Flashcards in Spondyloarthropathies, PMR, FM Deck (54):
What are spondyloarthropathies?
group of inflammatory arthropathies that share distinctive clinical, radiographic and genetic features:
- inflammatory axial spine involvement
- asymmetrical peripheral arthritis
- enthesitis (inflammation of sites where tendons and ligaments attach to bone)
- inflammatory eye disease
- mucocutaneous features
- negative RF
- high freq of HLA B27 abs
- familial aggregation
- ankylosing spondylitis
- reactive arthritis (Reiter's syndrome)
- psoriatic arthritis
- enteropathic arthritis (crohns and UC)
Disease associations w/ HLA-B27?
- ankylosing spondylitis: over 90%
- reactive arthritis 85%
- reiters syndrome 80%
- IBD 50%
- psoriatic arthritis 50%
- whipple's disease 30%
What is ankylosing spondylitis (AS)? Extra-articular manifestations?
- chronic inflammatory disease of jts of the axial skeleton
- higher incidence at higher latitudes, Scandinavian countries
- changes seen in sacroiliac jts and hips
- inflammation around enethesis
- extra-articular manifestations:
aortic valvular disease
restricted chest expansion
Diagnostic features of AS?
- insidious onset low back pain for longer than 3 months
- improves w/ exercise not rest
- morning stiffness for longer than 30 min
- awakened by pain during the 2nd half of the night
- alternating buttock or posterior thigh pain
- sites of enthesitis
- sacroiliitis on x-ray
diff in characteristics of inflammatory back pain and mechanical back pain?
- inflammatory: prolonged AM stiffness and max pain is early AM, exercise improves sxs, chronic duriation, 9-40 age at onset, on xray: sacroiliitis, vertebral ankylosis, syndesmophytes
- mechanical: minor am stiffness (less than 45 min), late in day max pain, exercise worsen sxs, duration can be acute or chronic, age at onset: 20-65 yrs, X-ray: osteophytes, malalignment
What willl you see on radiograph of ankylosing spondylitis?
- single most impt imaging technique for dx and f/u
- early changes are at sacral iliac jts: erosion and sclerosis
- involvement of apophysial jts of spine
- ossification of the annulus fibrosus
- calcification of the anterior and lateral spinal ligaments
- squaring and generalized demineralization of the vertebral bodies
- radiographic changes of the spine and are referred to as the bamboo spine
Characteristics of AS?
- typical pt is males aged 20-40
- sxs appear gradually and are usually not specifc to AS, time to correct dx is 8.5-14 yrs
- first sxs are typically chronic pain and stiffness in the middle spine assoc w/ referred to one or the other buttock or in the back of the thigh
- assoc w/ morning stiffness that improves w/ exercise
What is the modified New York criteria for dx for AS?
1. limited lumbar motion
2. low back pain for longer than 3 months - improved w/ exercise, not relieved w/ rest
3. reduced chest expansion
4. bilateral grade 2-4 sacroiliitis on xray
5. unilateral grade 3-4 sacroiliitis on xray
* definite AS if: criteria 4 or 5 plus 1, 2 or 3
What is Enthesitis?
inflammation of the entheses, sites where tendons or ligaments insert into the bone
What are extra-articular manifestations that may occur in AS?
- skin rashes (presents like psoriasis - onchylosis, nail pitting)
- eye inflammation: esp uveitis
- lung involvement
- cardiac involvement: w/ aortic valve disease
How common is anterior uveitis in AS?
- 30-40% of people w/ AS will experience iritis at least once
Early presentation of AS?
- sxs: LBP, stiffness, fatigue
- extra auricular manifestations: ocular skin/nail enthesitis
- disease progression: sacroiliitis
- morbidity/mortality: pain, fxnl limitation
Moderate AS presentation?
- sxs: spinal limitation, fxnl limits, night pain
- extra-articular manifestations: chronic uveitis, IBD
- disease progression: hip involvement, spondylitis
- morbidity/mortality: AS complications, drug toxicity, comorbidities
Severe AS presentation?
- sxs: spinal immobility
- extra-articular manifestations: aortitis, retristrictive lung, heart block
- disease progression: bamboo spine
- morbidity/mortality: fracture, death
What is Reactive arthritis? Triad? Diff types? Complications?
- acute inflammatory arthritis occurring 1-3 wks after infectious event (GU, GI, idiopathic)
- triad: arthritis+urethritis (cervicitis)+ conjunctivitis (classic triad found in less than 1/3 of pts)
- post-veneral onset (Reiters): MC and more common in males 5:1
- post-dysenteric: less common, equal in M and F
- course: usually self limiting (less than 6 mos), can become chronic w/o tx
- complications: acute anterior uveitis 5%, myocarditis, fasciitis
- decreasing incidence in HIV era (condom use)
Presentation of Reiter's syndrome?
msk signs and sxs:
- enthesitis- heel tendonitis
extra-articular signs and sxs:
- GU: dysuria and pelvic pain
- oral ulcers, tongue lesions
- palate erosion
- rashes- pustules, keratoderma blenorrhagica
- nail changes - dystophy
- genital lesions
- plantar periostitis
Infectious triggers for reactive arthritis?
