Basics
Description
- Chronic systemic inflammatory disorder that attacks the joints:
- Nonsuppurative, proliferative synovitis
- Destruction of the articular cartilage
- Ankylosis of the joint
- Involvement of knee is common.
- Baker cysts may be seen in chronic disease.
- Involvement of spine is limited to cervical region:
- May cause atlantoaxial subluxation
- Rarely results in cord compression
Juvenile rheumatoid arthritis (JRA) is a distinct entity (see "Arthritis, Juvenile Idiopathic").
- Genetics:
- Genetic predisposition related to HLA-DR4
- Female-to-male ratio is 3:1.
- Typical age of onset is between 30 and 50.
Etiology
- Etiology is unknown.
- Possible triggers include infection and autoimmune response.
- Prevalence is about 1% of both US and world population.
Diagnosis
Signs and Symptoms
- Malaise, fatigue
- Generalized musculoskeletal pain
- After weeks to months, patients develop swollen, warm, painful joints.
- Often worse in morning
- Joint involvement usually symmetric and polyarticular
- Starting in small joints of hands and feet:
- Later wrists, elbow, and knees
- Distal interphalangeal (DIP) joints of hand generally not involved:
- Presence of swelling in these joints should suggest another type of arthritis.
- Synovitis is typically gradual.
- Classic joint findings in long-standing disease:
- Metacarpophalangeal (MCP) swelling with ulnar deviation
- Swan neck and boutonniere deformities
- Extra-articular complications:
- SC nodules
- Vasculitis
- Pericarditis or myocarditis
- Pulmonary fibrosis
- Pneumonitis
- Sj ĥgren syndrome
- Mononeuritis multiplex
- Evidence of mild pericarditis on echocardiogram is found in up to 1/3 of patients.
- Consider ECG evaluation in these patients
- Patients usually present to ED owing to exacerbations of the disease or complication in other organ systems:
- Airway obstruction from cricoarytenoid arthritis or laryngeal nodules
- Heart block, constrictive pericarditis, pericardial effusion with possible tamponade or myocarditis
- Pulmonary fibrosis, pleuritis, intrapulmonary nodules, or pneumonitis
- Hepatitis
- Neurologic findings may result from cervical spine subluxation or ocular manifestations such as scleritis and episcleritis.
- Can also have retinal vasculitis in periphery, and recurrent iritis-consider in patients with photophobia, red eye, and decreased vision. These patients need ophthalmologic evaluation
- Complications of chronic steroid use:
- Infections
- Steroid-induced osteopenia and fractures
- Insulin resistance
- Glaucoma or IOP elevation, accelerated cataracts
- Patients may present with side effects related to chronic salicylate or NSAID use such as GI bleeding.
- Drugs such as methotrexate, gold, or d-penicillamine also have toxic side effects, most commonly GI but also neuropathic.
Essential Workup
- Primary diagnosis of rheumatoid arthritis (RA) is rarely made in the ED.
- Synovitis should be present for at least 6 wk; a minimum of 4 of the following 7 criteria as established by the American Rheumatism Association must be met to make the diagnosis:
- Stiffness of the involved joints in the morning for at least 1 hr
- Arthritis in 3 or more joints with effusion or soft tissue swelling
- Arthritis of joint in hand (wrist, MCP, or proximal interphalangeal [PIP] joint)
- Symmetric arthritis
- Rheumatoid nodules on extensor surfaces or juxta-articular surfaces
- Significantly elevated rheumatoid factor
- Characteristic radiographic changes include erosions and decalcification (not attributable to osteoarthritis).
- Other pertinent history: Malaise, weakness, weight loss, myalgias, bursitis, tendonitis, fever of unknown cause
- Initial workup should focus on demonstrating that other causes of arthritis are not present, especially septic arthritis, reactive arthritis, or gout.
- Arthrocentesis of a joint effusion may be required.
Diagnosis Tests & Interpretation
ECG, chest radiograph, C-spine or extremity radiograph, and hemoglobin testing are helpful if patient presents with complications of RA.
Lab
- CBC: Mild anemia with leukocytosis and thrombocytosis
- Erythrocyte sedimentation rate (ESR): Often >30. Guide for elevation is age/2 in men, (age + 10)/2 in women. Consider GCA in patients with elevated markers and RA with vision loss that is acute.
- C-reactive protein correlates with erosive disease
- Antinuclear antibodies (ANA) 30-40% positive screening tool
- Rheumatoid factor: Elevated in ~70% of cases
- Joint fluid analysis:
- Typically between 4,000 and 50,000 white cells
- Neutrophil predominance
- Microscopic Gram stain of fluid should show no organisms and no crystals.
- ECG: Conduction defects are rare, but heart block may be seen. May see evidence of pericarditis.
Imaging
- Joint radiograph:
- Joint effusion
- Juxta-articular erosions and decalcification
- Narrowing of joint space
- Loss of cartilage
- MRI of joints can detect early inflammation before plain radiograph
- CXR reveal pulmonary fibrosis, pleural changes, nodular lung disease, or pneumonitis:
- Cardiac silhouette may show changes related to myocarditis.
- Cervical spine radiograph:
- Atlantoaxial joint subluxation may occur.
Differential Diagnosis
- Osteoarthritis
- Septic arthritis
- Reactive arthritis
- Gonococcal arthritis
- Lyme disease
- Gout
- Connective tissue disorders
- Systemic lupus erythematosus (SLE), dermatomyositis, polymyositis, vasculitis, Reiter syndrome, and sarcoid
- Rheumatic fever
- Malignancy
Treatment
Pre-Hospital
Cervical spine immobilization and airway support as indicated
Initial Stabilization/Therapy
- ABCs:
- Manage airway with attention to C-spine immobilization during intubation.
