Rheumatology Flashcards
sx of arthritis clinically
Cardinal Features:
- Pain
- Swelling
- Redness
- Heat
Tenderness
Stiffness
Crepitation
Functional Impairment
number of joints affected
Mono - arthritis
Oligo - (2-4) joints
Pauci - 5
Poly - 6+ joints
characteristics of RA
- chronic
- disease course may begin 10 years before the “onset”:
- initial onset: RF+, Anti-CCP+, increased CRP
- pre-symptomatic phase: synovial inflammation
- disease onset: pain, inflammation, fatigue, damage
peak age: 25-45 years, more women, improves during pregnancy **
** majority of morbidity is d/t complications: heart disease, infection, immune complex malignancies **
Clinical Features:
- major hand deformities: have “ulnar deviation” and swelling in the PIPs
- joints space narrowing and erosions along with osteopenia in areas of inflammation symmetrically
- systemic manifestations are varied!
immunopathogenesis of RA
Type III Hypersensitivity (Immune complex deposition): RA is a localized immune complex disease
- inflammation is driven by acute phase reactants like CRP, fibrinogen and others - –> the greater the inflamm. the less viscous the plasma is and cells rapidly fall out of suspension
- CD4 T cells are activated by APC –> stimulate B cells to form RF and anti-CCP.
- immune complexes made of RF and anti-CCP form inside the joint –> sets off complement and activates inflammation
- complement is consumed in the RA joint and activates PMNs
- complement fixates along the cells, resulting in further inflammation
- can determine inflammation from C’ fixation
- T cells then react with the complement layed down, MAC causes destruction along with TNFalpha from macrophages
(TNF-α upregulates adhesion molecules on endothelial cells, promoting leukocyte influx into the joint. It also stimulates the production of other inflammatory mediators, such as interleukin 1 (IL-1), IL-6, and granulocyte-macrophage colony-stimulating factor (GM-CSF))
NOTE: TNFalpha is a major mediator in RA!!!!
tx: anti-inflammatory drugs
TNFalpha, IL-1, IL-6, CRP
- TNFalpha, IL1, IL6 and CRP are ALL pro-inflammatory cytokines seen in RA
ACR criteria for ddx RA
At least 4 criteria must be fulfilled:
- Morning stiffness lasting > 1 hour *
- Swelling in 3 or more joint areas *
- Swelling in hand joints *
- Symmetric joint swelling *
- Rheumatoid nodules
- Rheumatoid factor
- Erosions or osteopenia on hand x-rays
- Must be present > 6 weeks
- none of them are occasional sx
RA vs OA
RA: symmetric involvement
- most damage done is in the joints of the fingers, particularly the MCP and PIPs (NO DIPs)
- lots of damage is knees, wrists, ankle, feet, MTPs
- less damage done in elbow, AC, shoulder, C-spine
- ** seen in morning, decreases with activity!! ***
OA: weight bearing joints
- more degenerative, in DIPs
- involves larger joints such as the shoulder, knee, hip, PIPs, DIPs, L-spine, cervical spine
- ** seen with INCREASED activity!!!! **
pannus joint
- inflammation w/in the joint, looks like “ragged red fibers”
- T cells and lymphocytes have taken over the joint space, and a lot of cellular debris that is densely packed
- these appear as big swollen, erythematous joints
two best modailities for ddx RA
- Ultrasound (cheapest!)
- increased capillary permeability, results in increased fluid.
- US enhances fluid imaged
- better to ddx level of inflammation
- can see erosions present along with fluid in white lines - MRI
- utilizes H+ ion in order to image: detects fluid that is present
- areas of swelling or inflammation are white d/t fluid present in the joint space
- can detect synovitis and vascularity
** both best for ddx, level of inflammation, synovitis, tenosynovitis and extent of erosion
plain radiographs are not best way to determine the inflammation present
- water is the reason that U/S and MRI work
- Conventional X-ray radiography can be useful in the diagnosis of RA if erosions or juxta-articular osteoporosis are present, but is of little value for detecting synovitis, tenosynovitis, or soft tissue inflammation
extra-articular manifestations of RA: skin changes? organ changes? bone changes? vascular changes?