- enteric infections:
- urogenital infections:
chlamydia trachomatis, C. pneumoniae
What is psoriatic arthritis? How common is this? Presentation and course?
- chronic inflammatory arthropathy in setting of psoriasis
- etiology and genotype unclear
- 1-5% of US pop has psoriasis: 5-42% of these develop psoriatic arthritis (skin usually preceded jts):
frequency of PsA increases w/ disease severity and duration, est 350-400,000 pts in USA
- nail changes: pitting, dystrophy, onycholysis
- course: chronic, destructive arthritis in 30-50%
Clinical characteristics of psoriatic arthritis?
- inflammatory arthritis in DIPs, PIPs (pencil and cup deformity)
- asymmetric arthritis
- sausage digits
- nail pitting (onycholysis)
- no rheumatoid nodules
- RF test negative
- erosive arthritis w/o osteopenia
- sacroiliitis, often asx
- paravertebral ossification
Tx for all the spondyloarthropathies?
- tx sxs w/ NSAIDs initially
- PT, stretching and exercises to preserve spine and jt fxn
- maintain good posture
- sulfasalazine, methotrexate found to be beneficial
- anti-TNF aka TNF inhibitors (Remicade, Humira, Enbrel)
- prevent eye complications by early recognition and tx
Use of NSAIDs in tx spondyloarthropathies?
- effective for: inflammatory back pain, spinal stiffness, peripheral arthritis, enthesopathy
- no evidence that they inhibit disease progression
- FDA approved NSAIDs for AS: indomethacin, indomethacin-SR, EC ASA, naproxen, sulindac, diclofenac
- anecdotal reports and few studies suggest that specific NSAIDs may be more effective:
phenylbutazone: limited availability: risk of agranulocytosis
indomethacin: esp in long acting form
diclofenac: as effective as Indocin
When should you consider DMARDs for spondyloarthropathies?
- antiinflammatory therapy is insufficient to control sxs
- progression of inflammatory axial disease noted
- active persistent polyarthritis
- uncontrolled extra-articular disease:
What is Polymyalgia Rheumatica?
- PMR is an inflammatory condition of unknown etiology
- characterized by aching, stiffness in shoulder and pelvic girdles and the neck
- occurs in people older than 50
- usually responds to low doses of steroids
- is related to GCA, w/ bx proven GCA present in 4-21%
Epidemiology of PMR?
- prevalence of 52.5 cases/100,000 persons aged 50years and older
- incidence increases w/ age, peaks at 70-80yrs
- females greater than males in all age groups (2:1)
- higher incidence at higher latitudes, scandinavian countries
- rarely reported in blacks, but appears to have same presentation course and response to tx
Dx criteria for PMR?
criteria set a:
- pts 50yo or older
- bilateral aching and stiffness for more than 1 month and involving 2 of following areas: neck or torso, shoulders or proximal regions of the arms, hips or proximal aspects of the thighs
- ESR greater than 40mm/hr
- exclusion of other dx except for GCA
- presence of all tehse criteria defines PMR
criteria set b:
same as above, + morning stiffness lasting more than 1 hr, rapid response to prednisone
criteria set c:
criteria from above + depression and/or wt loss, bilateral upper arm tenderness, dx of probable PMR is made if any 3 or more of these criteria are fulfulled, presence of any 3 or more criteria yields a sensitivity of 92% and specificity of 80%
Etiology of PMR?
- probably polygenic in which mult enviro and genetic factors influence susceptibility and severity
- possible infectious triggers:
viruses: adenovirus, RSV, parvovirus, parainfluenza
bacteria: mycoplasma, chlamydia pneumoniae
- genetic component probable:
HLA-DRB1*04 and 1*01 appear to be most assoc w/ susceptibility to PMR
-genetic polymorphisms of additional genes involved in initiation and regulation of inflammatory rxn
- possible subclinical vasculitis
Clinical manifestations of PMR?
