's disease of bone is unknown. Scientists suspect a combination of environmental and genetic factors contribute to the disease. Several genes appear to be linked to getting the disease.
How was Paget's disease of bone discovered?
Many people with Paget's disease do not have any symptoms at all. The disease is often first discovered when X-rays are taken for another reason or when routine blood work indicates an elevated blood serum alkaline phosphatase level.
What is the best treatment for Paget's disease?
Osteoporosis drugs (bisphosphonates) are the most common treatment for Paget's disease of bone. Bisphosphonates are typically given by injection into a vein, but they can also be taken by mouth. When taken orally, bisphosphonates are generally well tolerated but can irritate the stomach.
How do you get rid of Paget's disease?
Paget disease can be treated by removing the entire breast (mastectomy) or breast-conserving surgery (BCS) followed by whole-breast radiation therapy. If BCS is done, the entire nipple and areola area also needs to be removed. If invasive cancer is found, the lymph nodes under the arm will be checked for cancer.
Is Pagets disease fatal?
The prognosis of Paget's disease of the breast depends on the type and stage of underlying cancer....Table. The five-year survival rate for Paget's disease of the breast by stage.Stage of cancerFive-year survival rate percentage (%)Stage I95.8%Stage II77.7%Stage III46.3%Stage IV14.3%Sep 22, 2021
What is Paget's disease of bone?
Paget's disease of bone (PDB) is a progressive monostotic or polyostotic metabolic bone disease characterized by focal abnormal bone remodeling, with increased bone resorption and excessive, disorganized, new bone formation. PDB rarely occurs before middle age, and it is the second most frequent metabolic bone disorder after osteoporosis, affecting up to 3% of adults over 55 years of age. One of the most striking and intriguing clinical features is the focal nature of the disorder, in that once the disease is established within a bone, there is only local spread within that bone and no systemic dissemination. Despite many years of intense research, the etiology of PDB has still to be conclusively determined. Based on a detailed review of genetic and viral factors incriminated in PDB, we propose a unifying hypothesis from which we can suggest emerging strategies and therapies. PDB results in weakened bone strength and abnormal bone architecture, leading to pain, deformity or, depending on the bone involved, fracture in the affected bone. The diagnostic assessment includes serum total alkaline phosphatase, total body bone scintigraphy, skull and enlarged view pelvis x-rays, and if needed, additional x-rays. The ideal therapeutic option would eliminate bone pain, normalize serum total alkaline phosphatase with prolonged remission, heal radiographic osteolytic lesions, restore normal lamellar bone, and prevent recurrence and complications. With the development of increasingly potent bisphosphonates, culminating in the introduction of a single intravenous infusion of zoledronic acid 5 mg, these goals of treatment are close to being achieved, together with long-term remission in almost all patients. Based on the recent pathophysiological findings, emerging strategies and therapies are reviewed: ie, pulse treatment with zoledronic acid; denosumab, a fully human monoclonal antibody directed against RANK ligand; tocilizumab, an interleukin-6 receptor inhibitor; odanacatib, a cathepsin K inhibitor; and proteasome and Dickkopf-1 inhibitors.
When does PDB occur?
PDB rarely occurs before middle age, and it is the second most frequent metabolic bone disorder after osteoporosis, affecting up to 3% of adults over 55 years of age. One of the most striking and intriguing clinical features is the focal nature of the disorder, in that once the disease is established within a bone, ...