The above clinical scenario is most consistent with Paget's disease. The clinical photo shows a bowed leg and mixed lucent/sclerotic deformity pattern on radiograph. Paget's disease is often asymptomatic, but presenting symptoms can include chronic pain and deformity, similar to the above clinical scenario. Once Paget's disease is diagnosed, patients are often treated with bisphosphonates during the active disease process to help control osteoclast lytic activity and reduce pain.
Tieg provides a review of Paget's disease of bone and treatment indications. In his review, he discusses that most patients with Paget's disease are asymptomatic, but in those that do present, pain is the most common presenting symptom. He recommends medical and surgical treatment of asymptomatic patients who have active disease at sites where complications are likely to develop.
Altman did a review of 290 patients with Paget's disease of bone. His findings showed 83% had one or more rheumatic syndromes. He found the rate of osteoarthritis related to Paget's disease was elevated in the hip and knee. Rheumatoid arthritis, hyperuricemia, and gout did not appear increased in this group.
Mangham et al looked the rate of secondary sarcomas as a result of Paget's disease of bone. They found the rate to be around 0.3%, with male predominance. They also found that widespread skeletal involvement by Paget's disease was not a significant risk factor for malignant transformation.
Answer 1: TNF-a inhibition is used to treat rheumatoid arthritis (Infliximab, Etanercept).
Answer 2: IL-1 receptor inhibition is used to treat rheumatoid arthritis (Anakinra).
Answer 3: Estrogen receptor blockade is used to treat certain types of breast cancer and ovarian cancer (Tamoxifen).
Answer 5: COX inhibition is used to treat inflammation (NSAIDs).
Tiegs RD. Paget's disease of bone: indications for treatment and goals of therapy. Clin Ther. 1997 Nov-Dec;19(6):1309-29; discussion 1523-4.
PMID:9444442 (Link to Abstract)
Altman RD. Musculoskeletal manifestations of Paget's disease of bone. Arthritis Rheum. 1980 Oct;23(10):1121-7.
PMID:6448603 (Link to Abstract)
Mangham DC, Davie MW, Grimer RJ. Sarcoma arising in Paget's disease of bone: declining incidence and increasing age at presentation. Bone. 2009 Mar;44(3):431-6.
PMID:19064007 (Link to Abstract)