Connective tissue disorders
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Transcript 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
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 Arthritis
Symptoms: 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.
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
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
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
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
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
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
Mouth
Check for ulcerations (GI involvement)
Neurological
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
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 selfmanagement 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/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