Welcome to the Rare Classroom, a new series from Patient Worthy. Rare Classroom is designed for the curious reader who wants to get informed on some of the rarest, most mysterious diseases and conditions. There are thousands of rare diseases out there, but only a very small number of them have viable treatments and regularly make the news. This series is an opportunity to learn the basics about some of the diseases that almost no one hears much about or that we otherwise haven’t been able to report on very often.

Eyes front and ears open. Class is now in session.

The disease that we will be learning about today is:

Rheumatoid Arthritis

What is Rheumatoid Arthritis?

• Rheumatoid arthritis (RA) is an autoimmune disease in which the body’s immune system attacks the joints​
• Not considered rare; up to 1 percent of adults in the developed world may be affected in a given year
• Collagen (the main component of cartilage) is gradually destroyed which narrows joint space and eventually damages bone
• ​The abnormal immune response causes inflammation that can damage joints and organs including the skin, heart and lungs
  • Abnormal immune response causes inflammation of the synovium (the membrane that surrounds the joint and creates a protective sac)
  • Fluid and immune system cells accumulate in the synovium to produce pannus (a growth made of thickened synovial tissue)
  • The pannus produces more enzymes that destroy nearby cartilage, attracting more inflammatory white cells, thereby perpetuating the process
• Early diagnosis and prompt treatment can help prevent joint damage and organ destruction
• B cells and T cells are types of immune cells called lymphocytes
  • Lymphocytes are a type of white blood cell
  • If the T cell recognizes an antigen as “non-self,” it will produce chemicals (cytokines) that cause B cells to multiply and release many immune proteins (antibodies)
  • The antibodies circulate in the blood stream, recognizing the foreign particles and trigger
    inflammation
  • In RA, both T cells and B cells are overactive
• Rheumatoid arthritis occurs in three different disease courses:
  • Monocyclic – One episode ending within 2-5 years of initial diagnosis which does not recur
    • Rare
    • May result from early diagnosis or aggressive treatment
  • Polycyclic -The levels of disease activity fluctuate over the course of the condition
    • Changes over time
  • Progressive – Continues to increase in severity and is unremitting
    • Also known as moderate, severe or aggressive RA
    • Most common
• Around 1.3 million patients in the US
• In the US nearly $128 billion is spent in medical care and lost wages/productivity due to RA
• 60-70% with RA have a higher mortality rate than the general population
• Up to ½ of all RA patients become unable to work within 10-20 years

How Do You Get It?

• Women are affected at 2.5x the rate of men
• Onset of RA is usually middle-age, but it often occurs in the 20’s and 30’s
• Some Native American groups have higher prevalence rates
• The exact cause of rheumatoid arthritis is unknown
• Genetic risk factors for the disease can include:
  • A gene called PTPN22 nearly doubles a persons risk of developing RA
  • The PAD14 gene has been identified as a major risk factor in those of Asian descent
  •  The main genetic marker identified with RA is HLA (human leukocyte antigen)
  • The HLA gene does not cause RA but may make it more severe
  • Genetic variations in the HLA may predict how well a patient may respond to specific drugs
• Environmental/Biologic risk factors
  • Some researchers believe certain infections may stimulate the immune system to trigger and prolong the disease
  • Potential triggers include: E Coli, mycoplasma, parvovirus B-19, retroviruses, mycobacteria, and Epstein-Barr virus
  • Smoking is thought to more than double the risk of contracting RA
  • Endocrinologic and reproductive factors may also contribute to the development of RA
    • Live birth history, breast feeding and menstrual history may change the odds of developing RA

What Are The Symptoms?

• Common symptoms of rheumatoid arthritis can include:
  • Morning stiffness that lasts at least an hour
  • Stiffness after a period of inactivity
  • Swelling and pain in the joints
  • Limited motion and function of joints
  • Fatigue
  • Low fevers
  • Dry eyes and mouth
  • Firm lumps (rheumatoid nodules) which grow beneath
    the skin
• 70 percent of patients report that their disease prevents them from living a full, productive life
• Many complications are the result of chronic inflammation and can include:
  • Muscle weakness
  • Pregnancy complications (premature delivery, high blood pressure in the last trimester)
  • Joint deterioration/pain (debilitation, deformation)
  • Mood disorder (anxiety, depression, severe stress)
  • Peripheral neuropathy
  • Kidney and liver problems, possibly the result of long-term treatments
  • Anemia
  • Periodontal disease
  • Eye problems
  • Lymphoma and other cancers
  • Infections (likely due to treatment)
  • Heart disease/stroke
  • Skin issues (nodules, ulcers, rash)
  • Vasculitis
  • Osteoporosis (more common in older patients)
  • Lung disease (pulmonary hypertension, interstitial fibrosis)

How Is It Treated?

• The goal of treatment for rheumatoid arthritis is to reduce disease activity and achieve remission. Accomplishing these goals means:
  • Reducing inflammation
  • Preventing damage to the bones and ligaments of the joints
  • Preserving movement
  • Helping the patient be as free from side effects as possible
    over the long term
• Medications
  • Disease modifying anti-rheumatic drugs (DMARDs)
    • Primary treatment approach
    • A category of otherwise unrelated drugs defined by their use to slow down disease
      progression in RA
    • Effect decreases over time
    • Often used in combination
    • Treatment often begins soon after diagnosis
    • Can slow progression and alter disease course
    • May take months to take effect
  • Corticosteroids (steroids)
    • Controls inflammation and pain quickly
    • May be used early while waiting for DMARDs to take effect or an adjunct therapy in severe cases
  • Nonsteroidal anti-inflammatory drugs (NSAIDs)
    • Reduces pain and inflammation
    • Short onset of action
    • Does not alter disease course
• Surgery
  • Synovectomy
  • Arthroscopy
  • Joint Replacement (Arthroplasty)
  • Joint Fusion (Arthrodesis)
• Lifestyle changes
  • Exercise
  • Pain/stress management
  • Diet changes
• Assistive/Supportive interventions
  • Ointments
  • Orthotic devices
  • Aids for gripping and grabbing

Where Can I Learn More???

• Check out our cornerstone on this disease here.
• Learn more about this disease from the Arthritis Foundation.