Immune Disorders

Immune Disorders Colleen Carter RN, MSN 2014

Nursing Management of Clients with Organ Transplants • Transplant success tied to matching tissue antigens, HLA (Human Leukocyte Antigens) • Autograft • Allograft • Xenograft • Histocompatibility – ability of cells and tissues to survive transplantation without immunologic interference by the recipient

Host-versus-Graft Transplant Rejection • Complex process involving both antibody-mediated and cell mediated responses • Hyperacute Rejection – Begins immediately to 2-3 days after the transplant and can’t be stopped once it begins • Acute rejection – occurs 4 days to 3 mo post-transplant. Most common rejection and is treatable. • Chronic rejection – occurs from 4 mos to yrs post-transplant. No cure. Once organ cannot function, another transplant is only course.

Treatment of Transplant rejection Maintenance therapy – Ongoing immune suppressants Ex. cyclosporin, Imuran & a corticosteroid (prednisone) Rescue therapy – treats acute rejection. Ex: ALG , murononab-CD3. Most effective during first course of treatment

System Lupus Erythematosus (SLE) • Chronic, multi-system, autoimmune disease • Affects more women than men • Affects more African American women than European American women • SLE thought to be a combination of environmental and genetic factors • Extremely variable in its course and no way to predict its progression

Lupus: Signs and Symptoms • Skin: Butterfly rash • Raynaud’s Phenomenon

Lupus: Signs/Symptoms • Musculoskeletal System • Muscle and joint pain very common with exacerbations and remissions • Arthritis – affects primarily distal joints: hands, wrists, fingers, toes, ankles, knees, etc. • May have tendon involvement and rupture • Knees and hips can have treatment related osteonecrosis from steroid therapy

Lupus: Signs/Symptoms • Cardiac System • Pericarditis - most common cardiac manifestation • Myocarditis • Anemia • Leukopenia • Thrombocytopenia

Lupus: Signs/Symptoms • Respiratory System • Pleuritis – Inflammation of the pleura • Pleural Effusions – Fluid build-up between pleura and chest cavity • Pneumonia

Lupus: Signs/Symptoms • Gastrointestinal/Hepatic Systems • Can affect any area of the GI system as well as pancreas, spleen, or liver • Ex: oral ulcers, peptic ulcers, abdominal pain/N/V/D, pancreatitis, hepatomegaly, GERD, ulcerative colitis

Lupus: Signs/Symptoms • Renal System • Lupus Nephritis – leading cause of death among patients diagnosed with SLE (Lupus) • s/s to monitor for: fluid retention (edema, wt gain), hematuria, proteinuria, changes in urine output, hypertension

Lupus: Signs/Symptoms • Neurologic System • Neuropathies • Psychoses, depression • Seizures, migraine headaches • CNS vasculitis • Peripheral neuropathies CNS Vasculitis

Lupus: Signs/Symptoms • Constitutional Symptoms • Fatigue • Weight changes/loss or gain • Fever • Arthralgias

Lupus: S/S • Psychosocial Issues • Altered body image/poor self-concept • Chronic fatigue/weakness may prevent being as socially active as previously • Fear and anxiety may occur due to the unpredictability of flares or the progression of the disease, necessity of life style changes, etc.

Lupus: Diagnosis • Lab tests: Antinuclear antibody ESR (sed rate) Serum complement Various antibody titers CBC – looking for pancytopenia Specific testing for body system involvement Urinalysis/24 hr urine Diagnostic Imaging: CXR, Hand x-rays, CT OR MRI

Lupus: Treatment • Pharmacologic Management • NSAIDS • Antimalarials (Plaquenil) • Corticosteroid Therapy • Immunosuppressive agents (methotrexate or Imuran)

Lupus: Treatment • Non-pharmacologic Management • Avoid direct exposure to sunlight • Use sunscreen with SPF of 15 or higher • Wide brimmed hat when outside • Some physicians recommend avoiding use of oral contraceptives • Careful skin and hair care with mild soaps/shampoos

Lupus: Important Patient Education • Importance of skin care • Monitor body temp and other warning signs of a flare: increased fatigue, pain, abdominal discomfort, rash, headache, dizziness • Reproductive impact • Avoid exposure to infection • Need to follow treatment plan, including follow-up appointments and prompt reporting of a flare • Preventive health care • Medic Alert bracelet

Rheumatoid Arthritis (RA) • Chronic, progressive, systemic, inflammatory, autoimmune disease that affects joints and other tissues or organ systems. • Most prevalent in European Americans • Affects 0.5% to 1% of the population worldwide; women more frequently than men

RA Pathophysiology • Cause: believed to be genetic and environmental • Autoantibodies (rheumatoid factors) attack healthy tissue, especially synovial membranes, causing inflammation. Immune processes activated • Activation of the inflammatory and immune response damages the synovial membrane.

