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 passively 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