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| Year : 2005 | Volume
: 39
| Issue : 3 | Page : 193-194 |
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| Paget's disease : An unusual cause of backache in an adult male - A case report |
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Vivek Trikha, Ravi Mittal, PP Kotwal
Department of Orthopedics, All India Institute of Medical Sciences, New Delhi, India
Click here for correspondence address and email
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How to cite this article:
Trikha V, Mittal R, Kotwal P P. Paget's disease : An unusual cause of backache in an adult male - A case report. Indian J Orthop 2005;39:193-4 |
How to cite this URL:
Trikha V, Mittal R, Kotwal P P. Paget's disease : An unusual cause of backache in an adult male - A case report. Indian J Orthop [serial online] 2005 [cited 2013 May 18];39:193-4. Available from: http://www.ijoonline.com/text.asp?2005/39/3/193/36742 |
 Introduction | |  |
Backache is a multietiological condition and at times the cause is difficult to identify. Paget's disease is an uncommon condition causing backache. The incidence of Paget's disease in India is very rare and there are very few cases reported in literature. We report a case of backache where Paget's disease was the etiology.
| Case Report | | |
A 42-year old Indian male, presented with six months of progressively increasing pain in the lower back. The pain was non-radiating in nature, produced only by forward flexion and relived by taking rest or sitting upright. There was no history of cough with expectoration, fever or loss of weight. Physical examination revealed local tenderness over the lumbar spine. The patient had no neurological deficit. His haemogram, serum chemistry and urine examination was within normal limits. The chest radiograph was also normal. Radiographic and CT evaluation of the lumber spine were performed. Radiographs of the lumbar spine showed first lumbar vertebral body with increased density, especially in periphery and increased anteroposterior and lateral dimensions. The internal trabecular structure was coarsened [Figure - 1]. The axial section of the first lumbar vertebrae in CT showed a mixed appearance with multiple areas of lucency with some sclerotic changes mainly affecting the vertebral body [Figure - 2]. A skeletal survey failed to detect any further skeletal lesion. Technetium bone scan demonstrated an increase in radioactive isotope uptake activity at three hours only in the first lumber vertebra. There were no other abnormal areas of uptake. Serum electrophoresis and prostate specific antigen report were normal. A CT guided core biopsy revealed it to be Paget's disease. Since the serum chemistry was normal, he was treated only with NSAIDs and physical therapy. The patient is asymptomatic for the last three years with significant relief in pain and he is able to carry out normal activities of daily living.
| Discussion | | |
Paget's disease of bone, also known as "osteitis deformans", was described by Sir James Paget, an English physician, in 1877. It is a disorder of bony architecture resulting from a disturbance in the rate of bone turnover.
The patients affected are usually over forty years of age, with the disease occurring more frequently in Europeans and in those regions, where migration has occurred from Europe. It is regarded as uncommon in Asians, Scandinavians, and black Africans. It is inherited as an autosomal dominant trait with high penetrance [2] . The precise aetiology of the disease is unknown but it is likely that the disease is a result of a viral infection of the osteoclasts in a genetically susceptible host[3] . In India, Paget's disease is rare [4],[5],[6] .
Bone pain is the most common presenting symptoms. Pain may arise from increased vascularity, distortion of periosteum of focus of increased mechanical stress. Bowing of weight bearing bones is another common feature, most commonly on the femur; tibia and forearm [8] .The deformity is usually asymmetrical and is associated with stress fractures on the convex surfaces of the bowed bones. A characteristic appearance that distinguishes Paget's disease from other conditions is the increased diameter of the bones particularly those of the spine. The vertebral involvement demonstrates dense sclerotic bone in the periphery and greater radiolucency in the center, which is known as "picture frame" appearance. Plain radiographs also help in assessing the degree of deformity, secondary arthritis and secondary bone tumours. Scintigraphy is a sensitive but non-specific method of detecting the distribution of Paget's disease. Computed tomograms allow the visualization of areas of osteolytic and osteoblastic changes, not possible with radiographs.
Paget's disease is characteristically associated with an increase in the bone turnover but with normal concentrations of serum calcium, phosphate, parathyroid hormones and vitamin D metabolites [9] . The osteoclastic activity of the bone is observed by increased concentrations of urinary hydroxyproline, pyridinoline (PYR), while the increased osteoblastic activity results in elevated serum bone alkaline phosphatase and osteocalcin. Bone specific alkaline phosphatase (BAP) seems to have the best diagnostic accuracy as a measure of bone formation, with a sensitivity of 84% and a specificity of 100% 10 . In most cases, the diagnosis of Paget's disease can be made from the combination of symptoms, characteristic radiographic appearances and raised concentrations of bone turnover markers. In cases with uncertainty, a biopsy may be required.
Both bisphosphonates and calcitonon work by inhibiting the osteoclastic activity. Calcitonin helps in patients with milder form of Paget's disease. Different forms of calcitonin are available (salmon, human) for subctaneous, intramuscular and nasal administration. It acts through a specific receptor mechanism in osteoclasts, to alter cell function by a cyclic adenosine monophoshate mediated system. The treatment is given for nearly one year if no relapses occur.
Laboratory monitoring of alkaline phosphatase level should be followed initially once every month, then every three months for the first year and then every six months for five year. Treatment should be recommended when remodeling indices rise above the upper limits of normal or by 25% above the previous lowest point.[10],[11]
| References | | |
| 1. | Merkow RL, Lane JM. Paget's disease of bone. Orthop Clin North Am. 1990;21:171-189  |
| 2. | Hocking L, Slee F, Haslam SI et al. Familial Paget's disease of bone: patterns of inheritance and frequency of linkage to chromosome 18q. Bone. 2000;26:577-80 |
| 3. | Siris ES. Paget's disease of bone. J Bone Miner Res 1998;13:1061-5 [PUBMED] |
| 4. | Sridhar GR. Paget's disease in India: Is it truly rare? Natl Med J India. 1994;7:101 |
| 5. | Motilal BG, Mayilavahanan N, Sriram V, Soundarapandian S, Shanmugasundaram TK. Paget's disease (osteitis deformans). Ind J Orthop. 1992, 16; 130-133 |
| 6. | Shanmugasundaram TK. Paget's disease. Ind J Orthop. 1970;4:85-95 |
| 7. | Bhardwaj OP. Monostotic Paget's disease of bone. J India Med Assoc. 1964;43: 411-442 |
| 8. | Hosking D, Meunier PJ, Rings JD, Reginster JY, Gennari C. Paget's disease of bone : diagnosis and management. Br Med J. 1996;312:491494 |
| 9. | Devlin RD, Retallack RW, Fenton AJ, Grill V, Gutteridge DH, Kent GN, et al. Long term evaluation of 1,25-dihydroxyvitamin D after short term intravenous administration of pamidronate in Paget's disease of bone. J Bone Miner Res. 1994;9:81-5 |
| 10. | Alvarez L, Guanebens N, Peris P, Monegal A, Bedini JL, Deulofeu R, et al. Discriminative value of biochemical markers of bone turnover in assessing the activity of Paget's disease. J Bone Miner Res. 1995;10:458-65 |
| 11. | Siris ES, Jacobs TP, Canfield RE. Paget's disease of bone. Bull NY Acad Med. 1980; 56:285-304. IOA White Paper |
Correspondence Address:
Ravi Mittal
Department of Orthopaedics, All India Institute of Medical Sciences, New Delhi-110029
India
DOI: 10.4103/0019-5413.36742
[Figure - 1], [Figure - 2] |
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