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A Case of Paget's Disease

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A Case of Paget's Disease

  1. 1. Case 1 Jagdish K Prof. Dr. A. Gowrishankar’s unit
  2. 2. <ul><li>A 45yrs old lady presented to the opd with the chief complaints of </li></ul><ul><ul><li>Pain in the knee, low backache, pain in the thigh for the past 1yr. </li></ul></ul><ul><ul><li>Breathlessness on & off for 6months . </li></ul></ul>
  3. 3. <ul><li>Patient was apparently alright 1yr ago following </li></ul><ul><li>which patient developed pain in those areas, </li></ul><ul><li>insidious in onset, gradually progressive, no specific </li></ul><ul><li>character attributed. No specific aggravating / relieving factors . </li></ul>
  4. 4. <ul><li>Patient has breathlessness for the past 6months, while </li></ul><ul><li>doing her household activity and increases more </li></ul><ul><li>during exertion & relieved by rest. </li></ul><ul><li>Patient complains of occasional light headedness </li></ul>
  5. 5. <ul><li>No h/o </li></ul><ul><ul><li>Chest pain </li></ul></ul><ul><ul><li>Palpitation </li></ul></ul><ul><ul><li>Syncope </li></ul></ul><ul><ul><li>Presyncope </li></ul></ul><ul><ul><li>Abdominal pain </li></ul></ul><ul><ul><li>Nausea </li></ul></ul><ul><ul><li>Vomiting/ diarrhoea/ constipation/ bleeding from orifices. </li></ul></ul>
  6. 6. <ul><li>No h/o </li></ul><ul><ul><li>Diplopia </li></ul></ul><ul><ul><li>Dysphagia </li></ul></ul><ul><ul><li>Dysarthria </li></ul></ul><ul><ul><li>Motor deficits </li></ul></ul><ul><ul><li>Sensory deficits </li></ul></ul><ul><ul><li>Unconsciousness/ altered sensorium </li></ul></ul><ul><ul><li>Involuntary movements </li></ul></ul>
  7. 7. <ul><li>Attained menopause 2 yrs back, cycles regular during premenstrual period. No gynaec complaints in the post menopausal period </li></ul><ul><li>Vegan by diet, </li></ul><ul><li>Not a k/c/o DM/SHT/TB/Asthma </li></ul>
  8. 8. <ul><li>Patient has lost 10-15 cm of height in the past 2- 3 yrs. </li></ul>
  9. 9. Examination <ul><li>Comfortable at rest, </li></ul><ul><li>Conscious, oriented, afebrile </li></ul><ul><li>No pallor/ icterus/ cyanosis/ clubbing/ lymphadenopathy/ edema </li></ul><ul><li>Pulse: 110/min; </li></ul><ul><li>BP: 120/60 mm of hg, rt arm, supine position, SBP 130 mm of hg in lower limb by palpatory method. </li></ul><ul><li>JVP not elevated </li></ul><ul><li>RR : 16/min </li></ul>
  10. 10. <ul><li>Patient has fixed flexion deformity of hips </li></ul><ul><li>She has kyphosis , no tenderness of spine </li></ul><ul><li>There is Genu varum / tibia vara . </li></ul><ul><li>Other systems : normal </li></ul>
  11. 14. <ul><li>Problems: </li></ul><ul><ul><li>Bone pain </li></ul></ul><ul><ul><li>Loss of height </li></ul></ul><ul><ul><li>Breathlessness </li></ul></ul><ul><ul><li>Limb deformities </li></ul></ul><ul><ul><li>Probable High output state </li></ul></ul>