- Rheumatoid Nodules: bumps in the skin (always RF+)
- Sjogren’s Syndrome: Sicca sx (dry eyes, dry mouth, vaginal dryness, tracheo-bronchial dryness)
- +SS-A and SS-B - Felty’s Syndrome (rheumatoid arthritis, splenomegaly and neutropenia. )
- Vasculitis: microhemorrhages in fingernails, small vessel vasculitis
- Rheumatoid Lung
- Cardiac Disease
- Neuoromyopathy/ myelopathy
- Inflammatory Eye Dx: scleritis, and scleromalacia (“necrotizing scleritis”)
- Osteoporosis: see increased kyphosis of spine
- Lymphadenopathy
- Hyperviscosity
- Cryoglobulinemia
- Dermatologic
- Amyloidosis
two reasons for myelopathy of in RA of the cervical spine
- transverse ligament of axis begins to fail –> results in translation of atlas on the axis
- Nerve impingement b/w C1 and C2
Sx of spinal cord damage:
- Diminished motor power in arms & legs
- Severe neck pain often radiating to the occiput
- Dysesthesias of the fingers & feet
- A “marble sensation” in the limbs & trunk
- Jumping legs, due to spinal automatism
- Disturbed bladder function
lab findings in RA?
Rheumatoid Factor + (Ab directed against Fc portion of IgG –> makes immune complexes)
Anti – CCP Antibody +
ANA +
Elevated ESR or CRP
CBC: Anemia Thrombocytosis Hyperglobulinemia Leukopenia/Granulocytopenia
Glucose in body fluids – very low
- high level of anti-CCP along with high levels of anti-RF = 96% chance of having RA
Note: Value of RF is good tool for ddx and severity
- high titer = good predictor of erosiveness
- but ESR may be better predictor of severity of RA
Both tests are highly specific for RA (91% and 96%, respectively), and the absence of both markers is predictive of non-RA in approximately 80% of patients. However, neither marker is particularly sensitive. (only 39% of patients with early RA have both present)
tx for RA?
Two Goals:
- alleviate pain
- slow rate of joint damage
pharmacologic is most effective in early and late phase of RA
- NSAIDs (mainstay of tx):
- don’t halt degree progression
- have high toxicity, may be costly - Corticosteroids:
- chronic use assoc. w/ many side effects
- should only be used for acute flare-ups - DMARDs: Disease modifying antirheumatic drugs
- discontinuation rate is high
- need for continued monitoring
- delayed onset of action
ex: Methotrexate, Leflunomide, HCQ, sulfasalazine - Biologic Agents: $$
- opportunistic infections are major SE (TNF-alpha blockers) - Analgesics ***
- absolutely necessary to control pain and improve fn.
** also encourage PT/OT, weightloss , exercise, rest, splints, etc.
“swan neck” vs. “Boutonniere” deformity
- boutonniere: joint inflammation along the extensor hood - results in breaking of extensor hood and the finger becomes flexed at the PIP joint, extended at DIP
swan neck: enthecitis rupture, tendon pulls out of tip of finger, have flexed DIP
** both are seen in RA!!
screening for RA?
high RF = gives severity of disease – more likely in developing vasculitis
Cyclic citrullinated peptides: CCP test = screening test for RA,
** best test
- when have Anti-CCP positive and RF positive it increases the specificity
- absence of both tests is predictive of non-RA
Highly specific, lower sensitivity:
- neither marker is very sensitive, but if have markers then its specific (high positive predictive value)
- only 63% of patients with early RA are positive for either IgM RF or anti-CCP antibodies and 39% of patients with early RA are positive for both markers
ESR and C-reactive protein (CRP) — two markers of acute phase response — do not discriminate well between RA and non-RA arthritis in patients with early arthritis and are not regarded as predictive of persistent, erosive RA
ACR remission criteria for RA?
