Understanding Autoimmune Disorders and SLE

Autoimmune Disorders

• Immune response develops against one's own tissues.
• The body cannot distinguish "self" proteins from "foreign" proteins.
• The body normally recognizes its own cells as non-antigenic
• Immune response is triggered only in response to agents identified as foreign.
• Autoimmune disorders disrupt the ability to differentiate self from non-self.
• The immune system attacks the body's own cells as if they were foreign antigens.

Organ-Specific Autoimmune Diseases

• Hashimoto's thyroiditis
• Graves' disease
• Celiac disease
• Inflammatory bowel disease (IBD), including ulcerative colitis and Crohn's disease

Systemic (Multi-Organ) Autoimmune Diseases

• Affect vessels and/or connective tissue with variable signs and symptoms.
• Systemic Lupus Erythematosus
• Sjögren's Syndrome
• Scleroderma

Systemic Lupus Erythematosus (SLE)

• Inflammatory multisystemic disease with variable signs and symptoms.
• Females most often get the disease
• Includes several types of antibodies
• Antinuclear antibody (ANA) is associated with SLE
• Body systems most often affected by SLE: skin, kidneys, serosal membranes (mouth and nose), joints, and heart.

SLE Risk Factors

• Female gender: Nine to ten times more likely to develop lupus.
• Age: Diagnosed mostly between 15 to 45 years.
• Race/ethnicity: Higher incidence in African-Americans, Hispanics, Asians, and Native Americans.
• Family history.

SLE Signs and Symptoms

• Fatigue
• Fever
• Joint pain, stiffness, and swelling
• Butterfly-shaped rash on the face covering the cheeks and bridge of nose
• Skin lesions that appear or worsen with sun exposure (photosensitivity)
• Fingers and toes that turn white or blue when exposed to cold or during stressful periods (Raynaud's phenomenon)
• Shortness of breath
• Chest pain
• Dry eyes
• Headaches, confusion, and memory loss

SLE Diagnostic Findings

• Positive anti-nuclear antibody (ANA) test in 97% of patients
  • ANA attacks healthy cells, mistaking them for foreign invaders.
• Positive anti-dsDNA antibodies attack the DNA inside the cell nucleus in 50% of patients.
• Positive antibodies to Sm proteins in the cell nucleus: 30-40% of patients
  • Presence of this antibody almost always means that lupus is present
• Low complement levels: Present in inflammatory processes to strengthen the body's immune response, used up in patients with lupus.
• High levels of C-reactive protein (CRP): Reflection of the inflammatory process present in lupus.
• Erythrocyte sedimentation rate (ESR): Test for inflammation.
  • elevated protein causes red blood cells clump together
  • Usually high in people with active lupus

SLE Nursing Considerations

• Assess general fatigue level
  • Determine patient's daily activities that contribute to fatigue
  • Develop an energy-conserving plan for completing daily and other activities and work
  • Encourage rest periods as needed throughout the day to conserve energy
  • Encourage patient to get 8-10 hours of sleep at night
• Encourage exercise as tolerated
• Pain management
• Encourage compliance with medication regime
• Assess the daily dietary intake: Food diary and encouragement of healthful eating
• Weight monitoring, including recording patient's weight at each visit and home weight monitoring
• Monitor WBC count
• Teach patient to look for the signs and symptoms of infection, particularly urinary and respiratory infections.
  • Instruct patient to call physician if signs and symptoms of an infection or fever occur.
• Explore possible ways of concealing skin lesions and hair loss
• Allow patient to express feelings and needs: Assess for the presence of depression, anxiety, and other stressors.

Systemic Sclerosis (Scleroderma)

• Chronic hardening and tightening of the skin and connective tissues.
• Scleroderma is a group of rare diseases that more often affects women with common occurrence between 30 and 50 years.
• Signs and Symptoms: tightening of the skin, joint pain, exaggerated response to cold (Raynaud's disease), and heartburn.
• The treatments include medication, physical therapy, and surgery.

CREST Syndrome

• Limited symptoms of scleroderma referred to as CREST.
• C: Calcinosis; Calcium deposits under the skin and in tissues (hard white dots).
• R: Raynaud's phenomenon.
• E: Esophageal dysmotility; Heartburn.
• S: Sclerodactyly; Thick skin on fingers.
• T: Telangiectasias; Enlarged blood vessels, red spots on the face and other areas.

Systemic Sclerosis - GI Tract

• Esophagus experiences atrophy and fibrosis of the wall leading to dysphagia.