RA Signs/Symptoms • Onset may be acute and severe (usually precipitated by a stressor such as surgery or an infection) • Or onset may be insidious • Joints primarily affected are hands, wrists, knees and feet • Joint involvement usually bilateral and symmetric. • Disease symptoms described as early or late and joint (articular) or systemic (extra-articular)

RA Signs/Symptoms • Early Disease Manifestations: • Joint stiffness, swelling, pain • Systemic: • Low-grade fever • Fatigue • Weakness • Anorexia • Paresthesias

RA Signs/Symptoms • Late Disease Manifestations • Joint deformities (swan neck and ulnar deviation) • Moderate to severe pain and morning stiffness Swan neck deformity Ulnar deviation

RA Signs/Symptoms • Late Disease Manifestations – Systemic • Osteoporosis • Anemia • Weight loss • Subcutaneous nodules • Peripheral neuropathy • Vasculitis • Pericarditis • Sjogren’s syndrome • Renal disease

RA Diagnosis • Based on patient’s hx, physical assessment, and diagnostic tests • Lab tests: • Rheumatoid factor • ANA • ESR and C-reactive protein • CBC • Synovial fluid exam • X-rays of affected joints

RA Treatment • Surgical Management: • A synovectomy to remove inflamed synovium may be necessary for knee or elbow • Total joint arthroplasty may be necessary for joint deformity and destruction • Arthrodesis (joint fusion) to stabilize joints such as cervical vertebrae, wrists, and ankles.

RA Treatment • Pharmacological Management: • NSAIDS • Antimalarials • Corticosteroids, oral or intra-articular for temporary relief • Disease-Modifying Antirheumatic Drugs (DMARDS) to reduce disease activity: methotrexate, Imuran, Cytoxan or BMR’s: Humira, Enbrel, Remicade

RA Treatment • Non-pharmacological treatment • Plasmapheresis • Balanced program of rest and exercise – energy conservation • Physical and Occupational therapy • Heat and cold • Assistive devices and splints • Balanced nutrition

Scleroderma (Systemic Sclerosis) • Autoimmune disorder of connective tissue • Characterized by hardening(sclero) and thickening of the skin (derma), blood vessels, synovium, skeletal muscles, and internal organs • Approximately 300,000 people in the US have Scleroderma • Affects women more than men by 3:1 • Affects more African Americans than Caucasians

Scleroderma - Pathophysiology • Early stages very similar to SLE – often misdiagnosed • Can be limited or diffuse • May have CREST syndrome: • Calcinosis • Raynaud’s phenomenon • Esophageal dysmotility • Sclerodactyly • Telangiectasia

Scleroderma Signs/Symptoms • Musculoskeletal - Joint pain • GI: Dysphagia and reflux, esophagitis, diarrhea or constipation, abdominal cramping and malabsorption • Skin: Bilateral, symmetric swelling of hands and sometimes feet. After edematous phase, the skin becomes hard and thick. • Facial changes – skin tightening leads to loss of skin lines, appearance of disappearing lips

Scleroderma Signs/Symptoms • Facial skin symptoms: • Tightening of the skin • Disappearing lips • Decreased mobility of eyelids • Evolving process Body image and psychosocial issues

Scleroderma Signs and Symptoms • Cardiovascular: Raynaud’s Syndrome, Myocardial fibrosis, Pericarditis and dysrhythmias • Pulmonary: Lung fibrosis, pulmonary hypertension, exertional dyspnea • Renal – proteinuria, hematuria, hypertension, and renal failure

Scleroderma Diagnosis • ANA: of at least 1:40 • Lab tests/results similar to SLE: ESR – elevated; CBC- may show anemia, RF – elevated in about 30% • Barium swallow – may show esophageal dysmotility • X-ray of hands & wrists – muscle atrophy, osteopenia • No single diagnostic test – overlap with other autoimmune diseases so diagnosis may initially be difficult

Scleroderma Treatment • Corticosteroids & immunosuppressants – tried to slow the progression • Protect hands from the cold • Report fever to the • Skin protection, moisturizer and careful ongoing assessments • Gastric secretion blockers for esophagitis/reflux. Avoid spicy foods, caffeine, alcohol • NSAIDS for joint pain • Be aware that the side effects from many of the pharmacological treatments can worsen symptoms of the disease