- AM Stiffness < 15 minutes
- No fatigue
- No joint pain
- No joint tenderness or pain on ROM
- No Soft Tissue Swelling in joints or tendon sheaths
- ESR 2 consecutive months
two etiologies for developing OA?
- Progressive deterioration & loss of articular cartilage, leading to loss of normal joint structure & function
Primary:
- Aging or Idiopathic
- Genetic: Nodal O.A.
Secondary:
- Due to disorders that damage joint surfaces
Clinical features of OA? major contrast b/w OA and RA?
OA:
- most common arthrotpathy, seen in older age
- deep achey pain
- primary pathology involves cartilage!
- defect in repair
- ** non inflammatory **
- involves active enzymatic factors
- multi-factorial etiology
OA is noninflammatory, its a degenerative disease vs. RA which is inflammatory!!!
- RA involves synovium, OA involves the cartilage and is noninflammatory
- OA is an enzymatic degredation of joint space rather than immune degredation!!
- morning stiffness is indicative of RA!
pathology of OA joint
= Progressive deterioration & loss of articular cartilage, leading to loss of normal joint structure & function
- *2 principle mechanisms initiate O.A.
1. Damage to normal articular cartilage by physical forces (macro or microtrauma) - Chondrocytes react: Release degredative enzymes and Inadequate repair response
- Fundamental defective cartilage fails under normal joint loading
- Type II collagen gene defect (Ochronosis)
- Ochronotic Cartilage (pigmented and defective)
Pathologic characteristics:
- altered chondrocyte function:
- loss of cartilage, thinning
- subchondrial bone thickening and sclerosis
- remodeling of bone
- marginal spurs: osteophytes
- cystic changes in subchondral bone
- mild reactie synovitis
subsets of OA
- Generalized O.A.
- Nodal O.A.
- Heberden’s nodes (DIPs swelling)
- Bouchard’s node (PIP swelling) - Spondylosis
- translation of bone, resulting in neuro problems - Erosive O.A.
- on Xray, see lucency and holes filled w/ fluid - Inflammatory O.A.
- see swollen joints - Diffuse Idiopathic Skeletal Hyperostosis (DISH)
- increased bone spurs - Chondromalacia patellae:
- see increased Q angle in knees
- see shredded appearance of patella, rides on the medial aspect of the chondyle
Heberden’s vs. Boushard’s nodes
- nodal osteoarthritis in hands -
Heberdens nodes: DIP
Bouchard’s nodes: PIP
1st CMC joint: base of thumb
Osteophyte vs. Syndesmophyte
= “bone spurs”
- osteophyte: chronic irritation results in spondylosis, see bone spur formation that looks like teeth or clam shell on either side (seen in OA)
- can see downward pointing osteophytes in the C-spine
- syndesmophyte: from inflammatory disease, ligaments ossify, resulting in appearance of bamboo spine - seen more in ankylosing spondylitis
spondylosis
- seen in OA: causes back pain
- results from degenerative OA of joints between the center of spiral vertebra and neural foramina –> results in space becoming narrowed and compression of nerve
Spine (spondylosis) involves disc degeneration & facet involvement
- Cervical
- Thoracic
- Lumbar
- Spinal Canal Stenosis
- DISH
Side note:
- Pars interarticularis fails:
1. spondylolysis: pars bridge fails
2. spondylolisthesis: L5 translates on S1 d/t spondylolysis
- Pars interarticularis fails:
management of OA
- pain control, improve function, enhance health/quality of life, avoid Rx with related SE’s
- no cure to OA!
- education, weight reduction, PT/OT
- NSAIDs/ analgesics
- SMOADs (structure modifying anti-osteoarthritis drugs) - ex. MMPI, doxycycline
relationship of OA with CPPD
- THINK CALCIUM PYROPHOSPHATE JOINT CRYSTALS WHEN INFLAMMATORY OSTEOARTHRITIS FAILS TO RESPOND!!!!
- think pseudogout, if not responding to normal OA tx
- will see CPPD depsoition in joints, along with hooked osteophytes and positive bifrengence
risk factors of OA?
