Rheumatoid arthritis

 = chronic systemic connective tissue disease

 = type III hypersensitivity = delayed hypersensitivity
 = immune complex disease (= formation of antigen-antibody complexes with
complement fixation) with T-cell–mediated autoreactivity against a joint
component
Prevalence 0.5-1.0% of population
:
Cause: genetic predisposition;? reaction to antigen from Epstein-Barr
virus / certain strains of E. coli
Peak age: 45-65 years; M:F = 1:3 if <40 years; M:F = 1:1 if >40 years
 Pathogenesis:
o injury to synovial endothelial cells; synovitis with synovial hyperplasia
and pannus formation mediated by TNF-‫خ‬± (tumor necrosis factor ‫خ‬±)
and IL-1 (interleukin 1) leads to invasion by local macrophages,
fibroblasts, and activated lymphocytes; invasion of articular cartilage +
bone by secretion of degrading enzymes (metalloproteinases)
 Diagnostic Criteria of American Rheumatism Association (at least 4 criteria
should be present):
o morning stiffness for 1 hour before improvement
o swelling of 3 joints, particularly of wrist, metacarpophalangeal or
proximal interphalangeal joints for >6 weeks
o symmetric swelling
o typical radiographic changes
o subcutaneous rheumatoid nodules
o positive test for rheumatoid factor

 rheumatoid factor (positive in 85-94%) = IgM-antibody

 antinuclear antibodies (positive in many)
Location bilateral symmetric involvement of >3 diarthrodial joints
: (polyarthritis)
  Symmetric arthritis of multiple small hand joints in >60%
of patients at initial presentation
 EARLY SIGNS:

- MR&US (methods of choice):

- synovial hyperemia (in acute disease + exacerbation of chronic disease)

- synovial swelling (edema + cellular infiltrates)

- pannus = tumor like focal proliferation of inflammatory tissue with destruction of

cartilage and bone

- pre-erosive subcortical cysts

- joint effusion

- bone marrow edema

* Radiography (indirect & nonspecific):

- fusiform periarticular soft-tissue swelling (result of effusion)

- paraarticular osteoporosis (inactivity due to pain + local inflammatory

hyperthermia)

- translucent subchondral end plate

- widened joint space (synovial swelling + fluid)

- effacement of fat pads

- subcortical synovial cyst

- erosion (up to 47% within 1st year after onset) initially at bare area (= site of
attachment of internal synovial layer of joint capsule to bone) due to lack of
protective cartilage layer

 LATE SIGNS:

- concentric joint space narrowing (due to destruction of cartilage, formation of

scar tissue, fibrosis)

- subluxation (due to laxity of capsule + ligaments from inflammatory destruction /

due to capsular shrinkage from fibrosis + scar formation):

- mallet finger = droopy distal phalanx due to disrupted extensor tendon insertion
site

- swan-neck deformity = hyperextension at PIP + flexion at DIP

- boutonni‫¨أ‬re deformity = flexion at PIP + hyperextension at DIP

- hitchhiker deformity = flexion at MCP + hyperextension at DIP

- dislocation

- marked destruction + fractures of bone ends:

- intraarticular loose bodies

- rice bodies = subset of loose bodies resembling polished rice (made of sloughed
fibrinogen-coated infarcted synovial tissue / precipitate of fibrin + fibronectin /
core of mononuclear cells, blood cells and amorphous material)

-bony ankylosis

 @ Hand & wrist (typical)

 o Target areas:
 all five MCP, PIP, interphalangeal joint of thumb, all wrist
compartments (especially radiocarpal, inferior radioulnar,
pisiform-triquetral joints); earliest changes seen in MCP 2 + 3,
PIP 3

- marginal + central bone erosions (less common in large joints); site of first
erosion is classically base of proximal phalanx of 4th finger

- changes in the ulnar styloid + distal radioulnar joint (early sign)

- flexion + extension contractures with ulnar subluxation + dislocation

 @ Cervical spine

- erosions of odontoid process (1) between anterior arch of atlas + dens, (2)
between transverse ligament of atlas + dens, (3) at tip of odontoid process

- anterior atlantoaxial subluxation (in >6%): >2.5 mm in adults, >4.5 mm in

children during neck flexion

- cranial settling= odontoid process projects into skull base due to significant
disease of atlanto-occipital and atlantoaxial joints

- lateral head tilt = lateral subluxation = asymmetry between odontoid process +

lateral masses of atlas

- stepladder appearance of cervical spine due to subaxial subluxations + absence of

osteophytosis:

- destruction + narrowing of disk spaces

- irregular vertebral body outlines

- erosion + destruction of zygapophyseal joints

- resorption of spinous processes

- osteoporosis

 @ Ribs

- erosion of superior margins of posterior portions of ribs 3-5

 @ Shoulder

- symmetric loss of glenohumeral joint space:

