polymyalgia rheumatica and giant cell arteritis
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DESCRIPTIONPolymyalgia Rheumatica and Giant Cell Arteritis. Definition of Fibromyalga. “Chronic and widespread pain located at 11 or more of 18 tender points”-ACR definition No specific diagnostic test and no cure. Clinical manifestations. Chronic and persistent pain with varying manifestations - PowerPoint PPT Presentation
Polymyalgia Rheumatica and Giant Cell Arteritis
Definition of FibromyalgaChronic and widespread pain located at 11 or more of 18 tender points-ACR definition
No specific diagnostic test and no cure.
Clinical manifestationsChronic and persistent pain with varying manifestationsDiffuse musculoskeletal pain, stiffness and fatigueJoints not swollen painful or redSensation such as tingling, numbness, burning, crawling are often describedPain is aggravated by change in weather, stress, fatigue, lack of sleep et cetera et cetera
Epidemiology2% of all US citizens affected: 5.5 millionMost common cause of generalized pain in women ages 20 to 50Prevalence increases with age: 2% ages 20 to 35, with 8% at age 70Females 10 times more common than males
Source: The Manual Tender Point Survey, D. Sinclair, T. Starz, D. Turk Univ. of Pittsburgh, School of Medicine
Tender pointsUsually described with a number of kilos of pressure
In reality no real guidelines
11 of 18 tender points
Fraught with controversy
: Comorbid conditionsIrritable bowel syndromeChronic fatigue syndromeMigraine headache syndromeSleep disorderAltered cognitive functionTM problems
Differential diagnosisPolymyalgia rheumaticaDermato or polymyositisHypothyroidismRheumatoid arthritisLyme diseaseToxic myopathies secondary to statin exposure or fibric acid
DefinitionsPolymyalgia Rheumatica (PMR) Inflammatory condition of unknown etiology.Giant Cell Arteritis (GCA) Vasculitis of medium or large arteries of unknown causeThey often occur together so are suspected to have the same underlying pathophysiology.
PMRDiagnosis is difficult and uncertain as the symptoms are non-specific. As such incidence is hard to predict.Shoulder and/or pelvic girdle pain with early morning stiffness forms the polymyalgic syndrome.There is no diagnostic test and is in many ways a diagnosis of exclusion.
Bird Criteria (3 or more)Bilateral shoulder pain or stiffnessOnset of illness less than 2 weeksInitial ESR >40Morning stiffness >1hrAge 65 or olderDepression and/or weight lossBilateral tenderness in upper arms
PMRTake care not to miss other causes of the symptomsPMR less likely to be the cause if: incomplete response to steroids, muscular weakness, prominent peripheral joint symptoms, lack of morning stiffness, unilateral symptoms.ESR/CRP are usually raised, normochromic normocytic anaemia is common, LFTs esp AlkP may be abnormal.
PMRIts complications are debility due to pain.20% will develop GCAResponse to steroid is rapid though some patients are hard to wean off steroids.
GCA3 times more common in femalesAlso known as temporal arteritisAffects cranial branches of arteries from arch of aorta and branches of ophthalmic artery.Associated with critical ischaemia : stroke blindness.These can occur very early in the disease.
- GCARare if
GCADouble vision or amaurosis fugax (transient painless loss of monocular vision of variable duration)Can have reduced visual acuity and/or visual field defects.Fundoscopy oedema/pallor of optic disc small retinal haemorrhages may also be seen
GCAInclude general CV examination check BP both arms as aortic dissection can occur.Check peripheral pulses and listen for carotid bruits.The American College Of Rheumatology (ACR) is a helpful diagnostic criteria.
ACR Criteria for GCA(three or more needed)Age 50 or greaterNew type of headacheClinically abnormal temporal artery (thickened, tender or nodular decreased pulsation)ESR 50 or >Abnormal artery biopsy necrotizing arteritis with mononuclear infiltrate or granulomatous infiltrate usually with multinucleated giant cells!
GCAInflammatory markers are not always raised do not withhold treatment if strong clinical suspicion.As PMR normochromic normocytic anaemia is common as are abnormal LFTs Use of temporal artery biopsy is debated - + confirms GCA but negative does not exclude due to skip lesions
GCALarge artery complications commonAortic aneurysm or dissection occurs in 18%!!!Patients with GCA have a higher risk of stroke/MI/CHF.A variety of neurological complications can also ensueEarly adequate dose steroids prevent most complications.
GIANT CELL ARTERITISPresentationHeadacheScalp tendernessThickened temporal arteriesJaw claudicationAcute visual lossWeight loss, anorexia, fever, night sweats, malaise & depression
GIANT CELL ARTERITIS Temporal Artery BiopsyArteries have skip lesionsultrasound/Doppler may help identify involved areasIf positive, confirms diagnosis helpful in management of future diseaseIf negative, doesnt exclude diagnosis, but need to think about an alternative diagnosis
Management of PMR and GCAFollows pattern of induction, maintenance and reduction of treatment.Most evidence for steroid dose is observationalOral prednisolone is the most common treatment used.
Management of PMR and GCAPMR no signs GCAPrednisolone 10-20mg od (after baseline investigations)70% improve in 1/52Inflammatory markers improve in 3-4/522) GCA without visual disturbance40-60mg prednisolone odR/V at 48hrs sooner if visual disturbance
Management of PMR and GCA3) GCA with visual disturbance- Medical emergency 60mg prednisolone urgent admission to ophthalmologists
MaintenancePMR and GCA continue dose steroid for up to 4 weeks then can be decreased every 2-4weeks 5mg a time in GCA and 2.5mg in PMR.When on 10mg decrease every 4-6weeks by 1mgWhen on 7mg maintain at this for 12months before reducing further.Steroids should only be stopped if proximal pain and morning stiffness have resolved.Helpful to monitor CRP/ESR during aboveCan relapse requiring step up in steroid again
Who and when to Refer?GCA any with visual symptoms even if on treatment should be referred on that day to ophthalmology.Other patients with GCA routine referral to rheumatology or general medicine for shared care.PMR can be managed in GP settingConsider secondary care referral if doubt as to diagnosis, poor response to treatment or adverse effects with steroid treatment.
ConsiderationsDont forget long term risks of steroid treatment especially if used for 12-24+monthsFrequent BP and bloods should be offered Osteoporos is important considerationEncourage increased activity
PMR pain+stiffness proximal muscles GCA vasculitis headaches/scalp tendernessGCA vasculitis headaches/scalp tendernessGCA can cause irreversible blindnessESR + CRP are commonly raised in GCA +PMRDramatic response to corticosteroids in bothRelapse common during steroid reductionPatients require long term steroids thus use osteoporosis prophylaxis as per local guidelines.