Age Obesity Genetics Gender: women have more problems Menopause
Local factors: Muscle Strength Joint proprioception Repetitive use Configuration of joint Trauma
presentation of OA?
Joints involved:
- pain in lumbar spine, knee, hip, Cervical spine, PIPs/DIPs, first metatarsophalaneal (MTPs)
- Knees are most common lcoation!!!
- in spine see spondylosis: involving disc degeneration and facet involvment
Sx of OA:
- Insidious onset
- Joint pain associated with movement
- Limitation of motion/ function
- Minimal stiffness after rest
- Referred pain
- Acute flares suggest another diagnosis
- Systemic symptoms are rare
Physical Signs:
- bony changes at joints
- creptius
- no inflammation within the joint, or seen w/in fluid
- instability
- limited ROM
- joint line tenderness
- spasm or atrophy of adjacent mm.
why is OA painful?
- not pain in cartilage, rather the things surrounding it -
Cartilage is avascular & aneuritic: Synovitis Joint capsule/ligament stretching Periosteal irritation from osteophytes Trabecular microfractures Muscle spasm Intraosseous hypertension
OA Labs/DDx testing?
- No specific diagnostic test:
- ESR and RF are appropriate for age
** synovial fluid is class 1 (non-inflammatory)**
Xrays (best tool):
- cartilage loss/joint space narrowing
- subchondral sclerosis
- osteophytes at joint margins
- subchondral cysts
What is commonly seen on Xray?
- narrowed joint space,
- osteophytes,
- increased density of sub - chondral bone
- bone cysts
DISH
- disease associated with OA
Diffuse Idiopathic Skeletal Hyperostosis – osteophytes go wild (but remember, non-inflammatory) - see lots of bone spurs!
- exuberant osteophytosis of spine/spans > 3-4 vertebral segments
- preservation of disc spaces
- ***ligamentous calcification
- > 5 yoa
- M > F
- **associated with DM
- ant. cervical osteophytes - - dysphagia
cause:
- osteoblasts start to ossify and cause tendons to become bone, results in tons of osteophyte production
Commonly seen…
- T spine is the best place to look for DISH
- upward pointing lumbar osteophtres are characteristic of DISH
- ligamentous calcification at entheses
- foot and ankle entheses
- see “candle wax ossification” - DISH seen most often on the right!
NOTE: if have dextracardia, then will have left sided DISH
environmental factors of OA?
- fluoride
- synthetic retinoids (Accutane)
- IGF-1
- obesity/metabolic conditions
essentials of ddx of OA?
Commonly secondary to other articular disease
A degenerative disorder without systemic manifestations
Pain relieved by rest; morning stiffness brief; articular inflammation minimal
X-ray findings: narrowed joint space, osteophytes, increased density of sub - chondral bone, bone cysts
cholesterol crystals
- rare
- lipid crystals DO polarize light: shine all difft. colors (like Lisa Frank)
- can be seen in lipid laden joint effusion or RA and chronic infection
monoclonal protein crystals
- rare
- areas of lytic diseases such as MM, that have S100A4 Abs
- reacts w/ inflamm. cells and damages cartilage, can form joint mice
calcium phosphate hydroxyapatite cyrstal
- rare
- results in calcific tendinitis
- calcium phosphate crystals can be aspirated from joint
** crystals DO NOT react to polarized light **
calcium oxalate crystal
- rare
- results in oxylosis (deposition of oxalate) of the spine, hand and nephrolythiasis*
oxylosis ** affects the terminal tufts of the joints ** (cotton ball deposition)
- can cause oxylosis of the retina
- can cause renal stones
two most common crystal disease?
monosodium urate = gout
** YIPA (“negative bifrengence”, when in plane of light)
calcium pyrophosphate = pseudogout (CPPD)
** glows blue in the plane of light
Pathogenesis:
crystal formation and deposition in the joint –> Abs coat the crystal –> inflammation of the joint results in hot swollen joint
epidemiology of hyperuricemia/gout?