- Treat complications of RA as appropriate.
Ed Treatment/Procedures
- Salicylates or NSAIDs are 1st-line treatment for RA:
- If 1 NSAID fails, another NSAID from a different chemical class may work better.
- Early treatment of RA is important as joint changes may be most progressive during the 1st 18 mo.
Medication
- Glucocorticoids, methotrexate, and other 2nd-line therapies should be initiated by a rheumatologist.
- Aspirin (ECASA): Adult: 900 mg PO QID (2.6-5.4 g/d); peds: 60-90 mg/kg/d QID up to 3.6 g
Note: Enteric coated aspirin has delayed absorption and its analgesic effects will be delayed compared to regular aspirin. Doses of aspirin needed for anti-inflammatory effect approach toxic doses. Patients should be closely monitored and dose carefully titrated to avoid toxicity.
- Auranofin: 3-9 mg/d (peds: 0.15 mg/kg/d up to 9 mg) divided BID
- Celecoxib (Celebrex): 100-200 mg PO BID; peds: N/A
- Hydroxychloroquine: Adult: 200-600 mg/d divided BID
- Ibuprofen (Ibuprin, Advil, Motrin): 200-800 mg (peds: 10 mg/kg) PO q6h
- Leflunomide: 100 mg PO daily for 3 d, then maintenance dose of 10-20 mg PO daily; peds: N/A
- Methotrexate: 7.5 mg once/wk
- Prednisone: Maintenance: 5-10 mg PO daily; acute exacerbations: 20-50 mg PO daily; peds: Maintenance: 0.1 mg/kg/d PO, acute exacerbations: 2-5 mg/kg/d PO
- Sulfasalazine: Adult: 500-1,000 mg PO BID; peds: 30-60 mg/kg/d BID. up to 2 g
- Not recommended in children <6 yr
- NSAIDs and Tramadol for breakthrough pain.
- Newer DMARDs and monoclonals need to be dosed by a rheumatologist and should likely not be prescribed in the ED: Abatacept, Adalimumab, Anakinra, Etanercept, Infliximab, Rituximab, Tocilizumab.
Recent studies have shown possibly increased risk of cardiovascular event with NSAID medications, particularly with COX-2 inhibitors.
Follow-Up
Disposition
Admission Criteria
- Patients with severe or life-threatening presentations of RA and its complications should be admitted to hospital.
- Admission is warranted when diagnosis is unclear and serious illnesses such as septic joint or systemic vasculitis may be present or cannot be ruled out.
- Admission may be required for pain control.
- Admission may be required if patient has inadequate social support and is unable to maintain activities of daily living.
- Pediatric patients with fever and arthritis should be strongly considered for admission.
Discharge Criteria
Patients without serious complications may be managed as outpatients with appropriate medications and follow-up.
Issues for Referral
All patients should have primary physician for further therapy and care as well as appropriate specialty care referral such as rheumatologists, cardiologists, and orthopedics.
Pearls and Pitfalls
- Recognize that symmetric arthritis is more consistent with RA.
- Even patients with RA can get septic arthritis.
- Consult rheumatologist rather than initiate steroids or TNF antagonists from ED.
Additional Reading
- Imboden JB. The immunopathogenesis of rheumatoid arthritis. Annu Rev Pathol. 2009;4:417-434.
- Sanders S, Harisdangkul V. Leflunomide for the treatment of rheumatoid arthritis and autoimmunity. Am J Med Sci. 2002;323(4):190-193.
- Smedslund G, Byfuglien MG, Olsen SU, et al. Effectiveness and safety of dietary interventions for rheumatoid arthritis: A systematic review of randomized controlled trials. J Am Diet Assoc. 2010;110(5):727-735.
- Smith JB, Haynes MK. Rheumatoid arthritis: A molecular understanding. Ann Intern Med. 2002;136(12):908-922.
- The American College of Rheumatology Subcommittee on Rheumatoid Arthritis Guidelines. Guidelines for the management of rheumatoid arthritis: 2002 update. Arthritis Rheum. 2002;46:328-346.
Codes
ICD9
714.0 Rheumatoid arthritis
ICD10
- M06.9 Rheumatoid arthritis, unspecified
- M06.049 Rheumatoid arthritis without rheumatoid factor, unsp hand
- M06.079 Rheumatoid arthritis w/o rheumatoid factor, unsp ank/ft
- M06.069 Rheumatoid arthritis without rheumatoid factor, unsp knee
- M05.849 Oth rheumatoid arthritis with rheumatoid factor of unsp hand
- M05.869 Oth rheumatoid arthritis with rheumatoid factor of unsp knee
- M05.879 Oth rheumatoid arthritis w rheumatoid factor of unsp ank/ft
- M05.9 Rheumatoid arthritis with rheumatoid factor, unspecified
- M06.08 Rheumatoid arthritis without rheumatoid factor, vertebrae
SNOMED
- 69896004 Rheumatoid arthritis (disorder)
- 287007001 Rheumatoid arthritis - hand joint
- 287008006 Rheumatoid arthritis of ankle and/or foot (disorder)
- 201777003 Rheumatoid arthritis of knee (disorder)
- 201764007 Rheumatoid arthritis of cervical spine (disorder)