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Updated: Jun 19 2024

Rheumatoid Arthritis

Images
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  • summary
    • Rheumatoid Arthritis is a chronic systemic autoimmune disease caused by IgM cell-mediated immune response against soft tissues, cartilage, and bone. Patients present with insidious onset of morning joint stiffness, polyarthropathy, subcutaneous nodules, with progressive hand and wrist deformity. 
    • Diagnosis is made with a combination of physical examination, characteristic radiographs, and labs to evaluate for presence of RF and anti-CCP antibodies.
    • Treatment is primarily medical management with NSAIDS, DMARDS, biologics, antimalarials, and steroids.
  • Epidemiology
    • Incidence
      • most common form of inflammatory arthritis
    • Demographics
      • affects 3% of women and 1% of men
  • Etiology
    • Pathophysiology
      • immunology
        • cell-mediated (T cell-MHC type II) immune response against soft tissues (early), cartilage (later), and bone (later)
          • rheumatoid factor
            • an IgM antibody against native IgG antibodies
            • immune complex is then deposited in end tissues like the kidney as part of the pathophysiology
          • mononuclear cells
            • are the primary cellular mediator of tissue destruction in RA
          • IL-1, TNF-alpha
            • are part of cascade that leads to joint damage
          • immune response thought be related to
            • infectious etiology or
            • HLA locus
      • pathoanatomy
        • cascade of events includes
          • antigen-antibody and antibody-antibody reactions >
          • microvascular proliferation and obstruction >
          • synovial pannus formation (histology shows prominent intimal hyperplasia) >
          • joint subluxation, chondrocyte death/joint destruction, and deformity >
          • tendon tenosynovitis and rupture
    • Genetics
      • associated with specific HLA loci (HLA-DR4 & HLA DW4)
      • ~15% rate of concordance amongst monozygotic twins
    • Associated conditions
      • orthopaedic manifestations
        • see below
      • medical conditions & comorbidities
        • rheumatoid vasculitis
        • pericarditis
        • pulmonary disease
        • Felty's syndrome (RA with splenomegaly and leukopenia)
        • Still's disease (acute onset RA with fever, rash and splenomegaly)
        • Sjogren's syndrome (autoimmune condition affecting exocrine glands)
          • Decreased secretions from salivary and tear duct glands
          • Lymphoid tissue proliferation
  • Presentation
    • Symptoms
      • insidious onset of morning stiffness and polyarthropathy
      • usually affects hands and feet
        • DIP joint of hand is usually spared
        • may also affect knees, cervical spine, elbows, ankle and shoulder
    • Physical exam
      • subcutaneous nodules in 20% (strong association with positive serum RF)
      • ulnar deviation with metacarpophalangeal (MCP) subluxation, swan neck deformity
      • hallux valgus, claw toes, metatarsophlanageal (MTP) subluxation
      • joints become affected at later stage in disease process
  • Imaging
    • Radiographs
      • periarticular erosions and osteopenia
      • protrusio acetabuli
        • medial migration of femoral head past the radiographic teardrop
        • also seen in Marfan's syndrome, Paget's disease, Otto's pelvis and other metabolic bone conditions
      • joint space narrowing
      • central glenoid erosion
  • Studies
    • Labs
      • anti-CCP (cyclic citrullinated peptide, most sensitive and specific test)
      • anti-MCV (mutated citrullinated vimentin)
      • elevated ESR
      • elevated CRP
      • positive RF titer (most commonly IgM)
        • targets the Fc portion of IgG
        • elevated in 75-80% of patients with RA
      • joint fluid testing
        • decreased complement