- increased w/ age
- risk increases w/ degree of hyperuricemia (imp. marker of inflammation)
- middle-aged males
- incidence increases in women after menopause
- 2-3% of adult male population are affected by gout
** NOTE: hyperuricemia is a marker of atherogenesis: should alert clnician to overall increased risk in CVD!!! **
uric acid production
- body is always producing uric acid –> contributing to uric acid pool
- normally the pool isn’t too big, and is handled by the kidneys –> excretion
Pathogenesis of hyperuricemia:
- uric acid production increased: 10% cases
- leukemias/lymphomas**
- ethanol**
- ineffective erythropoiesis
- cytotoxic drugs (when instituted, must give something first to shut down uric acid cycle)
- glycogen storage diseases
- G6PD deficiency - inability to excrete uric acid: (decreased renal excretion) - 90% cases
- CKD
- dehydration
- acidosis
- low dose salicylates
- diuretics
- cyclosporine
- hypoTH
acute gouty arthritis..
- what is name of first attack?
Abrupt onset & mono - articular inflammation –> episodic disease
** SEE sudden changes in uric acid levels = best predictor of an attack
- 1st attack “podagra”: start out monoarticular inflammation (red swelling of one joint)
- Attacks are polyarticular with time
- Tophi indicative of chronic urate overload
- Needle shaped negatively birefringent MSU crystals
Clinical course of classical gout…
- what is tophi?
Stage 1: see hyperuricemia but no arthritis
Stage 2: see hyperuricemia, with acute intermittent arthritis w/ increasing severity
Stage 3: hyperuricemia with chronic arthritis and joint attack
- “tophi”: bone and cartilage destruction (looks like a small white boil)- toothpaste like substance, more than subcutaneous, located w/in the joint
- see oligoarthritis in stage 3
allopurinol use for gout?
allopurinol is contraindicated in acute gout!
but is good for prophylactic use to decrease uric acid
NOTE: never stop allopurinol if they are on it and have an acute attack, just give indomethacin to get it down
Clinical features of pseudogout?
= Calcium Pyrophosphate Deposition Disease (CPPD)
- most commonly occur in the knee, wrist, hip, shoulder
- inflammatory arthritis, increased incidence with age
- see rhomboid, weakly positive birefringent CPPD crystals
- see chondrocalcinosis
assoc. medical conditions:
- hyperPTH
- hypoTH
- hemochromatosis
- Wilson’s Disease
- O.A.
NOTE: calcification of the cartilage is pseudogout!
management of gout?
- arthrocentesis (using syringe to drain it)
Acute tx:
- NSAIDs: indomethacin is most effective (best for acute attack)
- corticosteroids
Prophylactic tx:
- Uric acid lowering agents:
1. xanthin oxidase inhibitors: allopurinol, febuxostat, (don’t give during acute attack)
2. uricosuric agents: probenicid, fenofibrate, losartan
3. uricase: procloticase
Dr. Told’s guide to tx crystal diseases:
1. Asymptomatic Hyperuricemia = no treatment but check diuretic use and ASA use and niacin use.
- Acute Attack = NSAID’s Indomethacin still is the best.
- COX-2’s - OK- others are too slow.
- CORTICOSTEROIDS= Good for poor renal function and Injectible use. - Uric Acid Lowering =
- Colchicine can be a “Crap Shoot”
- Allopurinal and Probenecid & Fabuxostat are best bets.
dieting and gout?
even going on a strict purine lowering diet and no alcohol (nearly purine free) will only decrease uric acid by 1MG%
high purine diet: shrimp, scallops, beef, rabbits, soy beans, chicken
Sjogren syndrome
= keratoconjunctivitis SECCA (KCS)
SECCA sx:
dry eyes and dry mouth (damage to eye surface, increased tooth decay, periodontal disease)
- parotid gland enlargement, salivary duct stone, dental caries
- dry vaginal mucosa
- Primary type:
- exocrine gland dysfunction, occurs in isolation, dry eyes and mouth - Secondary type:
- assoc. w/ other disease: RHD, RA, SLE, PBC, scleroderma, polymyocitis, hashimotos thyroiditis, polyarteritis, interstitial pulmonary fibrosis
Criteria for classification:
- Autoimmune exocrinopathy
- Ocular symptoms
- Oral symptoms
- Ocular signs (Schirmer’s or Rose Bengal)
- Characteristic histopathologic features
- Salivary gland involvement by testing
- Autoantibodies (RF, SS-A, SS-B)
- The presence of 4 of 6 criteria has a sensitivity of 93.5% & specificity of 94% **
Labs seen w/ Sjogrens?
