Rheumatic Disorders Part II


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Rheumatic Disorders Part II: Systemic Lupus Erythematosus, Systemic sclerosis, & Scleroderma

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Rheumatic Disorders Part II

  1. 1. Rheumatic Disorders Part II:SLE, PA, SSc, Fibromyalgia, Lyme disease, etc. Maria Carmela L. Domocmat, RN, MSN Instructor, School of Nursing Northern Luzon Adventist College
  2. 2. Rheumatic DisordersComprise autoimmune and inflammatorydisorders„the primary crippling disease”Inflammation of jointPrimary reason for work-related disabilityLeading cause of disability among 65 yrs oldand above Maria Carmela L. Domocmat, RN, MSN
  3. 3. What causes autoimmune disease? http://www.medscape.com/content/2000/00/40/87/408750/art-mrc4856.lymp.fig2.gif Certain variants or mutations in the MHC genes may result in abnormal MHCDomocmat, RN, MSN Maria Carmela L. proteins
  4. 4. Reaction to SelfOccurs when the immune system sees “self”antigens as “nonself”may be due to genetic factors, infectious agents,gender, and agethe autoimmune response results in tissuedamage Some damage occurs in only one or a few organs, in other cases it may be body-wide (systemic) Maria Carmela L. Domocmat, RN, MSN
  5. 5. Reaction to Self~ 3.5 % of people have autoimmune diseases On average, women are 2.7 times more likely to develop these diseases than menmost have no known cause or curetreatment is aimed at controlling symptoms Maria Carmela L. Domocmat, RN, MSN
  6. 6. Why does the immune system attack the body thatit‟s supposed to protect? failure to recognize some cells as “self” in rheumatic fever, the streptococcus antigen is very similar to a protein in heart tissue, so the body mistakenly identifies heart tissues as foreign cells seen as foreign are attacked and destroyed may be only a few select cells or organs (organ-specific) – e.g., multiple sclerosis, juvenile diabetes, rheumatic fever may be systemic - e.g., systemic lupus erythematosus, rheumatoid arthritis Maria Carmela L. Domocmat, RN, MSN
  7. 7. Maria Carmela L. Domocmat, RN, MSN
  8. 8. Maria Carmela L. Domocmat, RN, MSN
  9. 9. Maria Carmela L. Domocmat, RN, MSN
  10. 10. Systemic Lupus Erythematosus (SLE)a chronic systemic autoimmune disease Complexes of anti-self antibodies and antigen deposit in, and cause damage to, tissue1 million sufferers in the U.S. Strikes women nine times more often than mensymptoms may include butterfly-shaped rash on face,fatigue, headachestriggered by environmental effects in persons who aregenetically susceptible Maria Carmela L. Domocmat, RN, MSN
  11. 11. Lupus sufferers develop IgG antibodies todouble stranded DNA and nuclear antigens.Antibodies to T cells may also be produced.Immune complexes formed during the diseaseprocess may end up in the kidneys and thencause damage by initiating an inflammatoryresponse that starts with complement fixation.This may ultimately cause kidney failure.Blood vessels and skin may also be attacked byantibodies. Antibodies to erythrocytes aresometimes produced. Maria Carmela L. Domocmat, RN, MSN
  12. 12. s/sPainful or swollen joints and muscle painUnexplained feverRed rashes, most commonly on the faceChest pain upon deep breathingUnusual loss of hairPale or purple fingers or toes from cold or stress (Raynaudsphenomenon)Sensitivity to the sunSwelling (edema) in legs or around eyesMouth ulcersSwollen glandsExtreme fatigue Maria Carmela L. Domocmat, RN, MSN
  13. 13. Diagnostic Tools for LupusMedical historyComplete physical examinationLaboratory tests: Complete blood count (CBC) Erythrocyte sedimentation rate (ESR) Urinalysis Blood chemistries Complement levels Antinuclear antibody test (ANA) Other autoantibody tests (anti-DNA, anti-Sm, anti-RNP, anti-Ro [SSA], anti-La [SSB]) Anticardiolipin antibody testSkin biopsyKidney biopsy Maria Carmela L. Domocmat, RN, MSN
  14. 14. DxMost specific tests Seen exclusively in SLE Antibodies to double-stranded DNA Antibodies to Sm and ribosomal P proteinsMost sensitive test ANA (anti-nuclear antibody) assay fluorescent Maria Carmela L. Domocmat, RN, MSN
  15. 15. Systemic Lupus Erythematosus (SLE) Damaged kidney (left) caused by immunoglobulin deposits (right)Butterfly rash of lupus Maria Carmela L. Domocmat, RN, MSN
