polymyalgia rheumatica
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 2nd, 3rd, & 4th 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 relapseTherapy 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 arthritisM. 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 arthritisM. Cutolo1, M. A. Cimmino1 and A. Sulli1 (Rheumatology 2009 48(2):93-95)
PMR Sero–ve LO-RA
Synovitis 23% > 80%
Age Relatively younger Older
Arthritis of PIP, MCP and wrist joints
Less frequent
More myalgia Main sign
ESR & CRP higher ESR, CRP & IL6. Mildly elevated
HLA allele HLA-DRB1 allele HLA-DRB1 allele
Response to 15 mg steroid
Dramatic response Slow response