RHEUMATOID VARIANTS
JUVENILE RHEUMATOID ARTHRITIS
 Commonest connective
 Proliferatuve synovitis like in adults
 Rh factor present in only 20%
 Knees and wrist most common
 growth disturbance on ulnar side of wrist – producing a short ulna and 5th
metacarpus – produces ulnar deviation and subluxation
 disease onset below 5 years old causes severe growth retardation of hand
 Types:
1. polyarticular (40%) – most likely to continue into adult RA
2. mono/pauciarticular (40%) – affects <5 joints
3. systemic (Still’s disease) - intermittent high fevers,
rash,lymphadenopathy, hepatosplenomegaly, uveitis, myalgia and transient
arthritis
 Features are different to adult RA
1. shortening of ulna (due to differential epiphyseal growth)
2. flexion and ulnar deviation of wrist
3. radial deviation and loss of flexion of MCPJ
Treatment
 largely nonsurgical – splint, hand therapy are cornerstone
 synovectomy may give pain relief and shown to cause no harm
 spontaneous fusion common, corrective osteotomies may be required
PSORIATIC ARTHRITIS
 seronegative arthropathy like Reiter's syndrome, ankolosing spondylitis, arthritis
of inflammatory bowel disease
Clinical features
 Scaly erythematous rash
 Inflammatory arthritis(5-10 % of pts with psoriasis)
 Skin involvement on scalp, face and extremities( on hands may complicate
surgery , rash improves with sunlight thus operate in summer)
 Nails changes(80%) (pitting, leukonychia, crumbling of the nails)
 Only 5% of psoriatic patients have arthritis
 Features of arthritis
o May resemble RA very closely
o Fibrosis is the key feature of psoriatic arthropathy associated with
osteolytic destruction
o 95% peripheral joint inv.
o 25% polyarthritis
o osteolysis and widened joint spaces
o bone proliferation distal to the joint and tapering proximally ("pencil in
cup")
o arthritis mutilans
o opera glass hand
o ankylosis of distal joints
DIFFERENCE BETWEEN RA AND PSORTIATIC ARTHRITIS
1. nail changes and skin rash
2. fibrosis
3. Swan neck and Boutonnière deformities rare
4. involvement of the DIPJ
5. lower incidence of tenosynovitis and tendon rupture
6. no rheumatoid. nodules
7. usually asymmetric disease
Moll and Wright classification
1. Spontaneous fusions that maintain digital length
2. osteolysis with bone loss
3. Joint stiffness with RA-like deformities
DIIGITAL DEFORMITY
 flexion deformity of PIP without hyperextension of DIP
 MCP hyperextension
 Mallet finger
 MCP extension contractures
 joint fusion PIP best option
 DIP frequently involved, rarely needs Rx, spontaneously fuse
THUMB DEFORMITY
 MP flexion, IP hyperextension
 stiffness at CMC
 MP and IP joint fusions
 CMC interpositional arthroplasty
WRIST DEFORMITY
 common
 fusion and distal ulnar excision
SYSTEMIC LUPUS ERYTHEMATOSUS
Clinical
 multisystem disease
 pericarditis
 pleuritis
 renal disease
 skin lesions (erythematous maculopapular)
 raynauds
 facial erythema
 female 9:1
 young usually

diagnosis based on the American Rheumatism Association Preliminary Criteria
need 4 of 14 criteria
Arthritis
 ligamentous and volar plate laxity and tendon subluxation are the hallmarks
 leads to joint imbalance rather than joint destruction primarily
 joint deformities occur without erosive destruction
 wrist, digits and thumb most commonly affected
Treatment
 team approach
 treat the disease
 symptomatic Rx
 passively correctable deformities best treated with exercise and splinting
WRIST DEFORMITY
 soft tissue surgery assoc with high recurrence rates
 total wrist dislocation ---wrist fusion
 ulnar translocation of the wrist---partial wrist fusion with radiolunate or
radiocarpal fusion
 dorsal subluxation of the ulnar---Darrach procedure (stabilization with ECU)
DIGITAL DEFORMITY
 MP ulnar deviation and volar subluxation
 weak extension
 Rx arthroplasty with silicone implant
 consider tendon realignment but note high recurrence rate
 Recommended technique
 realign extensor, reef radial sagittal band, release contracted ulnar sagittal band,
ulnar intrinsics release or step lengthening
 if arthroplasty performed then above soft tissue techniques also required
PIP JOINT DEFORMITY
 hyperextension, flexion or lateral deformities
 mild and passively correctable then consider soft tissue procedures
 otherwise arthrodesis required
THUMB DEFORMITY
 lateral subluxation of IP
 MP joint affects IP and vice versa
 joint fusion required
 consider IP MP CMC joint fusions
SCLERODERMA
 systemic sclerosis
 generalized disease
 involves skin, gastrointestinal tract, kidneys, lungs, heart and hands
 females predominates




disorder of small blood vessels and connective tissue that leads to fibrosis
CREST syndrome
Raynauds phenomenon - white then turn blue and finally reddish in colour
Overlap syndrome - scleroderma patients with assoc findings of lupus,
dermatomyositis or RA
2 forms of systemic sclerosis
1. one with limited and localised skin involvement
2. more common diffuse type
DIGITAL DEFORMITY

most frequent deformity
1. PIP flexion contractures secondary to lose of extension
2. extensor mechanism thins and then ruptures
3. skin thins and then breaks down, exposed joint and osteomyelitis
4. secondary hyperextension of MP and joint contracture, stiffness
other
hyperextension of PIP secondary to volar subluxation of MP similar to RA
1st web contracture
Recommended technique

indications for surgery
1. increasing deformity with functional loss
2. development of skin breakdown
3. extruding calcific material
Ulceration
 usually at finger tips, slow healing
 skin may breakdown at bony prominences at PIPJ and MPJ
 often heal with splinting and good wound care
 With many patients, often spontaneously heal so treat conservatively
 Occasional surgical debridement, dressings, topical and systemic antibiotics as
required
Calcinosis
 intracutaneous or subcutaneous
 seen in 15% of diffuse type and 44% of limited cutaneous type excision or curette
and dress wounds
 firm and tender, may extrude
 surgery to help extrusion of calcium deposits for symptom relief
o curettage and saline irrigation is helpful
o allow to heal by secondary intention
DIGITAL JOINT DEFORMITY
DIP usually terminalisation or fusion
PIP fusion if surrounding skin OK - with bone shortening usually enough skin for
closure
MP
vascularity improves as more proximal
treat first then address PIP
MP arthroplasty
if tight dorsal skin consider volar approach metacarpal head resection
joint remodels with relatively good function
vascular insufficiency (Raynauds)
Digital sympathectomy