Polymyalgia RheumaticaPMR

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Polymyalgia Rheumatica(PMR)

• DR M Rehman
• GP STR3
• Taybank M C ,Dundee

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What is Polymyalgia Rheumatica?

• Polymyalgia rheumatica (PMR) is a term first used
  in 1957 to describe an inflammatory condition
  which is characterised by severe bilateral pain
  and morning stiffness of the shoulder, neck and
  pelvic girdle.
• There is some controversy as to whether or not
  PMR represents a form of giant cell arteritis
  (GCA), however the balance of evidence would
  appear to suggest that they are two distinct and
  relatively common diseases which often co-exist
  and which share many common features.

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Epidemiology

• PMR is a common condition of older age. It is
  rare under the age of 50, but the incidence rises
  with age with a peak between the ages of 70-80.
• The incidence of disease in patients over 50 is
  about 100 per 100 000.
• There is an increased incidence of the disorder
  at higher latitudes.
• Women are more frequently affected than men with
  a MF ratio of approximately 13.

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Aetiology

• The aetiology of PMR is as yet unknown, however
  it has a modest familial aggregation. It is
  linked to HLA-DRBI04 and 01 alleles, which
  suggests that a genetic predisposition to
  environmental factors may play a part.
• A viral or infectious cause has also been
  suspected due to the increased prevalence of
  antibodies to respiratory syncytial virus and
  adenovirus in PMR and the association between the
  increased incidence of the disorder and epidemics
  of Mycoplasma pneumoniae,chlamydia pneumoniae and
  Parvovirus B19.

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Presentation

• PMR may present with a variety of signs and
  symptoms, however the most common presenting
  symptoms include bilateral, severe, persistent
  pain in the neck, shoulders and pelvic girdle.
• Other symptoms and signs may include
• Pain on active and passive movement of joints (
  shoulders 70-95, hips and neck 50-70)
• Morning stiffness of more than one hour and also
  after periods of rest.
• Myositis
• Lethargy
  Contd.

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Presentation- contd.

• Loss of weight
• Depression
• Fever
• Joint effusions
• Asymmetric peripheral arthritis ( mainly knee
  and wrist) Carpal tunnel syndrome Oedema of
  hands, wrists, ankles and feet

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Differential Diagnosis

• The differential diagnosis will include many
  other conditions which may produce arthralgia
  including
• Elderly onset rheumatoid arthritis (EORA)
• Systemic lupus erythematosus
• Polymyositis
• Spondyloarthropathy
• Bacterial endocarditis
• Myeloma
• Paraneoplastic syndromes
• Primary systemic amyloidosis

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Investigations

• Investigations which will be useful in making a
  diagnosis of PMR include
• ESR - raised to a level of at least 40 mm/hour,
  although up to 20 of patients may have a normal
  ESR at diagnosis. Plasma Viscosity can be used
  instead of ESR.
• C-reactive protein - more sensitive than ESR.
• IL-6 levels - usually raised, and a useful marker
  of disease activity.

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Investigations-contd.

• FBC - anaemia of chronic disease may be present.
• Anti-cyclic citrullinated peptide ( anti-CCP) -
  useful in differentiating patients with EORA and
  a polymyalgic onset of disease, antibodies
  present in EORA but not in PMR.
• MRI/ultrasound examination of joints - may show
  proximal synovitis and/or bursitis.

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Associated diseases

• Giant cell arteritis co-exists in about 30of
  patients with PMR and shares many features of the
  disease.

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Management

• General Principles
• Document symptoms and level of any disability at
  diagnosis.
• Consider giant cell arteritis (GCA) in all people
  with polymyalgia rheumatica (PMR).
• Advise people with PMR to seek medical attention
  if they developed any symptoms of GCA--any new
  headache, jaw claudication or visual
  disturbances.
• Monitor response to treatment by assessing
  changes in clinical features and inflammatory
  markers ESR/PV,CRP.
• Manage any residual physical or psychosocial
  disability caused by the disease.
• Consider other possible diagnoses, particularly
  if symptoms are not responding to treatment.