        • may have elevated RF levels
  • Diagnostic Criteria (1987 Revised Criteria for Diagnosis of RA)
    • Morning stiffness ≥ 1h
    • Swelling in ≥ 3 joints
    • Rheumatoid nodules
    • Radiographic changes of the hand including bony erosions and decalcification
    • Symmetric arthritis
    • Serum rheumatoid factor
    • Arthritis of the hand (MCP, PIP) and wrist
      • have ≥4 of 7 criteria for a 6 week period
  • Treatment
    • Nonoperative
      • pharmacologic treatment
        • indications
          • mainstay of treatment
        • medications (see table below)
          • first line includes NSAIDS, antimalarials, remittent drugs (gold, sulfasalazine, methotrexate), steroids, cytotoxic drugs
          • more aggressive approach with DMARDs is now favored over pyramid approach
        • outcomes
          • significant advances in pharmacologic management have significantly changed prognosis of disease
    • Operative
      • operative treatment dictated by specific condition
        • significant advances in pharmocologic management have led to a decrease in surgical intervention
      • important to obtain preoperative cervical spine radiographs
      • Pharmacologic Management of RA
      •  Low dose steroids
      • Corticosteroids
      • Disease modifying anti-rheumatic drugs (DMARDs)
      • Methotrexate
      • A folate analogue with anti-inflammatory properties linked to inhibition of neovascularization therapeutic effects increased when combined with tetracyclines due to anti-collagenase properties
      • Leflunomide
      • An inhibitor of pyrimidine synthesis
      • Sulfasalazine
      • Exact mechanism unknown, but associated with a decrease in ESR and CRP
      • Hydroxychloroquine
      • Blocks the activation of toll-like receptors (TLR), which decreases the activity of dendritic cells, thus mitigating the inflammatory process
      • Others
      • D-penicillamine
      •  Biologic Agents / TNF antagonists
      • Etanercept (Enbrel)
      • TNF-alpha receptor fusion protein (TNF type II receptor fused to IgG1: Fc portion) that binds to TNF-alpha
      • Infliximab (Remicade)
      • Human mouse chimeric anti-TNF-alpha monoclonal antibody
      • Adalimumab (Humira)
      • Human anti-TNF-alpha monoclonal antibody
      • Golimumab (Simponi)
      • Human anti-TNF-alphamonoclonal antibody
      • Certolizumab (Cimzia)
      • Pegylated human anti-TNF-alpha monoclonal antibody
      • DMARDS / Biologic Agents /IL-1 antagonists
      • Anakinra (Kineret)
      • Recombinant IL1 receptor antagonist
      • Rituximab (Rituxan)
      • Monoclonal antibody to CD20 antigen (inhibits B cells)
      • Abatacept (Orencia)
      • Selective co-stimulation modulator that binds to CD80 and CD86 (inhibits T cells)
      • Ustekinumab (Stelera)
      • Monoclonal antibody targeting IL-12 and IL-23
      • Tocilizumab (Actemra)
      • IL6 receptor inhibitor (2nd line treatment for poor response to TNF-antagonist therapy)
      • Preoperative management 
      • Medication
      • When to Stop/Restart
      • NSAIDs
      • Stop 5 half lives before surgery (stop ASA 7-10 days before)
      • Steroids
      • Dosing depends on level of potential surgical stress
      • Methotrexate (MTX)
      • Continue
      • Leflunomide
      • Continue for minor procedures. Stop 1-2 days before major procedures, restart 1-2 wks after
      • Sulfasalazine
      • Continue
      • Hydroxychloroquine
      • Continue
      • Ustekinumab
      • Stop 1 week prior to procedure. Restart >14 days postoperatively.
      • TNF antagonists (etanercept, infliximab, adalimumab)
      • Plan surgery at the end of dosing +1 week interval (~13 weeks last dose).  Restart 10-14 days after.
      • IL-1 antagonist (anakinra)
      • Continue for minor procedures. Stop 1-2 days before for major procedures. Restart 10 days after.
      • Rituximab
      • Stop 7 months before major surgery.