Systemic Sclerosis - Lungs, Heart & Vasculature

• Lungs experience Interstitial fibrosis
• Heart experiences Interstitial fibrosis of myocardium
• Vasculature experiences Raynaud's phenomenon & Polyarteritis nodosa

Systemic Sclerosis - Diagnostic Findings

• Positive ANA is 95% of patients
• Increased erythrocyte sedimentation rate
• Hypergammaglobulinemia
• Increased creatine phosphokinase levels in patients with muscle involvement
• Increased urea and creatinine levels in patients with kidney involvement
• C-reative protein: 25%, correlate with disease activity, severity, poor pulmonary function, and shorter survival.
• Serum procollagen 3 peptide: marker of disease activity.

Systemic Sclerosis - Autoantibodies in SSC

• Topoisomerase-l: dcSSc can cause ILD, cardiac involvement, scleroderma renal crisis
• Centromere proteins are IcSSc.
• Increased digital ischemia, calcinosis, isolated PAH from autoantibodies
• Increased PAH, ILD, scleroderma renal crisis, and myositis from U3-RNP dcSSc.
• Th/TO IcSSc can cause ILD and PAH.
• PM/Scl IcSSc can cause calcinosis and myositis.
• Ul-RNP MCTD can cause PAH
• RNA polymerase III dcSSc cause extensive skin and scleroderma renal crisis
• Notations: dcSSc, diffuse cutaneous SSC; IcSSc, limited cutaneous SSC; ILD, interstitial lung disease & PAH, pulmonary arterial hypertension.

Nursing Considerations & Patient Teaching for Systemic Sclerosis

• Disease process, with a primary focus on presenting symptoms.
• Education about prescribed medications.
• Reduce the risk of vasoconstriction:
  • Avoidance of very cold temperatures
  • Avoidance of caffeinated beverages, chocolate, medications known to cause vasoconstriction.
  • Smoking cessation.

Scleroderma - Nutrition Therapy & Diet Changes

• Avoid spicy foods and very hot foods, large meals
• Four to six small meals a day
• Maintain upright position 1 to 2 hours after eating
• Drink plenty of fluids
• Take multivitamins
• Allowed foods from Grain Groups: Soft bread (toast, rolls, biscuits, muffins, cornbread), cooked cereals, dry cereals with milk, cooked noodles, rice, crackers, pancakes & waffles
• Avoided foods from Grain Groups: Hard rolls, bread sticks, bagels & popcorn
• Allowed fats: Butter, oil and jelly
• Avoided fats: Bacon, margarine, mayonnaise, salad dressings & gravy
• Dairy products: Cream (Sour & Whipping), coffee, cream cheese, olives
• Avoided dairy products: Nuts, deep-fried crispy Foods
• Allowed Dessert: Sherbet, gelatin, puddings, cakes & pies
• Avoided dessert: Desserts containing nuts or dried fruits.
• Allowed miscellaneous: Sauces & Soups
• Avoided miscellaneous: Dessert containing nuts or dried fruits
• Avoided Snacks/Seasonings: Potato or snack chips

Sjögren's Syndrome

• Autoimmune disease where the body attacks and damages the tear ducts in the eyes and the salivary glands.
• Exact etiology unknown but may be due to environmental factors, genetics, sex hormones (more common in women over 40 years), and viral infections.
• Signs and Symptoms: Abnormal sense of taste, burning/redness of eyes, blurry vision, difficulty chewing, swallowing or talking, etc.
• Also presents signs such as dry, itchy skin, enlarged salivary glands, fatigue, tooth decay/early tooth loss, dry cough, hoarseness, vaginal dryness.

Sjögren's Syndrome Diagnosis

• Physical Examination
• Eye examination
• Laboratory studies
  • Anti-nuclear antibodies (ANA)
  • Anti-SSA and SSB antibodies
  • Rheumatoid factor
• Biopsy of salivary glands

Sjögren's Syndrome Diagnostic Findings

• Schirmer's test measures tear production in the eyes
• Ocular surface staining, that involves the examination of the surfaces of the eyes for damage and dryness
• Salivary gland function scans
• Lip biopsy to look for inflammation of the glands and saliva production
• Sialometry, which measures the flow of saliva
• Ultrasonography of the major salivary glands to reveal characteristic structural changes that can aid in diagnosis.

