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Category: Medical

Topic: Pharmacology

Level: Paramedic

Next Unit: Drugs Affecting the Blood: Coagulants and Anti-Coagulants

19 minute read

Autoimmune Disease

Autoimmune disease is a pathologic condition caused by an adaptive autoimmune response, which is an immune response directed against an antigen within the body of the host, termed a self-antigen. The response may be induced by a foreign or self-antigen and usually involves a T-cell and B-cell response.

The development of autoimmune disease depends upon an imbalance between pathogenic factors generated by autoreactive T cells and B cells and the regulatory factors that normally control the immune response.

The most common autoimmune diseases are:

  • Rheumatoid arthritis: a chronic inflammatory disorder affecting many joints, including those in the hands and feet and other peripheral joints.
  • Lupus: an inflammatory disease caused when the immune system attacks its own tissues, including kidneys, brain, blood vessels, lungs, and heart.
  • Celiac disease: an immune reaction to eating gluten, a protein found in wheat, barley, and rye; it leads to damage of the small intestines.
  • Inflammatory Bowel Disease (IBD)Crohn's disease, ulcerative colitis.
  • Sjögren's syndrome: an immune system disorder characterized by dry eyes and dry mouth.
  • Polymyalgia rheumatica: an inflammatory disorder causing muscle pain and stiffness around the shoulders and hips.
  • Myasthenia Gravis: (MG) is characterized by chronic weakness and fatigue of the voluntary muscles. It results from antibodies to the nerve receptors on the muscles and is treated with colinergic medications.
  • Multiple sclerosis: a disease in which the immune system eats away at the protective covering of nerves, called myelin, used to speed nerve conduction.
  • Ankylosing spondylitis: an inflammatory arthritis affecting the spine and large joints.
  • Type 1 diabetes: a chronic condition in which the pancreas produces little or no insulin.
  • Alopecia areata: sudden hair loss that starts with one or more circular bald patches that may overlap.
  • Vasculitis: an inflammation of the blood vessels in the blood vessel walls.
  • Temporal arteritis: an inflammation of arteries in and around the scalp.


Drugs Used in Autoimmune Disease

IMMUNOSUPPRESSANTS: many people who receive immunosuppressant drugs are prescribed medications from more than one of these categories:

  • CORTICOSTEROIDS:prednisone, budesonide, prednisolone.

These are useful with "flares" of disease where exaggerated symptoms occur from time to time during the chronic course of the disease.

Complications/side effects include:

  • confusion,
  • restlessness,
  • headache,
  • nausea/vomiting,
  • thinning skin,
  • acne,
  • insomnia, 
  • weight gain;
  • infections due to the depression of the immune system.
  • "Moon facies:" with longstanding, chronic use of steroids, which causes extra fat to build up on the sides of the face, the face can assume a rounded shape that is full or puffy. It is mainly of cosmetic concern, but it is useful in tipping off EMS responders of likely long-term steroid use.
  • Drug-Drug Interactions: 
  1. Buproprion (Wellbutrin antidepressant) + steroid ⇒ seizures.
  2. Haloperidol (antipsychotic) + steroid ⇒ arrhythmias.
  • Withdrawal

Steroid use cannot be stopped abruptly; tapering the drug gives the adrenal glands time to return to their normal patterns of secretion. Withdrawal symptoms and signs can mimic many other medical problems :

  • weakness,
  • fatigue,
  • decreased appetite,
  • weight loss,
  • nausea, vomiting,
  • diarrhea,
  • abdominal pain).
  • CALCINEURIN INHIBITORS: cyclosporine, tacrolimus.

Calcineurin activates T-cells and their response. White blood cells, part of your immune system, normally fight substances in your body that aren’t there naturally, such as a transplanted organ. Cyclosporine stops white blood cells from attacking a transplanted organ. Calcineurin inhibitors are used in organ transplant to avoid rejection.

Complications/side effects include:

  • hypertension,
  • hypomagnesia,
  • renal thrombus,
  • stomach pain,
  • acne,
  • tremors,
  • headache,
  • kidney damage,
  • edema,
  • trouble breathing,
  • increased risk of infection.
  • mTOR INHIBITORS (mammalian target of rapamycin): sirolimus, everolimus.

mTOR inhibitors suppress a key catabolic process, autophagy, both by inhibiting its activation and by suppressing the production of lysosomes, the organelles in which autophagy occurs.

Autophagy is a normal physiological process in the body that deals with the destruction of cells in the body. It maintains homeostasis or normal functioning by protein degradation and turnover of the destroyed cell organelles for new cell formation. During autoimmune disease, the process of autophagy is upscaled and increased.

Complications/side effects include:

  • stomatitis,
  • rash,
  • anemia,
  • fatigue,
  • hyperglycemia/hypertriglyceridemia,
  • decreased appetite,
  • nausea,
  • diarrhea, and
  • interstitial lung disease.
  • IMDH INHIBITORS (inhibit the enzyme inosine monophosphate dehydrogenase*): azathioprine, leflunomide, mycophenolate.

IMDH is involved in the synthesis of adhesion receptors important in cell-cell contacts.

Complications/side effects include:

  • low white blood cell counts,
  • infection,
  • nausea/vomiting,
  • diarrhea,
  • fever,
  • muscle aches,
  • liver damage,
  • dizziness,
  • hypotension,
  • pancreatitis,
  • fatigue,
  • weight loss.
  • BIOLOGICS:abatacept (Orencia), adalimumab (Humira), etanercept (Enbrel), and others.

Biologics are substances made from a living organism or its products that either block the abnormal activity of T-cells or certain proteins, specifically tumor necrosis factor alpha (TNF-alpha) or pro-inflammatory interleukins. They are typically self-administered by the patient intramuscularly (IM).

Complications/side effects include:

  • allergic reactions,
  • site reactions,
  • chills,
  • weakness,
  • diarrhea,
  • nausea/vomiting,
  • rash.


In the Field

The above list of drugs and their details are for only a general background. In the field, most of the emergencies of autoimmune drugs to which you'll respond will deal with two main things:

1) Infections.

Any immunosuppressants will minimize the response to invading infection, so your most likely emergency will be due to infectious diseases and their consequences--bacteremia, sepsis, pneumonia, severe hyperthermia, etc.

A note about immunizations: measles, mumps, and rubella (MMR) vaccine and varicella (chickenpox) vaccine are live but weakened (attenuated) forms of these viruses. These types of immunizations are contraindicated for persons on immunosuppressants, for fear of giving the very illness they are designed to prevent.

2) Side Effects.

  • blood pressure changes,
  • nausea/vomiting, and
  • fatigue.

One notable interaction is when oral steroids and the antidepressant bupropion (Wellbutrin) are combined, which can provoke seizures.

Also, general malaise from stopping steroids abruptly.

Signs and symptoms of serious infection are straightforward as far as indications for transport go, but side effects will present as vague symptoms in someone who already has other medical conditions.

A simple history will mention autoimmune illness which should prompt suspicion of its contribution to that which you are responding, which will require an appropriate facility to evaluate properly.

Meaning: transport, with support and maintenance of ABC (airway, breathing, circulation), IV access, and oxygen if indicated.

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