  • Cervical Spondylitis
    • Cervical spondylitis includes
      • atlantoaxial subluxation
      • basilar invagination
      • subaxial subluxation
  • Finger Conditions
    • Rheumatoid nodules
      • epidemiology
        • most common extra-articular manifestation of RA
        • seen in 25% of patients with RA and associated with aggressive disease
        • an extraarticular process found over IP joints, over olecranon, and over ulnar border of the forearm
      • prognosis
        • erosion through skin may lead to formation of sinus tract
      • presentation
        • patients complain of pain and cosmetic concerns
      • treatment
        • non operative
          • steroid injection
        • operative
          • surgical excision
            • indications
              • cosmetic concerns, pain relief, diagnostic biopsy
    • Arthritis Mutilans
      • seen in patients with RA or psoriatic arthritis
      • digits develop gross instability with bone loss (pencil in cup deformity, wind chime fingers)
      • treated with interposition bone grafting and fusion
    • Ulnar drift at MCP joint
      • introduction
        • volar subluxation associated with ulnar drifting of digits
        • pathoanatomy
          • joint synovitits >
          • radial hood sagittal fiber stretching >
            • concomitant volar plate stretching
          • extrinsic extensor tendons subluxate ulnarly >
          • lax collateral ligaments allow ulnar deviation deformity >
          • ulnar intrinsics contract further worsening the deformity >
          • wrist radial deviation further worsens >
          • flexor tendon eventually drifts ulnar
      • presentation
        • extensor lag at level of MCP joint
      • treatment
        • operative
          • synovectomy, extensor tendon centralization, and intrinsic release
            • indications
              • early disease
          • MCP arthroplasty
            • silicone MCP arthroplasty is most common
            • indications
              • late disease
              • thumb MCP involvement + thumb IP involvment
            • techniques
              • important to correct wrist deformity at same time if it is radially deviated
              • synovectomy, volar capsular resection, ulnar collateral ligament release, radial collateral ligament repair/reconstruction, extensor tendon realignment, intrinsic tendon release
            • outcomes
              • ultimate function is less predictable
              • overall patient satisfaction of 70%
              • 1 year followup shows improved ulnar drift and extensor lag
            • complications
              • infection
              • implant failure
              • deformity recurrence
          • MCP fusion
            • indications
              • thumb MCP involvement without IP involvement
    • Boutonniere deformity
      • pathoanatomy
        • synovitis of PIP leads to central slip and dorsal capsule attenuation
        • increasing PIP flexion
        • lateral bands subluxate volar to axis of rotation of PIP
        • oblique retinacular ligament contracture causes extension contracture of DIP
      • treatment
        • splinting
          • for flexible PIP
        • extensor reconstruction (central slip imbrication or Fowler distal tenotomy)
          • for moderate deformity
        • PIP arthrodesis or arthroplasty
          • for rigid contractures
    • Swan neck deformity
      • pathoanatomy
        • terminal tendon rupture from DIP synovitis leads to DIP flexion/PIP hyperextension
        • FDS, volar plate and collateral ligament attenuation from synovitis leads to decreased volar support of PIP, and hyperextension deformity
        • lateral band subluxate dorsal to PIP axis of rotation
        • contracture of triangular ligament, attenuation of transverse retinacular ligament
      • treatment
        • splinting
          • for flexible PIP (prevent hyperextension)
        • FDS tenodesis or proximal Fowler tenotomy
          • for flexible PIP and failed splinting
        • dorsal capsule release, lateral band mobilization, collateral ligament and intrinsic release, extensor tenolysis
          • for rigid deformities
  • Thumb Conditions
      • Nalebuff Classification of Rheumatoid Thumb Deformities
      • Type
      • Description
      • Treatment
      • Type 1
      • Boutonniere (most common deformity, MCP flexion and IP extension)
      • Stage 1: Synovectomy with extensor hood reconstruction
      • Stage 2: MCP fusion or arthroplasty
      • Stage 3: IP and MCP fusion (if CMC is normal). IP fusion and MCP arthroplasty (if CMC is diseased)
      • Type 2