Sjögren's Syndrome: Nursing Considerations

• Inspect oral mucosa
  • Inspect Candida infection, ulcers, saliva pools and dental hygiene
• Recommend the following:
  • artificial saliva preparations
  • artificial tears
  • moisturizing nasal spray
  • artificial vaginal moisturizers
• Drink plenty of fluids
• Skin Care:
  • Inspect areas of trauma
  • Apply lubricants daily
  • Avoiding Shearing forces & frequent position changes
• Educate patient to increase liquid intake with meals
• Discourage spicy/dry foods from menu
• Suggest frequent and smaller meals
• Weigh patients weekly and review diet
• Encourage frequent dental visits
  • More frequent dental cavities for Sjogrens patients
• Do not take meds that cause dryness
• Wear protective eyewear outdoors

Thyroid Auto-Antibodies - Graves

• Graves' disease includes:
  • TSH receptor antibodies (TRAb) at ~ 90%
  • Thyroid peroxidase antibodies (TPOAb) at ~ 70%
  • Thyroglobulin antibodies (TgAb) at ~ 50-70%

Thyroid Auto-Antibodies - Hashimoto's Thyroiditis

• Hashimoto’s Thyroiditis includes:
  • TSH Receptor antibodies (TRAb) at ~10 - 15%
  • Thyroid peroxidase antibodies (TPOAb) > 90%
  • Thyroglobulin antibodies (TgAb) has > 80

Hashimoto's Thyroiditis

• Most common type of thyroid disease involving autoimmune damage.
• Includes lymphocytic infiltrate, fibrosis, and decreased thyroid hormone production
• Additionally, Autoantibodies (thyroglobulin and peroxidase)
• Hashimoto's can be associated with autoimmune disease.
  • Adrenal deficiency
  • Ovarian failure
  • diabetes

Graves’ Disease

• Most common cause of cases of hyperthyroidism: 60-80%.
• Caused by thyroid stimulating immunoglobulins, to be autoimmune.
• This is the result of binding to TSH receptors on thyroid, causing an increase hypersecretion of thyroid hormones.
• Creates an increase in hypertrophy & hyperplasia of thyroid follicles

Graves’ Disease & Clinical Manifestations

• This causes issues with the eyes, called Ophthalmopathy
  • Occurs in 50% of patients with this disorder
  • Resulting in visible Eyelid retraction
  • Can cause Periorbital edema
  • Even cases of Proptosis, exophthalmos.
  • Possibly Diplopia

Associated Conditions with Graves Diseases

• May show signs or be linked to other conditions, to include
  • Type I Diabetes Mellitus
  • Addison's Disease
  • Vitiligo
  • Pernicious anemia
  • Alopecia Areata
  • Myasthenia Gravis
  • Celiac Disease

Celiac Disease (Gluten-Sensitivity Enteropathy)

• Autoimmune condition, causing a compromised mucous layer leading to gluten issues
• Increased risk linked to genetic predisposition causing misdiagnosis
• Presents often in Caucasians and women
• More commonly seen in children - over 73,000 cases
  • Half may show no obvious signals or symptoms

Celiac Disease (Gluten-Sensitivity Enteropathy) Symptoms

• Can include: Diarrhea, Fatigue, Weight loss, Bloating, Abdominal and muscle pain, Nausea and vomiting
• Possible Anemia, usually linked with an iron deficiency
• Bone density loss resulting to osteoporosis.
• Skin rashes showing itchy blisters. Can show signs of Mouth ulcers, Headaches or joint pain

Links with Celiac Disease (Gluten-Sensitivity Enteropathy)

• May show Family history with celiac cases or dermatitis issues
• Linked to Type 1 diabetes
• May show links to Down syndrome
• Autoimmune thyroid disease
• Microscopic colitis with lymphocytic causes
• Addison's disease

Diagnostics Tests for Celiac Disease (Gluten-Sensitivity Enteropathy)

• Positive IgA antiendomysial antibody
• Positive IgA antitransglutaminase
• Positive IgA and IgG antigliadin antibodies
• Checking CBC, checking for Anemia
• During Endoscopy for Villus atrophy

Dietary tests for Celiac Disease (Gluten-Sensitivity Enteropathy)

• checking for Iron level
• Can test for Vitamin B12 level and amount of folate
• Albumin and total serum protein levels test
• Checking protein levels like c-reactive
• Erythrocyte sedimentation rate
• Checking Comprehensive reading on panel of metabolism

Gluten-Free Diet Guide

• Allowed starches and grains: Rice or wild rice, Amaranth, Dried beans, Cornmeal
• Pasta, breads and other baked goods: potato, rice, bean, buckwheat, soy, tapioca, arrowroot, quinoa, millet, and flax
• Potatoes, sweet potatoes, and yams - Fresh, canned and frozen fruit or fruit juices and canned and frozen vegetables with allowed ingredients
• Cold cereals such as puffed rice and corn, and fresh vegetables
• All milk products w/o gluten additives and aged cheese
• All unprocessed foods in this category and peanut butter
• Avoided are all breads, baked goods, crackers, noodles, pastas, and cereals

