Boutonnieres and swan necks…. sounds perhaps like a new store to shop for clothing to the layman. However, these terms pop a red flag in the minds of current students and clinicians. These deformities and changes are often seen and associated with cases of Rheumatoid Arthritis.


Over the past few weeks in clinic, I have seen several new cases of rheumatoid arthritis. Some of these with the classic physical exam findings, others with vague complaints of multiple joint pains and fatigue.


Rheumatoid arthritis (RA) affects 1 percent of the population worldwide, making it the most common inflammatory arthritis.1 The peak time that this disease occurs is usually from 30 to 50 years of age, but theoretically, it can occur at any age. This disease can be so severe that it leads to 35 percent of affected patients being on disability after ten years.1


Just like other autoimmune diseases, the cause of rheumatoid arthritis is often multifactorial including genetic predisposition, infection, or environmental factors such as smoking. In rheumatoid arthritis, patients will have activation of inflammation that causes advancement and generation of synovial cells in the joints. This leads to the formation of a pannus which causes joint cartilage disruption as well as erosion and degradation of the bone.


The fueling force behind the damaging process is the excess production of inflammatory cytokines.These being interleukin-6 and tumor necrosis factor primarily.1


Risk factors for RA include female sex, family history of disease, older age, and current or past smoking history. It has been seen that pregnancy and breastfeeding can lead to remission of RA. This is believed to be due to immunologic tolerance during gestation. However, patients in early menarche, as well as those with irregular menstrual periods, rheumatoid arthritis symptoms may worsen. Using vitamin E or oral contraception has not been seen to reduce this risk.


Patients with rheumatoid arthritis often will present with stiffness and pain in numerous joints, primarily in the metacarpophalangeal joints, proximal interphalangeal joints, and wrists, and often sparing the distal interphalangeal joints. Those with RA often will have morning stiffness which lasts for more than one hour, as well as boggy swelling, or slight synovial thickening on examination.1



Systemic symptoms such as fatigue, low-grade fever, and weight loss might be present as well. Swan neck deformity, boutonniere deformity, and ulnar deviation of the MCP joints may be present in more advanced cases of disease.



In 2010 the American College of Rheumatology and European League Against Rheumatism created a new criterion for RA.1 These newer criteria aim to diagnose and catch earlier cases of rheumatoid arthritis. This was completed by not requiring findings such as rheumatoid nodules or symmetric arthritis, and erosive changes on imagining, which occur later in disease, for diagnosis.



Additionally, Dutch researchers have created a tested clinical prediction tool for the diagnosis of RA in patients with undifferentiated arthritis. This tool aims to help find patients that are most likely to advance to rheumatoid arthritis and institute early care and referral.



Rheumatoid arthritis, just like most autoimmune diseases, is characterized by the presence of autoantibodies.1 Rheumatoid factor is often completed if there are concerns about rheumatoid arthritis, but this test is not specific for RA. It can also be positive in patients with hepatitis C and even if older patients that are healthy.1


Anti-citrullinated protein (ant-CCP) antibodies is a more specific test for RA. Around 50 to 80 percent of patients with rheumatoid arthritis will have a positive rheumatoid factor, anti-CCP antibodies, or both. Other laboratory findings that may be present would be a positive antinuclear antibody as well as an elevated ESR and CRP.


Other tests that can be beneficial to complete to aid in the decision-making process before starting therapy would be a CBC and CMP to assess for anemia, thrombocytopenia, as well as an assessment of renal and hepatic function.Patients diagnosed with rheumatoid arthritis or any other inflammatory arthritis should be referred to a rheumatologist promptly to establish treatment.1


Regarding treatment, there are several different options, but patient preference plays a significant role.Goals of treatment include reducing joint pain, swelling, preventing joint destruction and deformity, and helping to maintain quality of life.1


DMARDs are the mainstay of current treatment of RA.These can be both biologics and nonbiologics. Those that are biologics have monoclonal antibodies and receptors to block inflammatory cytokine activation which causes RA symptoms.1


Methotrexate is a first line treatment for RA unless contraindicated or the patient is unable to tolerate the medication. Leflunomide can be used as an alternative, but this medication often has more GI side effects.


Sulfasalazine or hydroxychloroquine can be used in patients with mild disease or those with symptoms, but with testing that showed seronegative rheumatoid arthritis.1


Combination therapy with two or more DMARDs is more efficacious than monotherapy, however, with multiple agents, there is a significant increase in the risk of adverse effects.1 If symptoms of RA are not well controlled on nonbiologic DMARDs, then biologic DMARD (BMARD) therapy should be instituted.


TNF inhibitors are the first line BMARD therapy.Unlike DMARDs, use of multiple BMARDs at once is not recommended because studies showed an unacceptable amount of related adverse effects.1Also, remember before starting BMARD therapy, TB testing is required.



NSAIDs and corticosteroids can be used for the treatment of RA to aid in control of pain and inflammation. These medications, however, should just be used for acute short-term management if possible, whereas DMARDs are preferred maintenance therapy.


Other complementary therapies such as vegetarian or gluten-free diets, therapeutic ultrasound, and thermotherapy lack data on effectiveness. Regarding herbal therapy, data has shown that gamma-linolenic acid and Tripterygium wilfordii have potential benefits. However, it is essential to educate patients that there have been serious side effects with the use of herbal therapy.


Physical and occupation therapy has been seen to improve quality of life and strength in patients with RA and is recommended.


In those patients with severe joint damage due to rheumatoid arthritis, joint replacement may be indicated, especially if symptoms are persistent with medical management.Long-term data on post-operative joint replacement in rheumatoid arthritis patients showed only 4 to 13 percent of patients needing joint revision after ten years.1


We have talked mostly about the effects of RA on the joints, but long-term this disease can affect multiple other organ systems.



Those with RA have a twofold increased risk of developing lymphoma caused by the inflammatory nature of the disease.


Long-term adverse effects of DMARD and BMARD therapy include Class III or IV CHF exacerbation, in which TNF inhibitors are contraindicated due to potentially worsening symptoms.BMARDs, leflunomide, and methotrexate should not be started in patients with active shingles, significant fungal infections, or bacterial infections that require antimicrobial therapy.1



The general population with RA tend to survive around 3 to 12 years less than those without RA. The increased mortality is believed to be due to elevated coronary artery disease risk, especially those with other risk factors for CAD including hypertension, hyperlipidemia, nicotine dependence, or diabetes.


However, the good news is that the novel BMARDs may reverse atherosclerosis progression and reduce the coronary artery disease mortality rate in those with rheumatoid arthritis.1




Resources


1.Am Fam Physician. Diagnosis and Management of Rheumatoid Arthritis 2011;84(11):1245-1252.


2.Ferri’s Clinical Advisor, Rheumatoid Arthritis. 2017.


3.Family Practice Notebook, Rheumatoid Arthritis. Accessed: March 3, 2018.


This article, blog, or podcast should not be used in any legal capacity whatsoever, including but not limited to establishing standard of care in a legal sense or as a basis of expert witness testimony.No guarantee is given regarding the accuracy of any statements or opinions made on the podcast or blog.