      • Boutonniere with CMC subluxation (uncommon, deformity primarily at CMC)
      • Same as Type 1 and 3
      • Type 3
      • Swan neck deformity (MCP hyperextension, IP flexion)
      • Stage 1:splinting vs CMC arthroplasty
      • Stage 2: MCP fusion
      • Stage 3: MCP fusion with first web release
      • Type 4
      • Gamekeeper deformity (metacarpal adduction, radial deviation of P1 with lax volar plate and UCL)
      • Stage 1 (passively correctable): synovectomy, UCL reconstruction, and adductor fascia release
      • Stage 2 (fixed deformity) MP arthroplasty or fusion
      • Type 5
      • Swan neck with MCP disease (MCP volar plate laxity)
      • MP stabilized in flexion by volar capsulodesis
      • Type 6
      • Skeletal collapse (arthritis mutilans) (MCP volar plate laxity)
      • Combination of arthrodesis
  • Flexor Tendon Conditions
    • Triggering
      • treatment
        • synovectomy + resection of FDS
    • Mannerfelt syndrome
      • introduction
        • rupture of FPL (most common flexor rupture) in carpal tunnel due to scaphoid spur
      • treatment options
        • FDS4 to FPL tendon transfer + excision of scaphoid spurs (may also lead to rupture index FDP2)
        • tendon graft + spur excision
        • IPJ fusion (for advanced disease)
    • FDP rupture
      • treatment
        • synovectomy + DIP fusion
    • FDS rupture
      • treatment is observation
  • Extensor Tendon Conditions
    • Extensor Tendon Rupture
      • epidemiology
        • frequency EDM > EDC (ring) > EDC (small) > EPL
      • treatment
        • tendon transfer, interposition graft, or Darrach's procedure
    • Radial sagittal band failure
      • extensor tendons migrate slip into ulnar gutter and volar to center of rotation of MCP joint
      • physical exam
        • lose active extension
        • if MCP placed in extension actively then patient can hold extended
      • treatment
        • sagittal band reconstruction (extensor hood reconstruction)
    • Vaughan-Jackson syndrome
      • introduction
        • rupture of digital extensor tendons from ulnar to radial
      • pathoanatomy
        • DRUJ instability + volar carpal subluxation results in dorsal ulnar head prominence and attritional rupture of the extensor tendons
          • EDM is the first extensor ruptured
      • treatment
        • EIP to EDC transfer and distal ulna resection
    • Differentials for loss of digital extension
      • PIN neuropathy
      • extensor tendon rupture
      • extensor tendon subluxation (torn radial sagittal band)
      • MCP volar subluxation
      • trigger finger
      • Common Tendon Transfers in RA
      • Ruptured Tendon
      • Tendon Transfer
      • EPL
      • EIP to EPL
      • EDQM
      • leave alone
      • EDQM and EDC5
      • EIP to EDC5 or EDQM to EDC piggyback transfer
      • EDQM, EDC5, EDC4
      • EIP to EDQM and EDC4 side to side to EDC3
      • Multiple tendon rupture
      • Use palmaris graft and FDS
  • Wrist Conditions
    • Caput-ulna syndrome
      • pathoanatomy
        • synovitis in the DRUJ > ECU subsheath stretching > ECU subluxation > supination of the carpal bones away from the head of the ulna > volar carpal subluxation > increased pressure over the extensor compartments > tendon rupture
        • distinguish from extensor lag caused by PIN compression neuropathy (seen in RA due to elbow synovitis)
      • treatment
        • Darrach distal ulna resection
          • must also relocate ECU dorsally with a retinacular flap or perform ECU stabilization of ulna
        • ulnar hemiresection
        • Sauvé-Kapandji (ulnar pseudoarthrosis)
          • has advantage of preserving the TFCC
          • good option for younger patients
    • Radiocarpal Destruction
      • pathoanatomy
        • synovitis and capsular distension leads to supination, radial deviation (angulation) of carpus
        • ulnar and volar translocation of the carpus on the radius
        • with scaphoid flexion, radiolunate widening, lunate translocation (ulnarwards)
        • secondary radioscaphoid arthrosis
        • ulnar deviation of the fingers at the MP joints creating the classic zigzag deformity
      • treatment
        • synovectomy
          • indications
            • early disease
          • technique
            • transfer of ECRL to ECU to diminish deforming forces (Clayton's procedure)
        • radiolunate fusion (Chamay) or radioscapholunate fusion
          • indications
            • intermediate disease with preserved midcarpal joint
        • wrist fusion
          • indications
            • advanced disease, poor bone stock
            • remains gold standard
