polymyalgia rheumatica
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polymyalgia rheumatica polymyalgia rheumatica Presentation Transcript

  • Case presentation Prepared by Dr R Musa
  • GP referral
    • 71 yrs old Lady presented few of months ago with :
      • History suggestive of PMR
      • Initially good response to 20mg of steroid
      • Difficult to ↓ steroid, (recurrent symptoms), persist ↑ ESR 50, CRP 91
      • Required ↑ dose to 30mg
      • Developed arthralgia with puffy hands
  • History
    • C/O;
      • Aching pain & stiffness in both arms, hands, knees & feet
      • Morning stiffness > 3 hours
      • Soft tissue swelling of both hands, swollen knees and ankles
    • No skin rash, no excess hair loss & (no psoriasis or F/H of psoriasis
    • No dry eyes / dry mouth
    • No Wt loss, normal bowel habits & No urinary symptoms
  • Social history
    • Never smoked.
    • Retired, married.
    • Medication
    • Prednisolone 25mg daily
    • Alendronic acid 70mg once a week
    • Atenolol 100mg
    • Doxazosin 8mg
    • Bendrofluazide 2.5mg
    • Paracetamol 1gm PRN
    • Omeprazole 20mg
  • On examination
    • No skin rash or nails changes.
    • Symmetrical synovitis involving the 2 nd , 3 rd , & 4 th MCP joints of hands, wrists, knees & ankles
    • No lymphadenopathy
    • Chest: clear
    • Heart: NAD
  • D/D
    • PMR
      • resistant to steroid therapy
    • RA
      • Sero-positive RA
      • Sero-negative RA (LO sero (-) RA)
    • Neoplasm
    • Infection
  • Investigation
    • RF 458
    • FBC (N)
    • ESR 39
    • CRP 50
    • U&E (N), LFT (N)
    • X-ray hands, Feet & CXR
  • erosion
  •  
  •  
  • Treatment
    • LO-RA:
      • MTX (10mg O/W & increase dose if no SE)
      • If erosion increase MTX dose & added HCQ
    • PMR:
      • 15 mg prednisone dramatic response
      • MTX (as steroid sparing)
      • 87.5% of MTX-treated patients and 53.3% of patients treated with prednisone alone were no longer on steroids at 76 weeks.
      • Significantly fewer patients on MTX had at least one flare up by the end of follow-up.
    • 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)
    • no differences were observed among groups: the proportion of patients who were free of relapses/recurrences at 22 and 52 weeks was similar
    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
  • PMR (diagnostic criteria)
    • > 50–60 yrs
    • Aching and stiffness in the shoulder and/or pelvic girdles > one month.
    • ESR > 40
    • Rapidly responds to Prednisolone 15mg
  • Pathogenesis of PMR
    • ↑ Production of IL-6
    • chronic stress lead to ↓ the hypothalamic–pituitary–adrenal (HPA) axis ↓ Production of adrenal hormones, like cortisol.
    • Functional (21 –hydroxylase) impairment in PMR due to;
      • Genetic defects or
      • Age-related increase serum TNF & IL-6 levels
      • TNF- was shown to inhibit the 21 -hydroxylase.
    • Steroid acting as a replacement for the reduced endogenous cortisol production, seems to be more efficient in PMR.
    • During steroid treatment ↓ ESR was more evident in PMR patients than in LO-RA patients.
  • Classic RA
    • Symmetrical peripheral joints involvement.
    • RF seropositivity
    • Development of joint erosions
    • Extra-articular manifestations
    • Positive anti-citrullinated peptide (CCP) antibodies
  • Sero (-) LO-RA
    • Mild symmetric synovitis in several patients with sero (-) LO-RA
    • Non-erosive course
    • Rapid and complete response to steroid.
    • 35% negative for both RF & anti-CCP
    • Notes;
      • Symptoms and signs of both PMR and LO-RA might alternate during the follow-up of the patients
      • 20% of PMR patients developed overt RA during the follow-up period
  • Polymyalgia rheumatica vs late-onset rheumatoid arthritis M. Cutolo1, M. A. Cimmino1 and A. Sulli1 ( Rheumatology 2009 48(2):93-95)
    • In leeds teaching hospital - 10 years follow up of
      • 142 Pt (LO-RA)
      • 147 (PMR)
      • 42 (PMR + TA)
    • PMR & LA sero (-) RA are different disease
    • High ESR + synovitis of wrist + one MCP/PIP at disease onset were;
      • predictive of whether a non-erosive sero (-) patient would ultimately be diagnosed as having sero (–)LO-RA or PMR
  • 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