  12. 15. Investigations <ul><li>CBC </li></ul><ul><ul><li>Hb: 12.2 </li></ul></ul><ul><ul><li>TC : 7,200 </li></ul></ul><ul><ul><li>DC : P60 L 40 </li></ul></ul><ul><ul><li>ESR : 6/12 </li></ul></ul><ul><ul><li>PCV : 36% </li></ul></ul><ul><ul><li>Plt: 1.5 </li></ul></ul><ul><ul><li>MCV: 32 </li></ul></ul><ul><ul><li>MCH: 29 </li></ul></ul><ul><li>RFT </li></ul><ul><ul><li>RBS: 84 </li></ul></ul><ul><ul><li>Urea: 23 </li></ul></ul><ul><ul><li>Creatinine: 0.6 </li></ul></ul><ul><li>Serum electrolytes: </li></ul><ul><ul><li>Na + : 136 </li></ul></ul><ul><ul><li>K + : 4.5 </li></ul></ul><ul><ul><li>Cl - : 96 </li></ul></ul><ul><ul><li>HCO 3- : 24 </li></ul></ul>
  13. 16. <ul><li>Peripheral smear : normocytic normochromic blood picture, adequate RBCs, WBCs & platelets </li></ul><ul><li>ECG : sinus tachycardia </li></ul>
  14. 17. CXR
  15. 18. X ray LS spine
  16. 21. X ray skull
  17. 22. <ul><li>Serum calcium : 8.8 mg/dl ( total & corrected) </li></ul><ul><li>Phosphate : 4 (N:2.4-4.1mg/dl) </li></ul><ul><li>ALP : 880 (N: 40-140 U/L) </li></ul><ul><li>PTH : 55 pg/ml (N:10-60 pg/ml) </li></ul><ul><li>Vitamin D3: 40 (N: 20-76 pg/ml) </li></ul>
  18. 23. <ul><li>TFT : </li></ul><ul><ul><li>T3 : 196 (N: 75 - 220 ng/dL) </li></ul></ul><ul><ul><li>T4 : 7.2 (N: 4 - 11 μg/dL) </li></ul></ul><ul><ul><li>TSH : 3.8 (N: 0.5-5.0 mIU/L) </li></ul></ul><ul><li>RBC transketolase: 500 (N:440± 120 μ g/ml/hr ) </li></ul>
  19. 24. <ul><li>ECHO : </li></ul><ul><ul><li>EF 66% </li></ul></ul><ul><ul><li>No RWMA </li></ul></ul><ul><ul><li>Normal LV systolic function </li></ul></ul><ul><ul><li>Calcific aortic sclerosis </li></ul></ul><ul><li>ABG : mild respiratory alkalosis </li></ul><ul><li>PFT : normal </li></ul>
  20. 25. <ul><li>Pelvic bone biopsy: </li></ul><ul><ul><li>Increase in no & nuclei of osteoclasts </li></ul></ul><ul><ul><li>Jigsaw puzzle pattern appreciated </li></ul></ul><ul><ul><li>Picture consistent with Paget’s. </li></ul></ul>
  21. 26. Pagets disease <ul><li>Sir James Paget first described it due to an inflammatory process </li></ul><ul><li>Used to be called as osteitis deformans , now called as osteodystrophica deformans. </li></ul>
  22. 27. <ul><li>A chronic disorder that can present as enlarged & misshapen bones </li></ul><ul><li>Excessive breakdown & formation of bone causes affected bone to weaken, presenting with pain, misshapen bone, fracture & osteoarthritis. </li></ul><ul><li>Disease typically affects one or few bones as opposed to osteopetrosis which affects all bones. </li></ul>
  23. 28. <ul><li>Rarely diagnosed before 40 yrs </li></ul><ul><li>Prevalence 1.5 – 8% depending on the age & the local prevalence </li></ul>
  24. 29. Etiology <ul><li>Viral </li></ul><ul><ul><li>Paget's disease may be caused by a slow virus infection (i.e ., paramyxoviruses ) present for many years before symptoms appear. </li></ul></ul><ul><ul><li>Measles though recent evidence has cast some doubt upon the measles association.[4] </li></ul></ul><ul><ul><li>Canine distemper virus </li></ul></ul><ul><ul><li>Respiratory syncytial virus </li></ul></ul>
  25. 30. <ul><li>Genetic </li></ul>Name OMIM Locus Gene PDB1 167250 6p ? PDB2 18q22.1 RANK PDB3 5q35 SQSTM1 PDB4 606263 5q31 ?