            • often combined with Darrach
        • total wrist arthroplasty
          • indications
            • sedentary patients with good bone stock
            • advantages over fusion is motion and best in patients with reasonable motion preop
  • Elbow Conditions
    • Rheumatoid elbow
      • nonoperative
        • rheumatoid elbow is mainly managed with medical management and cortisone injections
      • operative
        • arthroscopic or open synovectomy
          • indications
            • pain without instability
            • no significant loss of ROM
        • synovectomy and radial head excision
          • indications
            • focus of degeneration is in radiohumeral joint
            • posterior interosseous nerve compression secondary to radial head synovitis
          • technique
            • performed through lateral approach to elbow
        • interposition arthroplasy
          • indications
            • young active patients who are not candidates of TEA
          • technique
            • resection and contouring of humeral surface
            • cover humeral surface with cutis autograft, Achilles tendon, fascia, or dermal allograft
            • some use distraction external fixator to unload membrane and enhance its bonding to bone and improve motion
            • results less predictable than TEA, but avoids prosthetic complications
        • total elbow arthoplasty
          • indications
            • pain
            • loss of motion
            • instability
          • technique
            • semiconstrained device has best results
          • outcomes
            • reliable procedure for advanced RA of elbow
            • 5 lb single arm weight lifting restriction
  • Shoulder Conditions
    • Introduction
      • RA is most prevalent form of inflammatory process affecting the shoulder with >90% developing shoulder symptoms
      • commonly associated with rotator cuff tears
    • Evaluation
      • classic radiographic findings include
        • central glenoid wear
        • periarticular osteopenia
        • cysts
  • Hip Conditions
    • Protrusio acetabuli
  • Knee Conditions
    • Operative
      • synovectomy of knee
        • decreases pain and swelling but does not alter prevent radiographic progression and does not prevent the need for TKA in the future
        • normal synovium reforms, but degenerates to rheumatoid synovium over time
        • range of motion is not improved
      • total knee arthroplasty
        • rheumatoid arthritis is considered an indication for resurfacing of the patella during total knee arthroplasty
  • Foot & Toe Conditions
    • Introduction
      • usually bilateral and symmetric
      • forefoot joints are the first to be affected
      • human leukocyte antigen (HLA)-DR4 positive
    • Toe hyperextension deformity
      • the earliest manifestation of rheumatoid arthritis of the forefoot is synovitis of the MTP joints with eventual hyperextension deformity of the MTP joints including distal migration of the forefoot pad, painful plantar callosities and skin ulcerations over bony prominences.
      • treatment
        • arthrodesis of the 1st MTP joint and lesser MTP joint resections
    • Talonavicular arthritis
      • common to have degenerative changes
      • treat with fusion
  • Cervical Conditions
    • Present in 90% of patients with RA
      • diagnosis often missed
    • Cervical rheumatoid spondylitis includes three main patterns of instability
      • atlantoaxial subluxation
        • most common form of instability
      • basilar invagination
      • subaxial subluxation
  • Complications
    • Postoperative infection
      • history of prior surgical site infection (SSI)
        • is the most significant risk factor for development of another SSI
      • immunosuppressive therapy
        • the literature is controversial whether RA patients on immunosuppressive therapy have significantly increased infection rates for orthopaedic procedures
        • pharmacologic therapy may need to be changed prior to surgical interventions
          • surgery should be performed when immunosuppressive agents are at their lowest levels
            • etanercept should be discontinued 2 week prior to major urgical procedures
            • rituximab should be held for 7 months prior to major surgical procedures
            • adalimumab should be discontinued 10 days prior to surgery
            • the lowest level of infliximab is found 2 weeks prior to the next scheduled infusion
  • Prognosis
    • Significant advances in pharmacologic management have led to a decrease in surgical intervention
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