Autoimmune Diseases …“Lupus” Gail L Lupica PhD, RN, CNE Nurs 211

What is autoimmunity?

Autoimmunity is when the body begins to recognize the self antigens as foreign.

Why does this occur? It is the result of some initial insult to the immune system. Some can be traced easily and some can not. Viral induced Infections (rubella), bacterial induced infections, and drugs are some examples of triggering events.

SLE- “LUPUS” One of the most common, serious, autoimmune disorders Characterized by the production and the deposition of circulating immune complexes which contain auto anti-bodies which damage tissues. Do you remember which type of hypersensitivity reaction this is?

You’re correct, type 111. Review: Systemic antigens are released into blood. Immune complexes are formed in response to these antigens (even self antigens) Immune complexes deposit Inflammatory cascade is activated.

What effect do these immune complexes have on the intima of the vessels? What other organs are affected?…stay tuned

SLE Immune complexes damage: The glomerulus of the kidney Renal tubular basement membrane of the kidney Brain Heart Spleen Lungs GI tract Skin peritoneum

SLE Most often in women 20-40 years Genetic predisposition likely Chronic & unpredictable Associated with exacerbations and remissions Variable in its pattern course and severity. (Any organ can be affected by the immune complexes)

SLE- diagnosis? Must have 4/11 findings to meet diagnosis criteria: Facial rash (butterfly!!!!!) Discoid rash (scaly skin) Photosensitivity (rash in sun!) Oral /nasopharyngeal ulcers Arthritis in 2 peripheral joints Pleurisy/pericarditis (> serous fluid) Renal manifestations (proteinuria)

SLE -diagnosis Continued… Neurologic disorders (seizures, psychosis) Hematologic disorders (hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia) Immunologic disorders (+ antibody tests…antiDNA, antineuronal, anticoagulant, anti-WBC, anti-RBC, anti-PLT, anti-basement membrane) Presence of antinuclear antibody (characterizes SLE)

SLE- collaborative care NSAIDS (inhibit leukotriene, prostaglandin pathway or both/ relieve pain, headache and inflammation by blocking the cox pathways -What are the nursing implications? Plaquenil (antimalarial agent) Corticosteroids Immunosuppressive drugs (Cytoxan, Imuran)

SLE- Collaborative care Plaquenil (Hydroxychloroquine, an antimalarial agent works by inhibiting DNA and RNA synthesis. Although considered an anti-malarial agent (killing organisms and parasites that cause malaria) it also has anti-inflammatory actions. Prolonged treatment may be necessary.

SLE- collaboratve care Corticosteroids: Act by inhibiting body’s natural immune response Can be systemic or topical (local) /IV, po, topical, inhalation/ Is this always beneficial to limit the immune response? Why? Why not? Let’s take a look…

SLE- corticosteroids What do they do? Stabilize the cell membranes of neutrophils so they do not release their inflammatory substances or attack other WBCs Stabilize the cell membrane of the basophil so histamine is not released. Decrease activity of the B and the T- cells in the immune response. Inhibit macrophage activity which inhibits the engulfment of bacteria or virus-infected cells.

SLE- the down side of steroids The body naturally secretes corticosteroids from the adrenal cortex: (Cortisol) Adrenal glands are located just above each kidney. o Adrenal cortex o Adrenal medulla

SLE- steroids The adrenal cortex: Is responsible for secretion of three major groups of steroid hormones: q Mineralocorticoids (aldosterone): maintains sodium, and fluid balance qGlucocorticoids (cortisol): provide the body with the materials needed for energy; proteins, fats, glucose, water q Androgens (testosterone): hyper secretion can cause masculinizing changes

SLE-corticosteroids What happens if the body is given too much corticosteroid…

SLE -corticosteroids Effects on glucose metabolism: Cortisol increases the amount of glucose formed in the body by acting on the liver and enhancing gluconeogenesis (glucose production) How? It does this by stimulating adipose tissue to release free fatty acids (the substrate needed by the liver in order for gluconeogenesis to take place). Cortisol decreases the utilization of glucose by the tissues (in muscle, adipose and lymph). Then, uptake and metabolism are reduced. What do you get? Increased serum glucose concentrations!

SLE- corticosteroids Effects on fatty acid metabolism: ｷ Cortisol causes fatty acids to be released from the tissue stores into the plasma and then made available for energy utilization. (Although exactly how it does this is not completely understood.) These fatty acid stores are removed from one area of the body (arms and legs) and deposited in another (trunk). What do you get? Truncal obesity, and frail, thin extremities!

SLE- corticosteroids Effects on protein metabolism: Cortisol decreases protein stores in all tissues of the body except in the liver by preventing the synthesis of protein, and by breaking down protein stores from the cells into amino acids. (Protein synthesis in the liver is stimulated, however) What do you get? Loss of protein stores, and muscle wasting

SLE- cortocosteroid Cortisol decreases the inflammatory response to infection!! It also reduces the passage of water into and out of cells, and therefore decreases the amount of fibrous tissue formation. What results? Decreased collagen production.

SLE- corticosteroids- in sum EXCESS CORTIOCOSTEROIDS: Elevated serum glucose levels and DM... Loss of protein stores occurs, muscle wasting Humoral immunity is reduced, decreasing the threshold to infection Skin tissues lose collagen, and become very thin: tearing and bruising easily Excess body and facial hair growth in women (from excess androgen secretion) Mood swings and psychosis may occur as the effect of excess cortisol on cognitive function.

Truncal obesity: results from the mobilization of fat in the lower parts of the body, to the trunk causing the abdomen to become protuberant as the extremities become thin and wasted. ｧ Purple striae: appear on the abdomen as a result of the stretching of the abdominal skin. They’re purple due to the collagen deficit in the tissues. ｧ Round facial features: fat accumulation around the neck and cervical area is termed the buffalo hump.

SLE- disease management Monitor serial anti-DNA titers Monitor ESR What’s this? Teach about drug therapy. What do you think will be emphasized?

SLE-Nsg diagnoses Fatigue Acute pain Impaired skin integrity Activity intolerance Ineffective therapeutic regimen management (teach!)

SLE CASE STUDY!