A case of monostotic Paget's diseaseR Patnayak1, S Rajasekhar1, S Chintam1, TC Kalawat2, A Jena3
1 Department of Pathology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
2 Department of Nuclear Medicine, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
3 Department of Surgical Oncology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0022-3859.186396
Source of Support: None, Conflict of Interest: None
Paget's disease of the bone is a chronic disorder. It is characterized by disturbance of bone turnover in the form of focal areas of excessive osteoclastic bone resorption accompanied by a secondary increase in osteoblastic bone formation. , Paget's disease results in bone expansion and structural weakness. It can cause pain, fracture, and deformity, apart from other complications. Although many patients with Paget's disease of bone are asymptomatic, it depends on the population of the patients studied. ,, It is commonly seen in the aging population. It affects both genders with slight male predominance. The diagnosis of the disease is mostly based on radiological examination and biochemical markers of bone turnover. ,,
A 60-year-old diabetic male came with complaints of hematuria and right flank pain associated with fever and chills for 2 months. Thorough clinical examination did not reveal any abnormality. On imageology, dimercaptosuccinic acid scan cortical renography showed bilateral chronic pyelonephritis-related changes; spiral computed tomography of the abdomen and pelvis showed sclerotic and lytic foci in the right pubic bone with the possibilities of Paget's and metastasis [Figure 1]. Bone scan showed intense radiotracer uptake involving right pubic bone suggestive of monostotic Paget's disease [Figure 2] which was later confirmed by trephine biopsy which showed irregular bony trabeculae, cement lines, and areas of fibrosis [Figure 3].
Paget's disease was first described by Sir James Paget. Paget's disease can be unifocal or multifocal.
Any bone can be affected. The most commonly involved bones include pelvis, vertebrae, skull, femur, and tibia. Rarely, patients with Paget's disease develop sarcoma-like osteosarcoma.
Paget's disease can be diagnosed in patients by plain X-rays, radionuclide bone scanning, or biochemical testing of bone turnover parameters such as serum alkaline phosphatase. ,,
Paget's disease shows wide geographical variation. Earlier Paget's disease was thought to be uncommon in India. Recently, there are several case studies from South India. , Palkar and Mohan have reported 0.66% prevalence of Paget's disease in diabetic patients. However, association between diabetes and Paget's disease remains unclear.  Our patient was a diabetic.
Paget's disease of the bone may be asymptomatic initially, and it can be diagnosed by its classic radiologic features. It is treated by bisphosphonates, a group of antiresorptive drugs, with resultant decrease in the associated morbidity and mortality.  Singer et al. have recommended a single 5 mg dose of intravenous zoledronate as the treatment of choice in patients who have no contraindication such as renal impairment.  Zoledronate has greater potency and ease of use as compared to other available drugs such as calcitonin, etidronate, and pamidronate.  An early diagnosis of Paget's disease of bone can help to prevent potential complications.
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[Figure 1], [Figure 2], [Figure 3]