  16. 16. Lupus, discoid - view of lesions on the chest: This close-up picture of the neck clearly shows the typical rounded appearance of discoid lupus. The whitish appearance is caused by scaling. The two dark spots are biopsy sites and are not part of the disease.Maria Carmela L. Domocmat, RN, MSN
  17. 17. The round or disk shaped(discoid) rash of lupusproduces red, raised patcheswith scales. The pores (hairfollicles) may be plugged.Scarring often occurs in olderlesions. The majority(approximately 90%) ofindividuals with discoid lupushave only skin involvement ascompared to more generalizedinvolvement in systemic lupuserythematosis (SLE). Maria Carmela L. Domocmat, RN, MSN
  18. 18. Lupus erythematosis often produces a "butterfly rash" or malar rash. Typically, the rash also appears on the nose.Maria Carmela L. Domocmat, RN, MSN
  19. 19. LupusMaria Carmela L. Domocmat, RN, MSN
  20. 20. LupusMaria Carmela L. Domocmat, RN, MSN
  21. 21. exact trigger - not knownresearch has shown that the environmentalfactors include infectious agents excess UV light physical trauma emotional stress and some drugs (anticonvulsants, some forms of penicillin, and drugs used in estrogen therapy) Maria Carmela L. Domocmat, RN, MSN
  22. 22. Managementno cure for SLE.goal of treatment - control symptoms Maria Carmela L. Domocmat, RN, MSN
  23. 23. ManagementReduce inflammation and minimizecomplications NSAIDs - Aspirin Glucoccorticoids HydroxychloroquineManage exacerbationsPromote orientation and decision-makingProvide seizure precautionMonitor fluid status Maria Carmela L. Domocmat, RN, MSN
  24. 24. Treatment : Mild disease Mild disease NSAIDs - treat arthritis and pleurisy Corticosteroid creams – to treat skin rashes antimalaria drug (hydroxychloroquine) and low-dose corticosteroids for skin and arthritis symptomshttp://www.nlm.nih.gov/medlineplus/ency/article/000435.htm Maria Carmela L. Domocmat, RN, MSN
  25. 25. Treatment :severe Treatment for more severe lupus may include: Pulse Therapy – High-dose corticosteroids or medications to decrease the immune system response Cytotoxic drugs (drugs that block cell growth) MTX Azathioprine (Imuran) Cyclophosphamide (Cytoxan)http://www.nlm.nih.gov/medlineplus/ency/article/000435.htm Maria Carmela L. Domocmat, RN, MSN
  26. 26. Other Agents For SLE thrombocytopenia Attenuated androgen danazol IV Ig - Immune globulin intravenous (Gamimune, Gammagard, Sandoglobulin) immunosuppression in serious SLE flares. Platelet increase rapidly within hours after administration Indication – control acute bleeding; rapidly increase platelet to allow for splenectomy or other surgery For cutaneous s/s Dapson Maria Carmela L. Domocmat, RN, MSN
  27. 27. Maria Carmela L. Domocmat, RN, MSN
  28. 28. Drugs in clinical trialUseful for maintenance in lupus nephritis andother serious lupus cases; Inhibits antibodyproduction Mycophenolate (CellCept) ToterogensDehydroepiandrosterone (DHEA)Stem cell transplant Maria Carmela L. Domocmat, RN, MSN
  29. 29. Maintain skin integrityAvoid prolonged exposure to sunlight and otherforms of UV lighting including tanning bedswear protective clothing (long sleeves, large-brimmed hat, long pants), sunglasses, andsunscreen (SPF 30 or higher) when in the sun.Clean skin with mild soap (e.g., ivory)avoid harsh, perfumed substancesSkin is rinsed and dried well, apply lotion Maria Carmela L. Domocmat, RN, MSN
  30. 30. Maintain skin integrityAvoid excess powder and other dryingsubstancesDry skin thoroughly by patting rather thanrubbingApply lotion liberally to dry skin areasAvoid powder or other drying agents (e.g.,rubbing alcohol)Use cosmetics that contain moisturizersInspect skin daily for open areas and rashes Maria Carmela L. Domocmat, RN, MSN
  31. 31. For alopeciaRecommend use of mild protein shampoosAvoid harsh treatments (e.g., permanents orhighlights) until hair regrows during remission Maria Carmela L. Domocmat, RN, MSN
  32. 32. Severe or life-threatening symptomsHemolytic anemia, extensive heart or lunginvolvement, kidney disease, or central nervoussystem involvementoften require more aggressive treatment bydoctor specialists. Maria Carmela L. Domocmat, RN, MSN
  33. 33. it is also important to have: Preventive heart care Up-to-date immunizations Tests to screen for thinning of the bones (osteoporosis) Talk therapy and support groups may help relieve depression and mood changes that may occur in patients with this disease. Maria Carmela L. Domocmat, RN, MSN