  26. 31. Pathogenesis <ul><li>Increased no & size of osteoclasts </li></ul><ul><li>Osteoclasts hypersensitive to Vitamin D3 & RANK ligand </li></ul><ul><li>Marrow stromal cell – increased expression of RANK-Ligand. </li></ul>
  27. 32. Pathogenesis <ul><li>Phases </li></ul><ul><ul><li>1 : osteoclastic </li></ul></ul><ul><ul><li>2 : osteoclastic & osteoblastic </li></ul></ul><ul><ul><li>3 : exhaustive & burnt out phase </li></ul></ul><ul><ul><li>All three phases may be seen at same time at different sites. </li></ul></ul>
  28. 33. Symptoms <ul><li>Bone pain is the most common symptom. It can occur in any bone affected by Paget's disease. It often localizes to areas adjacent to the joints. </li></ul><ul><li>Headaches and hearing loss may occur when Paget's disease affects the skull. </li></ul><ul><li>Pressure on nerves may occur when Paget's disease affects the skull or spine . </li></ul>
  29. 34. Symptoms <ul><li>Hip pain may occur when Paget's disease affects </li></ul><ul><li>the pelvis or thighbone. </li></ul><ul><li>Damage to joint cartilage may lead to arthritis. </li></ul><ul><li>Teeth may spread intraorally due to the intraoral force placed on the anterior teeth (especially maxillary central and lateral incisors) by the labial tissues, especially the muscles. </li></ul>
  30. 35. Symptoms <ul><li>Somnolence (drowsiness) may be due to vascular steal syndrome of the skull . </li></ul><ul><li>Paralysis may be due to vascular steal syndrome of the vertebrae. </li></ul><ul><li>Increased head size, bowing of limb, or curvature of spine may occur in advanced cases. </li></ul><ul><li>Hypercementosis in teeth may occur. </li></ul>
  31. 36. Diagnosis <ul><li>Calcium </li></ul><ul><li>Phosphate </li></ul><ul><li>ALP </li></ul><ul><li>Hydroxyproline </li></ul><ul><li>N telopeptide </li></ul><ul><li>C telopeptide </li></ul>
  32. 37. Differential diagnosis <ul><li>Fibrogenesis imperfecta ostium </li></ul><ul><li>Osteoblastic mets </li></ul><ul><li>Sickle cell anemia </li></ul><ul><li>Osteopetrosis </li></ul><ul><li>Pyknodysostosis </li></ul>
  33. 38. Treatment <ul><li>Decision about treating patients can be complicated because </li></ul><ul><ul><li>No two people are affected exactly the same way by the disease </li></ul></ul><ul><ul><li>Difficulty to predict the progress. </li></ul></ul>
  34. 39. When to treat??? <ul><li>Bone pain </li></ul><ul><li>Headache </li></ul><ul><li>Nerve related symptoms </li></ul><ul><li>Elevated SAP </li></ul><ul><li>High output state </li></ul>
  35. 40. Drugs <ul><li>Oral: </li></ul><ul><ul><li>Etidronate : 200-400mg OD for 6months </li></ul></ul><ul><ul><ul><li>Precautions </li></ul></ul></ul><ul><ul><li>Alendronate </li></ul></ul><ul><ul><li>Tiledronate </li></ul></ul><ul><ul><li>Risendronate </li></ul></ul>
  36. 41. Drugs <ul><li>IV </li></ul><ul><ul><li>Pamidronate: 30-60 mg iv in NS OD for 3days </li></ul></ul><ul><ul><li>Zolendronate : 5mg infusion in NS stat. once a year dose. </li></ul></ul>
  37. 42. <ul><li>Calcitonin : s/c thrice a week for 18months. Nasal spray not approved for treatment of Paget’s but for osteoporosis. </li></ul><ul><li>Surgery </li></ul>
  38. 43. Calcium levels <ul><li>Normal </li></ul><ul><li>Hypo </li></ul><ul><li>Hyper </li></ul>
  39. 44. Diet & Exercise <ul><li>1000-1500 mg of calcium </li></ul><ul><li>Adequate sunshine </li></ul><ul><li>400 units of Vitamin D3 </li></ul><ul><li>Especially when treating with bisphosphonates. </li></ul><ul><li>Administration of calcium & bisphosphonates should be separated by 2 hours. </li></ul>
  40. 45. Prognosis <ul><li>The outlook is generally good, particularly if treatment is given earlier. </li></ul><ul><li>Earlier the disease onset,morbidity is more ; </li></ul><ul><li>Disease does not spread to normal bones </li></ul><ul><li>Treatment can control Paget's disease and lessen symptoms, but is not a cure </li></ul><ul><li>Osteogenic sarcoma can occur </li></ul><ul><li>Life expectancy is not altered . </li></ul>
  41. 46. Physician & Paget’s disease <ul><li>Headache </li></ul><ul><li>Nerve problems </li></ul><ul><li>Vascular steal phenomenon </li></ul><ul><li>calcific aortic valve sclerosis; </li></ul><ul><li>High output state </li></ul>
  42. 47. Ivory vertebra
  43. 48. Picture frame vertebra
  44. 49. Osteoporosis circumscripta
  45. 50. carry home points <ul><li>Middle age – elderly ; </li></ul><ul><li>Bone pain,deformity ;nerve compression ;high output state; </li></ul><ul><li>Increased osteoclastic resorption ,compensatory osteoblastic activity ; </li></ul><ul><li>Normal electrolytes ;increased ALP & increased urinary markers ; </li></ul><ul><li>Bisphosphonates are the treatment ; </li></ul><ul><li>Prognosis is usually good ; </li></ul><ul><li>ZOLENDRONATE IS AVAILABLE IN STANLEY </li></ul>
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