Gout • Metabolic d/o characterized by an acute inflammatory arthritis triggered by crystallization of urate within the joints. • May be caused by an inborn error of metabolism or as the result of another disease process or factor; i.e. crash diets, renal insufficiency • Affects approximately 3 million Americans each year, and over twice that man have been affected at some time. • Occurs more often in men. More common in women who are post-menopausal or taking diuretics

Gout • 3 clinical stages • Asymptomatic – not detected unless a uric acid level is checked. • Acute - Extremely painful joint inflammation, usually in the great toe, called podagra • Chronic – After repeated episodes of acute gout, urates are deposited in various other connective tissues: synovial fluids (gouty arthritis); subcutaneous tissue (tophi) and kidneys(can form kidney stones and result in kidney failure)

Gout treatment • Pharmacological Management • Acute Gout: NSAID (Indocin) or ibuprofen; and colchicine. Taken until symptoms subside • Chronic gout: Prevention is key. Zyloprim (allopurinol) lowers uric acid levels. Benemid(probenecid) promotes excretion of uric acid. May be given one or the other or a combination.

Gout treatment • Non-pharmacological Management • Dietary restrictions on high-purine meats (red and organ meats) and seafood (shellfish and oily fish with bones) may be recommended • No alcohol • Avoid aspirin and diuretics • Drink enough fluid to maintain daily urine output of 2000 mL or more

Lyme Disease • Most common tick-borne illness in North America • Carried by the infected deer tick (black-legged tick) • Occurs most often in children and young adults living in rural areas • Risk factors: Spending time in wooded or grassy areas Having exposed skin Not removing ticks promptly or properly

Lyme Disease: Pathophysiology • After an incubation period of 3-30 days after the bite, the spirochete migrates outward into the skin • Forms a characteristic erythema migrans (bull’s eye rash) • May spread to other skin sites, organs, or nodes

Lyme Disease: Signs/Symptoms • Stage I: • Skin: Bull’s eye rash • Musculoskeletal: pain and stiffness in muscles and joints • Constitutional: flu-like symptoms, fever, chills, fatigue, body aches • If not treated or treatment is unsuccessful, progresses to Stage II

Lyme Disease Signs/Symptoms • Stage II: • Migratory musculoskeletal pain and swelling; especially in the knees • Carditis with dysrhythmias, dyspnea, palpitations • CNS disorders: meningitis, Bell’s palsy, numbness or weakness in limbs, impaired muscle movement • If not diagnosed and treated can progress to Stage III

Lyme Disease Signs/Symptoms • Stage III (months to years after the tick bite) • Chronic recurrent arthritis • Chronic fatigue • Cardiac complications • Thinking/memory issues

Lyme Disease: Treatment • Antibiotic therapy: doxycycline, tetracycline, amoxicillin, cefuroxime, erythromycin. May be given for 3-4 weeks • Aspirin or another NSAID for relief of arthritic symptoms • May need assistive devices (splints/crutches) • Prevention is the Key

Preventing Lyme Disease • When walking in wooded or grassy areas: • Wear long pants tucked into socks, long sleeves, hat, gloves, shoes • Use insect repellents (10-30% DEET) • Check yourself, your children and your pets for ticks • Remove a tick with tweezers by pulling straight out. Clean with alcohol other antiseptic.

Fibromyalgia • Common rheumatic pain syndrome with wide-spread musculoskeletal pain, stiffness, and tenderness • Women affected 9 times more often than men • Genetic and environmental factors are thought to contribute • Requires a hx of widespread pain in all 4 quadrants of the body for a minimum duration of 3 months and pain in at least 11 of 18 trigger points

Fibromyalgia Signs/Symptoms • Fatigue, moderate to severe • Sleep disorders • Problems with cognitive function (Fibro Fog) • Irritable Bowel Syndrome • Headaches and migraines • Anxiety and depression • Environmental sensitivities

Fibromyalgia Treatment • Pharmacological Management is symptomatic: • 3 drugs approved as of 2009 to reduce pain levels and improve sleep: Lyrica, Cymbalta, and Savella • Tricyclic antidepressants such as amitryptyline may help with pain and sleep as well • SSRI’s - treat depression and anxiety

Fibromyalgia Treatment • Non-pharmacological Management • Physical therapy • Regular exercise routine • Alternative therapies: Massage, acupuncture, chiropractic, yoga • Stress management • Therapy to assist with depression/anxiety

Immune Disorders