Connective tissue disorders

1. Connective tissue disorders Jimmy Hall, RN, BSN

2. OBJECTIVES Describe care for patients with systemic lupus erythematosus (SLE) Describe systemic effects of SLE Devise a teaching plan for the SLE patient Identify modifications in lifestyle

3. Connective tissue disorders • Involves a family of similar disorders • Rheumatoid Arthritis (RA) • Systemic Lupus Erythematosus (SLE) • Polymyositis • Scleroderma • Polymyalgia rheumatica

4. Common features • Women are affected more frequently • “Multisystem” diseases • Many overlapping features • For example: • Lupus/ Scleroderma Symptoms: Scleroderma features thickened skin from excess collagen, leading to scar tissue while SLE features a rashes due to skin inflammation and a “butterfly” rash to face related to photosensitivity • Lupus/ Rheumatoid Arthritis Symptoms: Lupus (joint pain), RA (joint swelling) Lab tests: Sed rate, C-reactive Protein, ANA

5. Polymyositis Inflammation of many muscles Causes muscle weakness Shoulders, hips, thighs 5-10 cases per million adults per year Treatement: high-dose corticosteroid therapy or immunosuppressants

6. Scleroderma “Hard Skin” due to collagen deposits in skin and other parts of the body 18-20 people per million per year Can be localized (to hands or feet) or can be systemic (affecting heart, lungs, kidneys, and intestines) No one test can diagnose Treatment depends on symptoms

7. Scleroderma Features Raynaud’s Phenomenon Swelling in hands Hard and rigid skin Dry skin (because sweat secretion is suppressed) Extremities stiffen Expressionless face Heart failure Difficulty swallowing (esophageal stiffening) Lungs scarred (affecting respiration) Hardening of intestinal mucosa Renal failure

8. Polymyalgia Rheumatica Involves proximal muscle discomfort and joint swelling Neck, shoulder, and pelvic muscles Occurs predominantly in Caucasians Patients are usually in people 50 years or older 52 cases per 100,000 people annually Usually have a first-degree relative with disease Genetic marker HLA-DR4 Diagnosis made by elimination Treatment: corticosteroids, NSAIDs

9. Polymyalgia Rheumatica Symptoms Fever Weight Loss/Anorexia Fatigue Depression Giant cell arteritis Headache with changes in vision and jaw claudication

10. Lupus Erythematosus • Four types: • Discoid lupus erythematosus (DLE) • Drug-induced lupus erythematosus • Neonatal lupus • System lupus erythematosus (SLE) • **Most common and most severe**

11. Did you Know? • What does “lupus” mean in Latin? a. Evil b. Wolf c. Suffering d. Butterfly

12. Wolf • Some doctors thought the rash resembled the pattern of fur on a wolf's face, and some thought that the rash resembled an attack of an animal. • One account claims that the term was given after a French mask that women reportedly wore to conceal the rash on their faces. The mask is called a loup, French for “wolf.”

13. Discoid Lupus Erythmatosus • Chronic skin condition of sores with scarring and inflammation • Often affects the face, scalp, and ears • Sensitive to sunlight • Corisone ointment used to slow progression and to improve the lesions. • Plaquenil is often prescribed – yearly eye exam!

14. Drug Induced Lupus Erythematosus • Results from long-term use of certain medications • Occurs often in men over age 50 because of the higher rate of having chronic diseases that require these medications. • Symptoms • Often complain of flu-like symptoms • Muscle pain • Joint Pain • Fever • Arthritis

15. Drug Induced Lupus Erythematosus • Medicines often linked to this are used to treat: • Heart Disease • Thyroid Disease • Hypertension • Neuropsychiatric disorders • Over 38 drugs have been linked • Procainamide (Pronestyl) – for arrhythmias • Hydralazine (Apresoline) – for hypertension • Isoniazid (Laniazid) – antibacterial • It is important to identify which medication is causing the problem and to stop the medication!