 - marginal erosions at superolateral aspect of humeral head

- osteoporosis

- elevation of humeral heads = narrowing of acromiohumeral distance (2‫آ‬° to tear /

atrophy of rotator cuff)

- widened acromioclavicular joint:

- erosions at acromial + clavicular end

- tapered margins of distal clavicle

- scalloped erosion on undersurface of distal clavicle opposite the coracoid

process (attachment of coracoclavicular ligament)

 @ Sacroiliac joint (rarely affected)

- typically asymmetric unilateral distribution

- shallow erosions + mild sclerosis

- rare ankylosis

 @ Hip (rarely affected)

- often appears normal during early disease process

- pannus formation (MR imaging)

- symmetric loss of joint space with axial migration of femoral head

- marginal + central erosions, cysts, localized sclerosis

- decompression of joint effusion into iliopsoas bursa through weak anterior

capsule displacing muscle + vasculature

- rupture of gluteal tendon

- protrusio acetabuli (from osteoporosis)

 @ Knee

Location medial + lateral femorotibial compartments; bilateral symmetric

:

- diffuse loss of joint space

- osteoporosis
 - superficial + deep marginal + central erosions

- subchondral sclerosis (especially in tibia)

- synovial herniation + cysts (eg, popliteal cyst)

- varus / valgus angulation (due to crumbling of osteoporotic bone of tibia +

ligamentous abnormalities)

 @ Foot (typical)
o Target areas:
 medial aspect of MT heads (2,3,4), medial + lateral aspect of
MT5 (earliest sign); interphalangeal joints of foot (esp. great
toe); midfoot joints; talonavicular, subtalar, tarsometatarsal
joints; bilateral + symmetric

- sinus tarsi syndrome = compression of tibial nerve

- calcaneal plantar spur

- retrocalcaneal bursitis

 EXTRA-ARTICULAR MANIFESTATIONS (76%)

o Felty syndrome (<1%)
 = rheumatoid arthritis (present for >10 years) + splenomegaly +
neutropenia

Age 40-70 years; F > M; rare in Blacks

:

o Sjogren syndrome (15%)

 = keratoconjunctivitis + xerostomia + rheumatoid arthritis

- Pulmonary manifestations

- pleural effusion, mostly unilateral, without change for months, usually not
associated with parenchymal disease

- interstitial fibrosis with lower lobe predominance

- rheumatoid nodules (30%): well-circumscribed, peripheral, with frequent

cavitation
 - Caplan syndrome (= hyperimmune reactivity to silica inhalation with rapidly
developing multiple pulmonary nodules)

- pulmonary hypertension secondary to arteritis

- Subcutaneous nodules

- Cardiovascular involvement

 Pericarditis (20–50%)
 Myocarditis (arrhythmia, heart block)
 Aortitis (5%) of ascending aorta ‫آ‬± aortic valve insufficiency

- Rheumatoid vasculitis

- Neurologic sequelae

 Distal neuropathy (related to vasculitis)

 Nerve entrapment (atlantoaxial subluxation, carpal tunnel
syndrome, Baker cyst)

- Lymphadenopathy (up to 25%)

- splenomegaly

Cystic Rheumatoid Arthritis

 = intraosseous cystic lesions as dominant feature
Pathogenesis increased pressure in synovial space from joint effusion
: decompresses through microfractures of weakened marginal
cortex into subarticular bone
 Increase in size + extent of cysts correlates with increased level of activity +
absence of synovial cysts

Age as above; M:F = 1:1

:

- juxtaarticular subcortical lytic lesions with well-defined sclerotic margins

- relative lack of cartilage loss, osteoporosis, joint disruption

DDx gout (presence of urate crystals), pigmented villonodular synovitis

: (monoarticular)

Juvenile Rheumatoid Arthritis

 = rheumatoid arthritis in patients <16 years of age; M < F

Location early involvement of large joints (hips, knees, ankles, wrists,

: elbows); later of hands + feet
 - radiologic signs similar to rheumatoid arthritis (except for involvement of large
joints first, late onset of bony changes, more ankylosis, wide metaphyses)

- periarticular soft-tissue swelling

- thinning of joint cartilage

- large cystlike lesions removed from articular surface (invasion of bone by

inflammatory pannus); rare in children

- articular erosions at ligamentous + tendinous insertion sites

- joint destruction may resemble neuropathic joints

- juxtaarticular osteoporosis

- balloon epiphyses + gracile bones (epiphyseal overgrowth + early fusion with

bone shortening secondary to hyperemia)

- Hand / foot

o “rectangularâ€‌phalanges (periostitis + cortical thickening)

o ankylosis in carpal joints

- Axial skeleton

Location predominantly upper cervical spine

:

- ankylosis of cervical spine (apophyseal joints), sacroiliac joints

- decreased size of vertebral bodies + atrophic intervertebral disks

- subluxation of atlantoaxial joint (66%)

- thoracic spinal compression fractures