CBC= ANEMIA, LEUKOPENIA, EOCINOPHELIA
HYPERGAMMAGLOBULINEMIA
(Polyclonal)
RA POSITIVE 70% OF THE TIME
ANA POSITIVE 95% OF THE TIME
clinical test for parotid gland enlargement
- Schirmer’s test: put paper unerneath the tear ducts, if positive test will see very little tear production
- Wetting of < 5mm/5min of the filter paper strip is abnormal & indicates reduced lacrimal secretions. - parotid gland enlargment
- Rose Bengal staining + indicates inflammation & irritation of the conjunctival layer. (staining around the iris)
nucleolar/nuclear SSA pattern
associated strongly w/ Sjogrens syndrome along with dermatomyositis
Anti-SSB finely speckled pattern
not as strongly associated with sjogrens - more just assoc. w/ AI diseases in general - can also be assoc. w/ SLE
ACR criteria for ddx of JIA?
- Age at onset < 17 years
- Arthritis in 1 or more joints
- Duration of disease > 6 weeks
- Type of onset of disease during the first 6 mos:
Polyarthritis
Pauciarthritis
Systemic Disease - Exclusion of other forms of juvenile arthritis
common diseases mimicking JIA?
SLE
Reactive Arthritis
Lyme Disease
Dermatomyositis
Kawasaki Disease
Rheumatic Fever
Inflammatory Bowel Dx
Hematologic Dx
Vasculitis
Septic Arthritis
Toxic Synovitis of Hip
Neoplasia
Infantile-onset multi-systemic inflammatory disease
Psychogenic Disease
sx of JIA vs. growing pains?
Growing pains:
- age 6-13 y/o
- distrubution in lower extremities: thighs, calves, shins (NOT joints)
- pain most freq. late in day/night (NOT morning)
- normal growth and development
tx: heat, massage, analgesics
Systemic Onset JIA
- what are the extra articular manifestations?
- what are tests?
- prognosis?
= “still’s disease” : very worrisome, kids look real sick
Signs/Sx:
- systemic signs/sx:
- malaise, fever, rash, adenopathy, hepatosplenomegaly, serositis, hepatitis, DIC, anemia, cardiac problems
- see a variable “fever curve”
- “JIA rash” - salmon pink, transient, cicrumscribed macular, 2-6mm pencil eraser, often distributed on trunk (chest, axilla, thighs, arms), pruritis is unusual - MSK sx:
- arthritis, myalgia, arthalgia - age of onset: usually risk of poor health
- 1/2 have recurrent episodes, 1/3 have progressive arthritis
- amyloidosis occurs in some children with this disease
Tests:
- ESR: high
- CBC: anemia, leukocytosis, thrombocytosis
- IgM RF negative
- ANA negative
Management:
- splinting to prevent deformity
- PT/OT, NSAIDs, steroids in severe cases
two types of polyarticular JIA?
> 5 joints (30% of JIA)
Subgroups:
RF (+) –> adolescent, severe, similar to adults
RF (–) –> milder disease
iridocyclitis, iritis, uveitis??
- chronic uveitis = pauci type 1
- iridocyclitis = develops in 2/3 of pauci type 1
- acute iritis = pauci II
Juvenile arthritis: JIA
juvenile idiopathic arthritis
- NOT a homogenous entity,
- no uniformed prognosis or tx
demographics:
- onset <9 y/o
- 30-50,000 kids in US
JIA subtype classification:
- Systemic Onset (10 – 15%)
- 1 or > joints involved
- extra-articular features > 6 weeks - Pauciarticular (1-5)
- Subtype I: Classic
- Subtype II: Spondylitic
- Subtype III: Psoriatic - Polyarticular >6 joints
- Rheumatoid Factor (RF) Positive
- RF Negative
Pauciarticular Disease: Type I
- 4-5 joint involvement
- age M
- most common type!
