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polymyalgia rheumatica

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polymyalgia rheumatica

  1. 1. Case presentation Prepared by Dr R Musa
  2. 2. GP referral <ul><li>71 yrs old Lady presented few of months ago with : </li></ul><ul><ul><li>History suggestive of PMR </li></ul></ul><ul><ul><li>Initially good response to 20mg of steroid </li></ul></ul><ul><ul><li>Difficult to ↓ steroid, (recurrent symptoms), persist ↑ ESR 50, CRP 91 </li></ul></ul><ul><ul><li>Required ↑ dose to 30mg </li></ul></ul><ul><ul><li>Developed arthralgia with puffy hands </li></ul></ul>
  3. 3. History <ul><li>C/O; </li></ul><ul><ul><li>Aching pain & stiffness in both arms, hands, knees & feet </li></ul></ul><ul><ul><li>Morning stiffness > 3 hours </li></ul></ul><ul><ul><li>Soft tissue swelling of both hands, swollen knees and ankles </li></ul></ul><ul><li>No skin rash, no excess hair loss & (no psoriasis or F/H of psoriasis </li></ul><ul><li>No dry eyes / dry mouth </li></ul><ul><li>No Wt loss, normal bowel habits & No urinary symptoms </li></ul>
  4. 4. Social history <ul><li>Never smoked. </li></ul><ul><li>Retired, married. </li></ul><ul><li>Medication </li></ul><ul><li>Prednisolone 25mg daily </li></ul><ul><li>Alendronic acid 70mg once a week </li></ul><ul><li>Atenolol 100mg </li></ul><ul><li>Doxazosin 8mg </li></ul><ul><li>Bendrofluazide 2.5mg </li></ul><ul><li>Paracetamol 1gm PRN </li></ul><ul><li>Omeprazole 20mg </li></ul>
  5. 5. On examination <ul><li>No skin rash or nails changes. </li></ul><ul><li>Symmetrical synovitis involving the 2 nd , 3 rd , & 4 th MCP joints of hands, wrists, knees & ankles </li></ul><ul><li>No lymphadenopathy </li></ul><ul><li>Chest: clear </li></ul><ul><li>Heart: NAD </li></ul>
  6. 6. D/D <ul><li>PMR </li></ul><ul><ul><li>resistant to steroid therapy </li></ul></ul><ul><li>RA </li></ul><ul><ul><li>Sero-positive RA </li></ul></ul><ul><ul><li>Sero-negative RA (LO sero (-) RA) </li></ul></ul><ul><li>Neoplasm </li></ul><ul><li>Infection </li></ul>
  7. 7. Investigation <ul><li>RF 458 </li></ul><ul><li>FBC (N) </li></ul><ul><li>ESR 39 </li></ul><ul><li>CRP 50 </li></ul><ul><li>U&E (N), LFT (N) </li></ul><ul><li>X-ray hands, Feet & CXR </li></ul>
  8. 8. erosion
  9. 11. Treatment <ul><li>LO-RA: </li></ul><ul><ul><li>MTX (10mg O/W & increase dose if no SE) </li></ul></ul><ul><ul><li>If erosion increase MTX dose & added HCQ </li></ul></ul><ul><li>PMR: </li></ul><ul><ul><li>15 mg prednisone dramatic response </li></ul></ul><ul><ul><li>MTX (as steroid sparing) </li></ul></ul><ul><ul><li>87.5% of MTX-treated patients and 53.3% of patients treated with prednisone alone were no longer on steroids at 76 weeks. </li></ul></ul><ul><ul><li>Significantly fewer patients on MTX had at least one flare up by the end of follow-up. </li></ul></ul><ul><li>Infliximab in the treatment of polymyalgia rheumatica: a double-blind, randomized, placebo-controlled study. Salvarani C, Macchioni PL, Manzini C, et al. Ann Intern Med (2007) </li></ul><ul><li>no differences were observed among groups: the proportion of patients who were free of relapses/recurrences at 22 and 52 weeks was similar </li></ul>reducing dose of steroid should be based on sign & symptoms rather than CRP & ESR value, which dose not predicate relapse Therapy usually last two years, relapse usually in the 1st or 2nd month
  10. 12. PMR (diagnostic criteria) <ul><li>> 50–60 yrs </li></ul><ul><li>Aching and stiffness in the shoulder and/or pelvic girdles > one month. </li></ul><ul><li>ESR > 40 </li></ul><ul><li>Rapidly responds to Prednisolone 15mg </li></ul>
  11. 13. Pathogenesis of PMR <ul><li>↑ Production of IL-6 </li></ul><ul><li>chronic stress lead to ↓ the hypothalamic–pituitary–adrenal (HPA) axis ↓ Production of adrenal hormones, like cortisol. </li></ul><ul><li>Functional (21 –hydroxylase) impairment in PMR due to; </li></ul><ul><ul><li>Genetic defects or </li></ul></ul><ul><ul><li>Age-related increase serum TNF & IL-6 levels </li></ul></ul><ul><ul><li>TNF- was shown to inhibit the 21 -hydroxylase. </li></ul></ul><ul><li>Steroid acting as a replacement for the reduced endogenous cortisol production, seems to be more efficient in PMR. </li></ul><ul><li>During steroid treatment ↓ ESR was more evident in PMR patients than in LO-RA patients. </li></ul>
  12. 14. Classic RA <ul><li>Symmetrical peripheral joints involvement. </li></ul><ul><li>RF seropositivity </li></ul><ul><li>Development of joint erosions </li></ul><ul><li>Extra-articular manifestations </li></ul><ul><li>Positive anti-citrullinated peptide (CCP) antibodies </li></ul>
  13. 15. Sero (-) LO-RA <ul><li>Mild symmetric synovitis in several patients with sero (-) LO-RA </li></ul><ul><li>Non-erosive course </li></ul><ul><li>Rapid and complete response to steroid. </li></ul><ul><li>35% negative for both RF & anti-CCP </li></ul><ul><li>Notes; </li></ul><ul><ul><li>Symptoms and signs of both PMR and LO-RA might alternate during the follow-up of the patients </li></ul></ul><ul><ul><li>20% of PMR patients developed overt RA during the follow-up period </li></ul></ul>
  14. 16. Polymyalgia rheumatica vs late-onset rheumatoid arthritis M. Cutolo1, M. A. Cimmino1 and A. Sulli1 ( Rheumatology 2009 48(2):93-95) <ul><li>In leeds teaching hospital - 10 years follow up of </li></ul><ul><ul><li>142 Pt (LO-RA) </li></ul></ul><ul><ul><li>147 (PMR) </li></ul></ul><ul><ul><li>42 (PMR + TA) </li></ul></ul><ul><li>PMR & LA sero (-) RA are different disease </li></ul><ul><li>High ESR + synovitis of wrist + one MCP/PIP at disease onset were; </li></ul><ul><ul><li>predictive of whether a non-erosive sero (-) patient would ultimately be diagnosed as having sero (–)LO-RA or PMR </li></ul></ul>
  15. 17. Polymyalgia rheumatica vs late-onset rheumatoid arthritis M. Cutolo1, M. A. Cimmino1 and A. Sulli1 ( Rheumatology 2009 48(2):93-95) Slow response Dramatic response Response to 15 mg steroid HLA-DRB1 allele HLA-DRB1 allele HLA allele Mildly elevated higher ESR, CRP & IL6. ESR & CRP Main sign Less frequent More myalgia Arthritis of PIP, MCP and wrist joints Older Relatively younger Age > 80% 23% Synovitis Sero–ve LO-RA PMR

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