CONNECTIVE TISSUE DISORDERSJimmy Hall, RN, BSN

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

CONNECTIVE TISSUE DISORDERS

Involves a family of similar disorders Rheumatoid Arthritis (RA) Systemic Lupus Erythematosus (SLE) Polymyositis Scleroderma Polymyalgia rheumatica

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 ArthritisSymptoms: Lupus (joint pain), RA (joint swelling)Lab tests: Sed rate, C-reactive Protein, ANA

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

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

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

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

Polymyalgia Rheumatica Symptoms

Fever Weight Loss/Anorexia Fatigue Depression Giant cell arteritis

Headache with changes in vision and jaw claudication

LUPUS ERYTHEMATOSUS

Four types:1. Discoid lupus erythematosus (DLE)2. Drug-induced lupus erythematosus3. Neonatal lupus4. System lupus erythematosus (SLE)

**Most common and most severe**

DID YOU KNOW?

What does “lupus” mean in Latin? a. Evil b. Wolf c. Suffering d. Butterfly

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.”

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!

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

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!

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.

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

SLE cause

Genetic factors Hormonal factors Environmental

factors Medications

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.

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

SYSTEMIC LUPUS ERYTHEMATOSUS Sypmtoms

Fatigue Fever Weight loss or gain Joint Pain, stiffness and swelling Butterfly Rash on face* Skin lesions Memory Loss

Mouth sores Hair loss Raynaud’s

phenomenon Chest pain Dry eyes Easy Bruising Anxiety Depression

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

A butterfly rash occurs in more than 50% of SLE

patients

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

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

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

SLE DIAGNOSIS

No single lab tests confirms SLE Lab tests reveal

Anemia Thrombocytopenia Leukocytosis Positive ANA

SLE Diagnosis

Lab testsAntinuclear Antibody (ANA) TestErythrocyte Sedimentation Rate (Sed

Rate)Coomb’s testC-reactive Protein

Other tests: Complete Blood Count (CBC) Urinalysis Kidney and Liver assessment Chest X-ray Electrocardiogram Syphilis test

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

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

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

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.

Does an elevated SED rate diagnose someone with SLE?

No. It only indicates that there is an inflammation.

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

TREATMENT

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

TREATMENT

Corticosteroids Nonsteroidal anti-inflammatory drugs

(NSAIDs) Antimalarial Drugs

Other potential medications: Antidepressants Narcotics

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

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

Antimalarial Drugs Hydroxychloroquine (Plaquenil) is most commonly

prescribed. Mechanism of action is unknown Plaquenil improves:

Inflammation Muscle and joint pain Fatigue Skin inflammation

HYDROXYCHLOROQUINE (PLAQUENIL)

Side Effects: Nausea Stomach cramps Photosensitivity

Avoid UV light exposure Wear Sunscreen

Eye problems Color blind Loss of vision

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

1. Sit in an area with good lighting, wear your reading glasses and look at the grid at a comfortable position (30-40cm).

2. Cover one eye 3. 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. 4. Repeat the test with the other eye

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

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)

ALTERNATIVE MEDICINE

Omega-3 Fish Oil Flaxseed

Cayenne (Capsicum) creams - analgesic

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

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

DID YOU KNOW?

Michael Jackson had Lupus

LIFESTYLE CHANGES

Avoid Sunlight Control Infection Birth Control Get plenty of rest Don’t smoke Eat a healthy diet

Therapy

Physical therapy can be beneficial in order to: Improve range of motion and functional mobility Identify exercises that are beneficial for the

patient Occupational therapy can benefit the patient

by Restoring functional independence (bathing,

feeding, toileting) Identifying appropriate equipment (raised toilet

seats, splints, elastic shoelaces)

Nursing Diagnoses… What to do? Fatigue

Sleep Rest Exercise Treat Depression

Impaired skin integrity Avoid sun/UV light

exposure Wear sunscreen/clothing

when exposed

Body image disturbance Support groups Education: rash can come

and go Medication

Lack of knowledge for self-management decisions Educate patient Disease involves multiple

systems Avoid sunlight Dietary changes due to

increased risk for cardiovascular disease

Potential side effects Follow up care

ANY QUESTIONS?

Post Test

Which of the following is not a feature of Systemic Lupus Erythematosus:

It affects women more than men It affects Caucasians more that African

Americans. It primarily affects those ages 15-44. It is commonly associated with a butterfly rash.

Which of the following is not a feature of Systemic Lupus Erythematosus:

It affects women more than men It affects Caucasians more that African

Americans. It primarily affects those ages 15-44. It is commonly associated with a butterfly rash.

When caring for a patient taking hydroxychlorquine (Plaquenil), the nurse overhears the patient’s conversation. What statement by the patient indicates teaching is needed?

“Me and my friend are going to eat seafood this afternoon.”

“It doesn’t matter if I am around my child when taking this medicine.”

“It’s been 5 months since I got my eyes last checked.”

“I am taking my first trip to the beach next week, and I am so excited!”

When caring for a patient taking hydroxychlorquine (Plaquenil), the nurse overhears the patient’s conversation. What statement by the patient indicates teaching is needed?

“Me and my friend are going to eat seafood this afternoon.”

“It doesn’t matter if I am around my child when taking this medicine.”

“It’s been 5 months since I got my eyes last checked.”

“I am taking my first trip to the beach next week, and I am so excited!”

Systemic Lupus Erythematosus is an autoimmune disorder characterized by a hyperactive immune system attacking itself.

True False

Systemic Lupus Erythematosus is an autoimmune disorder characterized by a hyperactive immune system attacking itself.

True False

A positive ANA is diagnostic that a patient has Systemic Lupus Erythematosus

True False

A positive ANA is diagnostic that a patient has Systemic Lupus Erythematosus

True False

A 27 year old female, diagnosed with Systemic Lupus Erythmatosus, has recently started taking Methotrexate for her disease. She informs her nurse that she and her husband have decided to have a baby. What should the nurse’s response be?

“So, that’s why the doctor started you on Methotrexate. I am so excited for you.”

“That is great! Your disease must be doing better.” “That’s not a great idea considering you are taking

Methotrexate ” “I would not recommend that you have a baby.

Women who have children always pass on the disease to their children.”

A 27 year old female, diagnosed with Systemic Lupus Erythmatosus, has recently started taking Methotrexate for her disease. She informs her nurse that she and her husband have decided to have a baby. What should the nurse’s response be?

“So, that’s why the doctor started you on Methotrexate. I am so excited for you.”

“That is great! Your disease must be doing better.” “That’s not a great idea considering you are

taking Methotrexate ” “I would not recommend that you have a baby.

Women who have children always pass on the disease to their children.”

THE END…

References: Lupus Foundation of America, Inc. (2009). Prognosis and a hopeful

future. Retrieved February 15, 2010, from http://www.lupus.org/webmodules/webarticlesnet/templates/new_learnunderstanding.aspx?articleid=2238&zoneid=523

Lupus Research Institute. (n.d.). Lupus diagnosis. Retrieved February 15, 2010, from http://www.lupusresearchinstitute.org/lupus/lupus_diagnosis

Smeltzer, S., Bare, B., Hinkle, J., & Cheever, K. (2008). Textbook of medical- surgical nursing (Eleventh ed.). New York: Lippincott, Williams & Wilkins

Software Engineering. (n.d.). Plaquenil & your eyes. Retrieved February 15, 2010, from http://sw-eng.com/plaquenil-eyes.html