Clinical Features:
- joints < 5 involved: see knee, ankle, elbow, hip most often
- early growth abnormalities
- chronic uveitis** w/in 5 years (occurs in 1/3 cases): must have opthamologitst evaluation
- generalized inflamm. leads to early growth abnormalities
Tests:
- ESR increased of wnl
- CBC: all wnl
- RF negative
- ANA often positive (40-75%)
- HLA A2, DR5, DRw6, & DRw8
course/prognosis:
- early detection necessary!
- exacerbations/remissions
- long-term prognosis of joints is good
- complications: alteration of growth in affected limb, 2/3 haveuveitis and iridocyclitis in both eyes
- need opthamology follow up!
Pauciarticular Disease: Type II
= “Juvenile spondyloarthropathy”
General:
- age >9 y/o, males more
Clinical features:
- Peripheral arthritis primarily in lower extremities
- Enthesopathies: swollen where tendon hooks into the bone
- Acute iritis
- **Sacroiliac pain in some
- **Axial disease in some
- very poor flexibility
Tests:
- ESR: wnl or high
- CBC: wnl
- RF negative
- HLAB27 +
Course/Prognosis:
- functional outcome is good in 2/3 of cases
- 1/3 may develop spondylitis, hip and cervical problems
Pauciarticular Disease: Type III
= “psoriatic”
General characteristics:
- Age: ~8 years, F>M
- Family History of psoriasis
- Rarely systemic
Clinical features:
- Occasional severe destructive arthritis
- Dactylitis (swollen/inflamed digit): esp. tips of digits
- Asymmetric peripheral joints
- ***Psoriatic rash, nail pitting/onycholysis (psoriasis is normally extensor surfaces, silver/scaly and red)
- Flexor tenosynovitis
Tests:
- ESR varies, can be very high
- CBC: Hb/Hcg low, WBC high
- RF negative
- ANA +/-
Course/prognosis:
- Young onset (+/-) associated with iritis
- Remitting & relapsing, even into adulthood
- Occasionally severely destructive
- Occasionally spondylitis develops
HLA-B27 child w/ pain in joints?
think Pauciarticular Disease: Type II
Polyarticular JIA: RF negative
Any age, occasionally < 1 year, F > M
Clinical Features:
- Can affect any joint (K, W, A, PIP & DIP)
- Reduced neck & TMJ ROM
- Flexor tenosynovitis
+/- low grade fever
- Mild lymphadenopathy & hepatosplenomegaly
Tests:
- ESR increased
- CBC: anemia, mild leukocytosis, thrombocytosis
- RF negative
- ANA sometimes positive
Course/Prognosis:
- Variable
- May be monocyclic but prolonged with good functional outcome
- Recurrent episodes tend to cause progressive deformities
Polyarticular JIA: RF positive
> 8 years @ onset, F > M
** this is the most severe disease **
Clinical Features:
- Polyarthritis of any joint (small joints of W, H, A, F; K & H early)
- Rheumatoid nodules
- Vasculitis - uncommon & late
- arthritic malformations: dactylitis
- cervical subluxation
Tests:
- ESR increased
- CBC moderate anemia
- RF posititve (very high titer!)
- ANA may be positive
- HLA-dR4 often positive
- XRAYS show early erosive changes
Course/prognosis: - Persistent - serious joint destruction & poor function - Additional long-term hazards: C1-C2 subluxation aortic insufficiency & amyloidosis
HLA-DR4 positive
think Polyarticular JIA: RF positive
65 y/o man with lesion on head, hx of RA for 10 years, switched to biologic agent by opthamologist, skin lesion had grown rapidly, and patient had Sicca sx (dry eyes and mouth), elevated ESR, normal WBCs, increased LNs, RA pos, Anti Ro SSA pos, ANA weakly positive, temp of 99.8, and lungs with light basilar rales
cutaneous T cell lymphoma - indicated by the skin lesion, metastasized to the lungs
What is the cause of the lymphoma?