  34. 34. Outlook (Prognosis)How well a person does depends on the severityof the disease.The outcome for people with SLE has improvedin recent years. Many people with SLE have mildsymptoms. Maria Carmela L. Domocmat, RN, MSN
  35. 35. Outlook (Prognosis)Women with SLE who become pregnant areoften able to carry safely to term and deliver ahealthy infant, as long as they do not havesevere kidney or heart disease and the SLE isbeing treated appropriately. However, thepresence of SLE antibodies may increase therisk of pregnancy loss. Maria Carmela L. Domocmat, RN, MSN
  36. 36. Possible ComplicationsSome people with SLE have abnormal depositsin the kidney cells. This leads to a conditioncalled lupus nephritis. Patients with thiscondition may eventually develop kidneyfailure and need dialysis or a kidney transplant. Maria Carmela L. Domocmat, RN, MSN
  37. 37. Possible ComplicationsSLE causes damage to many different parts ofthe body, including: DVT or pulmonary embolism hemolytic anemia pericarditis, endocarditis, or myocarditis Fluid around the lungs (pleural effusions) and damage to lung tissue Maria Carmela L. Domocmat, RN, MSN
  38. 38. Possible ComplicationsPregnancy complications, including miscarriage Stroke thrombocytopenia Inflammation of the blood vessels Maria Carmela L. Domocmat, RN, MSN
  39. 39. http://t1.gstatic.com/images?q=tbn:ANd9GcSNZJeyTzNT2-n9x75SxwCiHpxGRsPy_cT_qqDvE5lc04P94kRW Maria Carmela L. Domocmat, RN, MSN
  40. 40. Cause is unknown.People with this condition have a buildup of asubstance called collagen in the skin and otherorgans. This buildup leads to the symptoms ofthe disease. Maria Carmela L. Domocmat, RN, MSN
  41. 41. Incidence and risk factors30 to 50 years old.Womenhistory of being around silica dust and polyvinylchloridecan occur with other autoimmune diseases,including SLE and polymyositis. In such cases, the disorder is referred to as mixed connective disease. Maria Carmela L. Domocmat, RN, MSN
  42. 42. ManifestationsTypes: localized and systemicLocalized scleroderma affects only the skin on the hands and face. develops slowly, and rarely, if ever, spreads throughout the body or causes serious complications. Maria Carmela L. Domocmat, RN, MSN
  43. 43. ManifestationsSystemic scleroderma, or sclerosis may affect large areas of skin and organs such as the heart, lungs, or kidneys. There are two main types of systemic scleroderma: diffuse disease limited disease (CREST syndrome) Maria Carmela L. Domocmat, RN, MSN
  44. 44. Diffuse, cutaneous sclerodermaearly: bilateral symmetrical swelling of fingers,face, feetskin – tense, wrinkle-free appearanceas progress: skin- more thickened, hidebound,shinyface- mask-likemouth- rigiddevelop also in GIT, heart, lungs, kidneyschanges progress rapidly Maria Carmela L. Domocmat, RN, MSN
  45. 45. Limited cutaneous sclerodermaskin changes confined to fingers and distalportions of extremities and faceslow progressionUsually develops CREST Maria Carmela L. Domocmat, RN, MSN
  46. 46. Maria Carmela L. Domocmat, RN, MSN
  47. 47. Raynauds phenomenonis characterized byfingers becoming whitedue to lack of blood flow,then blue due to oxygenconsumption, and finallyred as blood flow returns. Maria Carmela L. Domocmat, RN, MSN
  48. 48. Sclerodactyly The most classic symptom of scleroderma is a type of skin tightening called sclerodactyly. The initial stages of the disease involves swelling of the fingers. Later, as the connective tissue becomes fibrotic, skin on the fingers and toes becomes hard and shiny. The fingers can become difficult to bend and can form contractures due to the severe tightening of the skin.Maria Carmela L. Domocmat, RN, MSN
  49. 49. Maria Carmela L. Domocmat, RN, MSN
  50. 50. Telangiectasiais the dilation of smallsuperficial vessels andcapillaries that causenumerous flat red markson the hands, face andtongue. Telangiectasiacan be a symptom ofscleroderma or othersystemic diseases. Maria Carmela L. Domocmat, RN, MSN
  51. 51. Bone and muscle symptoms may include: Joint pain Numbness and pain in the feet Pain, stiffness, and swelling of fingers and joints Wrist pain Maria Carmela L. Domocmat, RN, MSN
  52. 52. Breathing problems may result from scarring inthe lungs and can include: Dry cough Shortness of breath Wheezing Maria Carmela L. Domocmat, RN, MSN