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Management-contd.

• Non Drug
• Patients with PMR are frequently elderly and may
  have mobility problems and difficulty with many
  aspects of daily living. Many patients will
  benefit from referral to a physiotherapist and
  occupational therapist for assessment

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Management-contd.

• Drug Therapy
• Prednisolone remains the drug of choice for
  treating PMR.
• Treatment is generally initiated at a relatively
  high dose e.g. 10-20mg per day, and reduced as
  clinical improvement and improvement in
  inflammatory markers allows. Treatment usually
  lasts 18-24 months, but may need to be longer.
• Patients may be able to come off treatment
  altogether but may require further courses of
  treatment for exacerbations.
• A small number of patients will need to remain on
  long term low dose steroids.
• Bisphosphonates, or if they are not tolerated
  Calcium and vitamin D supplementation, should be
  given to all PMR patients who are receiving doses
  of Prednisolone (or equivalent)gt5mg daily for gt6
  months.

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Management-contd.

• Several drugs have been proposed as steroid
  sparing agents in PMR. These include
• Deflazacort clinical trials have yet to show if
  this is as effective as prednisolone. The
  potential benefit is fewer adverse effects,
  particularly on bone metabolism.
• Pulse treatment with intravenous
  methylprednisolone
• Intramuscular methylprednisolone although there
  is a lower cumulative dosage than would have been
  used with oral prednisolone, clinical advantages
  remain to be demonstrated.
• Methotrexate there are inconsistent results and
  few advantages in the small trials yet conducted.
  
• Infliximab
• Etanercept
• Azathioprine

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Prognosis

• PMR usually responds well to treatment with
  steroids resulting in remission in the majority
  of cases.
• Relapse may occur, usually within 2 years of
  stopping the steroids, but the condition remains
  steroid responsive.
• Morbidity and mortality may occur as a result of
  immunosuppression or steroid side effects, and
  patients should be regularly monitored whilst on
  steroids.

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Summary points

• PMR occurs in patients who on average are over 70
  years of age.
• Cardinal symptoms are shoulder hip girdle pain
  with pronounced stiffness lasting at least one
  hour.
• Clinicians must be alert to mimics, including
  infection,malignancy,metabolic bone disease, and
  elderly onset Rheumatoid arthritis.
• ESR/PV or CRP , or both, is usually raised at
  disease onset
• Giant cell arteritis is present in about 30 of
  patients
• PMR is treated with oral glucocorticoids.

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Document references

• Barber HS Myalgic syndrome with constitutional
  effects polymyalgia rheumatica. Ann Rheum Dis.
  1957 Jun16(2)230-7.
• Salvarani C, Cantini F, Boiardi L, et al
  Polymyalgia rheumatica and giant-cell arteritis.
  N Engl J Med. 2002 Jul 25347(4)261-71.
• Gonzalez-Gay MA Giant cell arteritis and
  polymyalgia rheumatica two different but often
  overlapping conditions. Semin Arthritis Rheum.
  2004 Apr33(5)289-93. abstract
• Haworth S, Ridgeway J, Stewart I, et al
  Polymyalgia rheumatica is associated with both
  HLA-DRB10401 and DRB10404. Br J Rheumatol. 1996
  Jul35(7)632-5. abstract
• Cantini F, Salvarani C, Olivieri I, et al
  Erythrocyte sedimentation rate and C-reactive
  protein in the evaluation of disease activity and
  severity in polymyalgia rheumatica a prospective
  follow-up study. Semin Arthritis Rheum. 2000
  Aug30(1)17-24. abstract
• Devogelaer JP, Gennari C Deflazacort in giant
  cell arteritis. J Rheumatol. 2002
  Oct29(10)2244-5.

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