- persistent pain (for at least 1 month): aching and morning stiffness in neck, shoulder and pelvic girdles lasting 30 min, discomfort is bilateral, worse w/ movement, and usually interferes w/ ADLs
- shoulder pain is presenting sign in 70-95%, hips and neck 50-70%, pain usually radiates distally towards elbows and knees
- systemic signs seen in 1/3: fever, malaise, fatigue, anorexia, and wt loss
- exam reveals little evidence of proximal jt swelling or tenderness, MRI studies have shown subdeltoid and subacromial bursitis are more prominent than actual jt synovitis
- distal manifestations also seen in 1/2 of cases:
nonerosive, self-limiting, asymmetric peripheral arthritis (knee/wrist), carpal tunnel, distal extremity swelling and pitting edema over dorsum of hands and wrists, ankles and feet
Labs for PMR?
- ESR will be above 40mm/hr (nl in up to 20%)
- CRP less influenced by other factors, may be more sensitive and direct measure
- modest anemia of chronic disease in 2/3
- mildly abnormal LFTs in 1/3
- rheum factor and ANA usually negative
- CK and CPK enzymes are normal
DDx for PMR?
- SLE: look for pleuritic and pericarditis, leukopenia and thrombocytopenia, check for anti-dsDNA and anti-ENA abs
- RA: small jts of hands/feet, only partially responsive to steroids, considerable overlap b/t PMR and seronegative RA
- Polymyositis: symmetric proximal muscle weakness, pain not prominent, elevated CK, ALP, abnorm EMG, myositis on muscle bx
- late onset spondyloarthropathy
- malignancy: solid (kidney, ovary, stomach), heme (Myeloma, primary amyloidosis)
- infection: bacterial endocarditis
* lack of adequate response to prednisone and presence of atypical features should make one consider these
Tx and course of PMR?
- corticosteroids are DOC (10-20 mg/day) for 2-4 wks, then taper off
- trial of NSAIDs for 2-4 wks if mild
- complete or nearly complete resolution of sx is seen in a few days - absence of improvement should cause one to ? dx
- relapses do occur, more frequent in 1st 1-2 yrs
- follow ESR or CRP
- tx for 1-2 yrs is often reqd, sometimes longer
- watch for corticosteroid adverse effects
- methotrexate being used in refractory cases
- methylprednisolone had similar efficacy and fewer adverse effects
What is GCA?
- chronic vasculitis of medium and large vessels
- more common in people older than 50, incidence peaks at 70-80
- affects women more often 2:1
PP of GCA?
- vasculitis of extracranial branches of aorta, spares intracranial branches
- transmural inflammation leads to intimal hyperplasia - luminal occlusion
- sxs are due to end organ ischemia
Etiology of GCA?
- likely influenced by multiple genetic and enviro factors closely tied to PMR
- 1 pt may have both: 50% w/ GCA have PMR, 10% w/ PMR have GCA
- no evidence of autoabs
- cellular immune response involving T cells, APCs, macrophages
Presentation of GCA?
- onset usually gradual but may be abrupt
- most frequent sx: HA (2/3)
- usually accompanied by syndrome of systemic inflammation: fever, malaise, wt loss, anorexia (40%)
Sxs of GCA?
- HA (68%)
- jaw claudication (45%)
- transient visual sxs (16%)
- fixed visual sxs (14%)
- CNS abnormalities
- swallowing claudication/dysphagia (8%)
- tongue claudication (6%)
- limb claudication (4%)
Clinical signs of GCA?
- wt loss or anorexia (50%)
- decreased temporal artery pulsations (46%)
- fever (42%)
- artery tenderness (27%)
- erythematous or swollen scalp arteries (23%)
- large artery bruits (21%)
- fundoscopic abnormalities (18%)
Complications of GCA?
- aortic aneurysms
Lab findings of GCA?
- ESR over 50
- 22% of pts w/ bx-provven GCA have normal ESR
- therefore, normal ESR doesn't rule out GCA
- mild-moderate anemia of chronic disease
- elevated LFTs (1/3)
How do you dx GCA?
- temporal artery bx should be performed in all pts w/ suspected GCA
- sensitivity of unilateral bx: 88%
- negative predictive value: 91%
- bilateral bxs increase sensitivity, may be prudent
- imaging: MRI/MRA may be used to dx large-vessel GCA. Also may help guide bx by localizing inflammation, other possible imaging modalities include arteriography, US, and PET
Tx of GCA?
- glucocorticoids are the est tx
- initial dose: 40-60 mg prednisone daily
- slow taper after 2-4 wks if sxs controlled
- prolonged tx often reqd to avoid relapse
- IV pulse methylprednisolone (1000 mg IV dailyx 3 days) if vision loss present
- anti-platelet agents reduce ischemic events - low dose ASA recommended
- if clinical suspicion is high, tx shouldn't be delayed for bx
- temporal artery bx should be peformed as soon as possible after tx is initiated but bx results shouldn't be affected for several weeks
What is Fibromyalgia Syndrome (FMS)?