- secondary to biologic medications: needs to be taken off of the meds (even though may result in blindness)
cutaneous T cell lymphoma and RA are related.
49 y/o rancher, pain and swelling for two weeks. twisted knee, was not warm to touch, has popping sound, w/ joint line tenderness.
Xray: shows very limited joint space – thinking osteoarthritis
Do an ultrasound (will show more than an Xray in this case) - see that hyaline cartilage disappears on median side and is disrupted at the point of the second arrow
synovial fluid is clear d/t it being noninflammatory - confirms osteoarthritis
different aspiration of synovial fluid of knee?
if bloody - think acute damage/fracture
if cloudy and red, purulent - think sepsis
if yellow - think sepsis
if clear - think RA
viscosupplementation
tx for RA - in RA the fluid gets more viscous and watery - if you give a shot that makes it more thick, then it will help with RA
19 y/o female w/ difficulty swallowing and turning neck around, has hx of juvenile onset diabetes, had cystic acne and was tx with retinoids, has early morning stiffness which leaves in 20 mins after arising. ESR is slightly elevated, HbA1C is elevated (7.9). restriction of flexion and extension of mid t-spine, poor rotation in L-spine.
on Xray has bone spurs that go up, along with bone spurs on the knee.
ddx: thinking DISH (best seen on upper T spine, see fusion “candle wax” appearance) - part of the CREST syndrome
8 y/o w/ sudden onset of fever accompanied by fatigue. has developed night sweats and erratic fever pattern. Recently had neck pain with large LN’s noted in neck and was given Abs. NSAIDs reduce fever very little, but help the pain some. Pattern has been going on for 8 weeks
ddx: systemic onset JIA
- had rapid heart rate w/ S3 murmur - (signifies CHF), with cardiomegaly
- check for liver and spleen enlargement, along with hepatitis
- see rash on truncal region: salmon colored and confluent
45 y/o male w/ sudden onset of pain and swelling in right knee. had injury in high school, aggravates it with skiing. NSAIDs don’t give much relief. Gets relief with cholchicine
sudden onset = crystal disease!
(colchicine works for gout and pseudogout)!!
ddx = pseudogout, its presentation in the knee
- see chondrocalcinosis (cartilage has calcium in it)
tx: intra-articular steroids
50 y/o male with sudden onset pain in his left foot, and redness/swelling of heel and great toe. Better if elevated and drinks cherry juice, had similar event after a week-end of partying.
oligoarhtritis - 2 joints
“podagra” - 1st attack
ddx: gout = monosodium urate deposition
YIPA = negative birefrengence pattern
PIPs and DIPs vs MCPs
OA = PIPs/DIPs
RA = MCPs
most common joint affected by gout?
podagra often shows up in MTP
morning stiffness
think RA
CREST
= limited scleroderma (hardening of hands and feet)
Calcinosis Raynaud's phenomenon Esophageal dismotility Scleroderma telangiectasias
two viruses assoc. w/ arthralgias?
parvovirus B19 - acute polyarthritis, from kids w/ “slapped cheek” fever
chronic infection with hep C
both can mimic RA, but won’t have anti-CCP Abs
enthesis
where muscle joins bone
enthesitis = inflammation of this area
turbid synovial fluid
think RA
episodic intermittent attacks
think crystals!
labs for sjogren’s?
CBC: shows anemia, leukopenia
hypergammaglobulinemia
RA positive 70% of time
ANA positive 95% of time!
sialectasis
= cystic dilatation of the ducts within salivary glands
seen in Sjogren’s syndrome
extraglandular manifestations in primary sjogren’s?
fatigue, fever, lymphadenopathy
arthritis
raynaud’s, vasculitis
AI thyroiditis
pulmonary fibrosis, serositis
esophageal involvement
neuropathy
renal/bladder involvement
thrombocytopenia, lymphoproliferative disease