  53. 53. Digestive tract problems may include: Bloating after meals Constipation Diarrhea Difficulty swallowing Esophageal reflux or heartburn fecal incontinence Maria Carmela L. Domocmat, RN, MSN
  54. 54. Dx testsmild hemolytic anemia – due to mechanicaldamage of diseased small vesselsmild hypergammaglobulinemia (IgG)(+)RFproteinuria(+) ANA titers(+) SCL-70: serum anticentromere antibody Maria Carmela L. Domocmat, RN, MSN
  55. 55. Dx testsdefinitive dx requires presence of one major or 2minor criteria major: for proximal scleroderma –skin thickening and tightening of areas proximal to MCP joints; can affect entire face, extremity, neck, thorax, abdomen minor: scleorodactyly digital pitting scars bibasilar pulmonary fibrosis Maria Carmela L. Domocmat, RN, MSN
  56. 56. Medical managementThere is no specific treatment for scleroderma.Reduce inflammation, sclerosis, vasospasmReduce renal complicationsTreat pulmonary arterial hypertension (PAH) Maria Carmela L. Domocmat, RN, MSN
  57. 57. Nursing managementFacilitate muscle and joint movementMaintain skin integrityProvide educationPromote adequate nutritionPromote bowel eliminationMonitor for complications Maria Carmela L. Domocmat, RN, MSN
  58. 58. Reduce inflammation, sclerosis, vasospasmvasoactive agents: calcium-channel blockers –nifedipine 10-20 mg TIDanti-inflamm agents: Glucoccorticoids (low-dosesteroid therapy) – 10mg/dayimmunosuppressive agents orimmunomodulating agent : penicillamine 125 mg/day start: gradual increase has numerous toxic effects explain importance of periodic lab: liver, renal function Maria Carmela L. Domocmat, RN, MSN
  59. 59. Reduce inflammation, sclerosis, vasospasmminocycline: for diarrhea assoc withmalabsorption syndromeProton pump inhibitor: reduce acidity of gastricreflux Maria Carmela L. Domocmat, RN, MSN
  60. 60. Reduce renal complications ACE inhibitors: control hyperreninemia; prevent HTN and renal crisis Maria Carmela L. Domocmat, RN, MSN
  61. 61. Treat pulmonary arterial hypertension (PAH) PAH – leading cause of mortality Epoprostenol (Flolan) Bosentan (Tracleer) Terprostinil (Remodulin)Facilitate muscle movement full ROM of mouth and hands Maria Carmela L. Domocmat, RN, MSN
  62. 62. maintain skin integrity acutely ill: all digits and extremities – handle carefully and gently position self – for minimal discomfort pressure-reducing beds or air mattresses remove dressing carefully tape used only when absolutely essential IV, IM, etc – should only be in sites free of fibrosis and sclerosis –areas of tough, thickened skin and sclerotic veins cannot be easily punctured Maria Carmela L. Domocmat, RN, MSN
  63. 63. provide education modify dress protective clothing in cold weather eliminate use of vasoconstrictive substances Maria Carmela L. Domocmat, RN, MSN
  64. 64. promote adequate nutrition easy-to-swallow, high calorie snacks avoid foods contributing to esophagitis and gastric reflux remain upright for 1 to 2 hrs after eating avoid heavy snacks close to retiring use large wedge pillows – to elevate head and shoulder use small-angled toothbrush facial exercises Maria Carmela L. Domocmat, RN, MSN
  65. 65. promote bowel elimination eat high-fiber foods more fluids exercise eliminate food that precipitate diarrhea Maria Carmela L. Domocmat, RN, MSN
  66. 66. monitor for complications monitor potential complications supportive therapy – O2 educate factors that exacerbate pulmo condition (pollen, smoking, humidity) Maria Carmela L. Domocmat, RN, MSN
  67. 67. Outlook (Prognosis)Some people with scleroderma have symptomsthat develop quickly over the first few years andcontinue to get worse. However, in mostpatients, the disease slowly gets worse.People who only have skin symptoms have abetter outlook. Widespread (systemic)scleroderma can damage the heart, kidney,lungs, or GI tract, which may cause death.Lung problems are the most common cause ofdeath in patients with scleroderma. Maria Carmela L. Domocmat, RN, MSN
  68. 68. Prognosis: 10 year survival rate – 65% Maria Carmela L. Domocmat, RN, MSN
  69. 69. Possible Complications The most common cause of death in people with scleroderma is scarring of the lungs, called pulmonary fibrosis Other complications of scleroderma include: Cancer Heart failure High blood pressure in the lungs (pulmonary hypertension) Kidney failure Problems absorbing nutrients from food (malabsorptionhttp://www.nlm.nih.gov/medlineplus/ency/article/000429.htm Maria Carmela L. Domocmat, RN, MSN