16. Neonatal Lupus • Rare disorder caused by the transplacental passage of maternal autoantibodies • Only 1% of infants with positive maternal autoantibodies develop neonatal lupus erythematosus. • The most common clinical manifestations are cardiac, dermatologic, and hepatic. • The onset of neonatal lupus erythematosus occurs between birth and a few months of life.

17. Systemic Lupus Erythematosus • Chronic, autoimmune disease affecting skin, joints, and possibly other organs • An increase in autoantibody production, resulting from suppressor T-cell function, leads to immune complex deposition and tissue damage • Inflammation stimulates antigens, which in turn stimulate additional antibodies, and this repeats • The body attacks own tissues and organs anywhere in the body • Skin • Kidneys • Heart

18. SLE cause Genetic factors Hormonal factors Environmental factors Medications

19. How is SLE different from HIV/AIDS? • HIV/AIDS is an immune deficiency disorder, where the immune system fails in fighting off infection. • SLE involves a hyperactive immune system that involves the immune system fighting itself.

20. SLE facts • 1 per 1,000 to 2,000 people • It is a disease of flare-ups • Women age 15-44 are most affected. • Women develop SLE ten times more often than men. • It is three times more common in African Americans than Caucasians

21. Mouth sores Hair loss Raynaud’s phenomenon Chest pain Dry eyes Easy Bruising Anxiety Depression Systemic Lupus Erythematosus • Sypmtoms • Fatigue • Fever • Weight loss or gain • Joint Pain, stiffness and swelling • Butterfly Rash on face* • Skin lesions • Memory Loss

22. SLE and the body Pericarditis is the most common cardiac manifestation Serum creatinine levels and urinalysis are used to screen for renal involvement Renal involvement can lead to hypertension

24. A butterfly rash occurs in more than 50% of SLE patients

26. Complications

27. Did You Know? Renal failure and intercurrent infection are the most common causes of death associated with SLE – Lupus Foundation of America http://www.lupus.org/webmodules/webarticlesnet/templates/new_learnunderstanding.aspx?articleid=2238&zoneid=523

28. SLE Diagnosis • Diagnosis is based on a complete history, physical exam, and blood tests. • The American College of Rheumatology (ACR) has listed 11 criteria for diagnosis of SLE. • ACR states that if you have 4 or more of the 11 criteria you probably have lupus. http://www.lupusresearchinstitute.org/lupus/lupus_diagnosis

29. SLE Criteria Set Forth by ACR • Scaly, raised rash, called a discoid rash • Facial rash that is butterfly shaped and covers the bridge of the nose and spreads across the cheeks • Mouth Sores, usually painless • Sun-related rash, appearing after exposure to sunlight • Joint pain and swelling that occurs in two or more joints • Kidney disease • Swelling of the linings around the lungs or the heart • Low blood counts, such as anemia, thrombocytopenia, or leukopenia • A neurological disorder, such as seizures or psychosis • Positive ANA test • Other positive blood tests that may indicate an autoimmune disease

30. SLE Diagnosis • No single lab tests confirms SLE • Lab tests reveal • Anemia • Thrombocytopenia • Leukocytosis • Positive ANA

31. SLE Diagnosis Lab tests Antinuclear Antibody (ANA) Test Erythrocyte Sedimentation Rate (Sed Rate) Coomb’s test C-reactive Protein Other tests: Complete Blood Count (CBC) Urinalysis Kidney and Liver assessment Chest X-ray Electrocardiogram Syphilis test

32. Antinuclear Antibody (ANA) Test Antinulcear antibodies are a unique group of autoantibodies that have the ability to attack structures in the nucleus of cells. Nonspecific A positive (+) ANA test can indicate autoimmune disease

33. Erythrocyte Sedimentation Rate (Sed Rate) Checks for inflammation within the body Nonspecific The normal sedimentation rate is: In males is 0-15 mm/hour In females is 0-20 mm/hour

34. Coomb’s Test Looks for antibodies that bind to blood cells and cause premature red blood cell destruction Two types: Direct – used to detect if antibodies are bound to the surface of the red blood cell; often used to detect cause of anemia or jaundice Indirect – searches for unbound antibodies; often used to detect if a reaction to a blood transfusion A positive result indicates that there are antibodies acting against your red blood cells

35. C-Reactive Protein Test CRP is produced by the liver It is present when there is inflammation present in the body Often used to detect if treatment is working Elevated CRP levels indicated that there is an acute inflammation present.