Celiac Disease - Nursing Considerations

• Teach and educate:
  • How to read food labels
  • Avoid gluten containing products
  • Use vitamin and mineral supplementatio Support:
• Financial concerns
• Collaboration:
  • Dietitian, Community Resources
• Focuses on autoantibodies
• HLA DQ 2/8
• Autoimmune issues - celiac disease, creative-med-doses
• Enterocytes are killed - T Cell-mediated Cytotoxicity

Crohn's Disease: Etiology & Pathophysiology

• Chronic disorder that causes inflammation of the GI tract, most commonly affecting the small intestine.
• Transmural; affecting all layers of the mucosa.
• Begins with edema and thickening of the mucosa.
• Ulcers appear on the inflamed mucosa, causing fistulas and fissures.
• Scaring, thickening and narrowing of the GI tract.
• Usually diagnosed in adolescents.
• Prevalence has risen in the past 30 years and is seen more in smokers.

Crohn's Disease Clinical Manifestations

• Can include persistent diarrhea, loss of appetite and weight loss, possible rectal bleeding.
• Cramping abdominal pain (Steatorrhea & Fatigue)
• Complications: Bowel obstruction, Sores of ulcers, Fistulas & Malnutrition
• The intestinal surface is cobblestone-like: showing thickened wall & fat wrapping

Crohn's Disease diagnostic Findings

• Test for Positive anti saccharomyces cerevisiae antibody test (ASCA)
• May check, Iron level, vitamin B12 and folate levels
• Checking Albumlin levels, Comprehensive review of the Metabolic Panel
• Colonoscopy or sigmoidoscopy used to check for Ulcers or an presence of Inflammation
• Stool test is taken check for small signs of blood

Crohn's Disease - Nursing Considerations

• Teaching and education, with stress, medication, and eating right
  • Help patient manage Diet, diagnostic test, procedure assistance
• Support: Understanding of diseases, and the patients body image
• Collaborate: Get with a dietician, Gastroenterologist and Surgeon
  • Smoking cessation programs

Crohn's Disease: Nutrition Therapy

• Implement needed dietary changes, like
  • Limit Dairy Products and lower any intake of fat
  • Limit fiber amount
  • Help patients eat smaller meals & avoid problem foods
• Help manage and supply multivitamins, with intake of fluids

Ulcerative Colitis: Etiology & Pathophysiology

• Influences the Mucosal Layer, results in inflamed area, leads to Bleeding.
• To classify, we look for the types of colitis
• Extensive colitis extends to the hepatic flexure
• Proctosigmoidsitis moves toward junctions/areas
• Left-sided colitis moves toward the flexure
• Pancolitis starts from the rectum to the cecum area overall
• Proctitis is contained in the area of the rectum.

Ulcerative Colitis Clinical Manifestations

• Shows a pattern of Diarrhea and Intermittent tenesmus.
• Possible Rectal bleeding, fatigue.
• Also present LLQ of abdomen area.
• Types of classifications that occur Mild, Severe, Fulminant Severity:
  • Mild: < 4 stools each day
  • Severe: > 6 with more
  • Fulminant: > 10

Diagnostics Ulcerative Colitis

• Looking for Antibodies saccharomyces
• C- reative protein in tests for a series of 3 stool samples
• C & S, also check for a clostridium infection
• With help from and Ultrasound techs, Identify small Abscesses area
• Use colonoscopy & Sigmoidscopy will shows area is assess -Assess extent level of infection

Dietary Needs &Ulcerative Colitis

• Help with the dietary need with Low residue
• High proteins
• Decrease fiber
• Avoid fat
• Small Frequent Meals
• With small meals and frequent snacking

Ulcerative Colitis - Nursing Considerations

• Need you educate patient
  • Educated when there’s an Early infection & or recognition
  • Check is patient hydrating or not & or keeping food journal.
• Help with with emotional with Support with a care.
• With and and infections, with a skilled nurses -Get patients a skilled dietician to help

Treatment of Autoimmune Disease

• Control the autoimmune reaction with immunosuppressant medications.
• Corticoids aid in controlling inflammatory markers.
• In use of with Biological specific of used or need
• Help patients be aware so they can know, use, and need biological

Grave's Disease Result

• A result of Thyroid-stimulating hormone