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