Download Connective tissue disorders

CONNECTIVE TISSUE DISORDERS
Jimmy 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
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Involves a family of similar disorders
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Rheumatoid Arthritis (RA)
Systemic Lupus Erythematosus (SLE)
Polymyositis
Scleroderma
Polymyalgia rheumatica
COMMON FEATURES
Women are affected more frequently
 “Multisystem” diseases
 Many overlapping features
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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
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Polymyositis
Inflammation of many muscles
 Causes muscle weakness
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Shoulders, hips, thighs
5-10 cases per million adults per year
Treatement: high-dose corticosteroid therapy or
immunosuppressants
Scleroderma
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“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
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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

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Genetic marker HLA-DR4
Diagnosis made by elimination
 Treatment: corticosteroids, NSAIDs
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Polymyalgia Rheumatica
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Symptoms
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Fever
Weight Loss/Anorexia
Fatigue
Depression
Giant cell arteritis
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Headache with changes in vision and jaw claudication
LUPUS ERYTHEMATOSUS

Four types:
1.
2.
3.
4.
Discoid lupus erythematosus (DLE)
Drug-induced lupus erythematosus
Neonatal lupus
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
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Often complain of flu-like symptoms
Muscle pain
 Joint Pain
 Fever
 Arthritis
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DRUG INDUCED LUPUS ERYTHEMATOSUS
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Medicines often linked to this are used to treat:
Heart Disease
 Thyroid Disease
 Hypertension
 Neuropsychiatric disorders
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Over 38 drugs have been linked
Procainamide (Pronestyl) – for arrhythmias
 Hydralazine (Apresoline) – for hypertension
 Isoniazid (Laniazid) – antibacterial
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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
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Skin
 Kidneys
 Heart
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SLE cause
Genetic factors
 Hormonal factors
 Environmental factors
 Medications
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HOW IS SLE DIFFERENT FROM HIV/AIDS?
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HIV/AIDS is an immune deficiency disorder, where the
immune system fails in fighting off infection.
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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
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SYSTEMIC LUPUS ERYTHEMATOSUS
 Sypmtoms
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 Mouth sores
Fatigue
 Hair loss
Fever
 Raynaud’s
Weight loss or gain
phenomenon
Joint Pain, stiffness and swelling
 Chest pain
Butterfly Rash on face*
 Dry eyes
Skin lesions
 Easy Bruising
Memory Loss
 Anxiety
 Depression
SLE and the body
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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.
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http://www.lupusresearchinstitute.org/lupus/lupus_diagnosis
SLE CRITERIA SET FORTH BY ACR
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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
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Anemia
 Thrombocytopenia
 Leukocytosis
 Positive ANA
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SLE Diagnosis
 Lab
tests
Antinuclear Antibody (ANA) Test
 Erythrocyte Sedimentation Rate (Sed Rate)
 Coomb’s test
 C-reactive Protein
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Other tests:
 Complete Blood Count (CBC)
 Urinalysis
 Kidney and Liver assessment
 Chest X-ray
 Electrocardiogram
 Syphilis test
Antinuclear Antibody (ANA) Test
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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)
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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
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Coomb’s Test
Looks for antibodies that bind to blood cells and
cause premature red blood cell destruction
 Two types:
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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
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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.
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Does an elevated SED rate diagnose
someone with SLE?
No. It only indicates that there is an
inflammation.
Assessment
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Skin
Check for rashes or pigment changes
 Ask if there has been any skin changes and about
sensitivity to sunlight/UV light
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Heart
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Mouth
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Check for ulcerations (GI involvement)
Neurological
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Auscultation for pericardial friction rub, which is
associated with pleural effusions and myocarditis
Psychosis, depression, or seizures
Joints
Tenderness, swelling, warmth, pain during movement, and
edema
 Joint involvement is symmetric and similar to that of RA
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TREATMENT
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Goals:
Prevent progressive loss of organ function
 Reduce the likelihood of acute disease
 Minimize disease-related disabilities
 Prevent complications from therapy
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TREATMENT
Corticosteroids
 Nonsteroidal anti-inflammatory drugs (NSAIDs)
 Antimalarial Drugs
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Other potential medications:
Antidepressants
 Narcotics
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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.
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Side effects:
Osteoporosis
 Hyperglycemia
 Infection
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Nonsteroidal anti-inflammatory drugs
(NSAIDs)
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Acions:
Block cyclooxygenase (COX) enzymes and reduce
prostaglandin
 Reduce pain, inflammation, and fever
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Often taken with corticosteroids to minimize the
requirement of corticosteroids
Commonly used NSAIDs:
Aspirin
 Naproxen (Aleve)
 Ibuprofen
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Side effects:
Stomach ulcers
 Bleeding
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Antimalarial Drugs
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Hydroxychloroquine (Plaquenil) is most commonly
prescribed.
Mechanism of action is unknown
Plaquenil improves:
Inflammation
 Muscle and joint pain
 Fatigue
 Skin inflammation
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HYDROXYCHLOROQUINE (PLAQUENIL)
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Side Effects:
Nausea
 Stomach cramps
 Photosensitivity
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Avoid UV light exposure
 Wear Sunscreen
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Eye problems
Color blind
 Loss of vision
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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:
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kidney problems are present
 Taking medication for over 10 years
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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
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If you experience:
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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
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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:
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Cyclophosphamide (Cytoxan)
 Azathioprine (Imuran, Azasan)
 Methotrexate
 Mycophenolate (CellCept)
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ALTERNATIVE MEDICINE
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Omega-3
Fish Oil
 Flaxseed
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Cayenne (Capsicum) creams - analgesic
FISH OIL
Contains two omega-3: eicosapentaenoic acid
(EPA) and docosahexaenoic acid (DHA)
 Actions:
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Fight inflammation
 Reduce fatigue
 Stimulate circulation
 Also: reduce triglycerides, heart rate, blood pressure,
atherosclerosis, and increases brain activity
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Side effects:
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Bloating
Belching
Gas
Diarrhea
FLAXSEED OIL
Contains an omega-3 called alpha-linolenic acid
(ALA)
 Decreases inflammation in the body
 Improves kidney function
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Common Side Effects:
Bloating
 Abdominal pain
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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
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Therapy
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Physical therapy can be beneficial in order to:
Improve range of motion and functional mobility
 Identify exercises that are beneficial for the patient
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Occupational therapy can benefit the patient by
Restoring functional independence (bathing, feeding,
toileting)
 Identifying appropriate equipment (raised toilet
seats, splints, elastic shoelaces)
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Nursing Diagnoses… What to do?
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Fatigue
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Sleep
Rest
Exercise
Treat Depression
Impaired skin integrity
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Avoid sun/UV light
exposure
Wear sunscreen/clothing
when exposed
Body image disturbance
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Support groups
Education: rash can come
and go
Medication
Lack of knowledge for selfmanagement decisions
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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:
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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.
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Which of the following is not a feature of
Systemic Lupus Erythematosus:

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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?
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“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.

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True
False
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Systemic Lupus Erythematosus is an
autoimmune disorder characterized by a
hyperactive immune system attacking itself.
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True
False
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A positive ANA is diagnostic that a patient has
Systemic Lupus Erythematosus
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True
False
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A positive ANA is diagnostic that a patient has
Systemic Lupus Erythematosus
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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?
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“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?
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“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:
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Lupus Foundation of America, Inc. (2009). Prognosis and a hopeful future.
Retrieved
February 15, 2010, from
http://www.lupus.org/webmodules/webarticlesnet/t
emplates/new_learnunderstanding.aspx?articleid=2238&zoneid=52
3
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
medicalsurgical 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