36. Does an elevated SED rate diagnose someone with SLE? No. It only indicates that there is an inflammation.

37. Assessment Skin Check for rashes or pigment changes Ask if there has been any skin changes and about sensitivity to sunlight/UV light Heart Auscultation for pericardial friction rub, which is associated with pleural effusions and myocarditis Mouth Check for ulcerations (GI involvement) Neurological Psychosis, depression, or seizures Joints Tenderness, swelling, warmth, pain during movement, and edema Joint involvement is symmetric and similar to that of RA

38. TREATMENT Goals: Prevent progressive loss of organ function Reduce the likelihood of acute disease Minimize disease-related disabilities Prevent complications from therapy

39. TREATMENT Corticosteroids Nonsteroidal anti-inflammatory drugs (NSAIDs) Antimalarial Drugs Other potential medications: Antidepressants Narcotics

40. CORTICOSTEROIDS ***Single most important medication for treatment*** • Used topically, orally, or intravenous (IV) • Action • Block the production of substances that trigger allergic and inflammatory actions, such as prostaglandins. • They also impede the function of white blood cells which destroy foreign bodies and help keep the immune system functioning properly. • Side effects: • Osteoporosis • Hyperglycemia • Infection

41. Nonsteroidal anti-inflammatory drugs (NSAIDs) Acions: Block cyclooxygenase (COX) enzymes and reduce prostaglandin Reduce pain, inflammation, and fever Often taken with corticosteroids to minimize the requirement of corticosteroids Commonly used NSAIDs: Aspirin Naproxen (Aleve) Ibuprofen Side effects: Stomach ulcers Bleeding

42. Antimalarial Drugs Hydroxychloroquine (Plaquenil) is most commonly prescribed. Mechanism of action is unknown Plaquenil improves: Inflammation Muscle and joint pain Fatigue Skin inflammation

43. Hydroxychloroquine (Plaquenil) • Side Effects: • Nausea • Stomach cramps • Photosensitivity • Avoid UV light exposure • Wear Sunscreen • Eye problems • Color blind • Loss of vision

44. Hydroxychlorquine (Plaquenuil) • One of the worst side effects of taking Plaquenil is damage to the retina at the back of the eye. • Have a baseline eye exam • Have eye exams every 6-12 months after initiating therapy • Problems mostly occur when: • kidney problems are present • Taking medication for over 10 years

45. Sit in an area with good lighting, wear your reading glasses and look at the grid at a comfortable position (30-40cm). Cover one eye Look directly at the center dot. While looking directly at the dot note whether all lines of the grid are straight or if any areas are distorted, blurred or dark. Repeat the test with the other eye

46. If you experience: • distortion or curvy lines • blurring • holes or spots in some areas of the grid • SEE YOUR OPHTHALMOLOGIST IMMEDIATELY! http://sw-eng.com/plaquenil-eyes.html

47. Treatment continued • Immunosuppressives • Medications that suppress the immune system • Used mainly in severe cases of SLE who have not responded to other therapies • Serious Side Effects (ex: infection, liver damage, infertility, increased risk of cancer) • Commonly used: • Cyclophosphamide (Cytoxan) • Azathioprine (Imuran, Azasan) • Methotrexate • Mycophenolate (CellCept)

48. Alternative Medicine • Omega-3 • Fish Oil • Flaxseed • Cayenne (Capsicum) creams - analgesic

49. fish oil • Contains two omega-3: eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) • Actions: • Fight inflammation • Reduce fatigue • Stimulate circulation • Also: reduce triglycerides, heart rate, blood pressure, atherosclerosis, and increases brain activity • Side effects: • Bloating • Belching • Gas • Diarrhea

50. Flaxseed Oil • Contains an omega-3 called alpha-linolenic acid (ALA) • Decreases inflammation in the body • Improves kidney function • Common Side Effects: • Bloating • Abdominal pain