- clinical syndrome characterized by widespread muscular pain (usually chronic), fatigue and muscle tenderness
- not an inflammatory condition
- caused by abnormal sensory processing in the CNS
- people w/ FMS may be extremely sensitive to pain and other unpleasant sensations
Additional sxs w/ FMS?
- poor sleep (almost always(
- cognitive and memory problems
- numbness and tingling in fingers and toes
- irritable bladder
- TMJ disorder
- dry eyes and dry mouth
- morning stiffness
- anxiety and depression
- sxs including pain may wax and wane over time
What causes FMS?
- abnormally high levels of substance P in spinal fluid in some pts
- substance P impt in transmission and amplification of pain signals to and from the brain
- "volume control" is turned up too high in brain's pain centers
- familial tendency to develop FMS suggests genetic role
- can be triggered by physical, emotional or enviro stressors such as car accidents, repetitive injuries and certain diseases
- pts w/ RA and SLE are more likely to develop FMS
- lyme disease and OSA have been assoc w/ FMS
- sleep deprivation w/ disruption of delta-wave sleep (non-REM stage IV) is assoc w/ day-time fatigue and FMS
Who gets FMS?
- 1/50 Americans
- MC in middle aged women
- men and kids may also develop this
- pts w/ RA, SLE, and AS have higher incidence
- women w a family member w/ FMS are more likely to develop it
- widespread chronic pain (longer than 3 months) and
- 11/18 reproducible tender pts
at least 4 of: generalized fatigue, HA, sleep disturbance, neuropsych complaints, numbness/tingling, IBS
explained by no other condition
-at least 4 of the following sxs:
- generalized fatigue
- sleep disturbance
- neuropsych complaints
- numbness, tingling sensations
- Irritable bowel sxs
- xrays, blood tests, specialized scans such as nuclear medicine and CT, muscle bxs are all normal
- objective markers of inflammation such as ESR, CPR are normal
- must be distinguished from other common diffuse pain conditions such as RA, SLE, hypothyroidism and PMR
What are the problems in defining fibromyalgia?
-is it real if no clear pathophysiologic basis?
- gold std is expert opinion
- tender pts, sxs are subjective
- fewer than 11 tender pts?
- sxs are not clearly defined
- same dx criteria and dilemma for any illness lacking objective biologic markers (depression, migraine, IBS, CFS - chronic fatigue syndrome)
relationship b/t FMS and mood disorders?
- at time of FM dx, mood disorders are present in 30-50%, primarily depression
- increased lifetime and family hx of mood disorders in FM vs RA
- fibromyalgia co-aggregates w/ major mood disorder in families
Shared features of FM and depression?
- both have strong genetic predisposition and similar co-morbidity
- similar sleep disturbances
- similar cognitive disturbances
- orthostatic features, ANS dysfxn
- childhood abuse, stress
- can be debilitating
- imaging studies - unremarkable
- neuroendocrine studies: unremarkable
Is FMS a medical or psych illness?
- harmful and unproductive argument
- fruitless quandry to work out what came first
- for all pts, sxs are real and can be disabling
- need a dual tx approach targeting both physical and psych sxs
Tx guidelines for FMS?
- confirm dx, ID impt sx domains, their severity, and level of pt fxn
- eval for comorbid medical and psych disorders - assess psychosocial stressors, levels of fitness, barriers to tx - provide education about FMS, may reqr referral to specialist for full eval for psych or sleep clinic
- give all pts reassurance, give explanation - psychological factors, promote returnt to normal activities, exercise
Pharm tx for FMS?
- most pts: med trial (antidepressants, anticonvulsants), CBT, counseling, physical rehab
- no definitive cure
- best meds: tx pain and improve sleep disturbance:
TCAs: amitriptyline, cyclobenzoprine, anticonvulsants - pregabalin, gabapentin, SNRIs - duloxetine, milnacipran
- NSAIDs uneffective, long actign opioids ineffective
- benzos (diazepam and clonazepam) may be useful for pts w/ RLS or very severe sleep disturbance who haven't responded to other therapies
- trial of additional analgesics such as tramadol
Alt therapies for FM?
- alt therapies have been used but not well studied
- therapeutic massage
- myofascial release therapy
- pt self management:
schedule time to relax, including deep breathing and meditation, est routine for going to bed and waking up, aerobic exercise on regular basis
- avoid inactivity
- non-pharm tx - trigger pt injections
- stretching, strengthening
- self education: arthitis foundation
- support group
FM - prognosis and typical outcome?
- kids and individuals tx in primary care settings and those w/ recent onset of sxs generally have better prognosis
- FM doesn't herald onset of systemic disease
- there is no progressive, structural or organ damage
- primary care pts more commonly report complete remission of sxs
- most pts continue to work, but 10-15% are disabled
- there is often adverse impact on work and leisure activities
- most pts quality of life improves w/ medical management