Rheumatic Disorders Part II: SLE, PA, SSc, Fibromyalgia, Lyme disease overview

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

Rheumatic Disorders
Comprise autoimmune and inflammatory
disorders
„the primary crippling disease”
Inflammation of joint
Primary reason for work-related disability
Leading cause of disability among 65 yrs old
and above


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

Reaction to Self

Occurs when the immune system sees “self”
antigens as “nonself”

may be due to genetic factors, infectious agents,
gender, and age

the autoimmune response results in tissue
damage
Some damage occurs in only one or a few organs, in
other cases it may be body-wide (systemic)

Reaction to Self

~ 3.5 % of people have autoimmune diseases
On average, women are 2.7 times more likely to
develop these diseases than men

most have no known cause or cure

treatment is aimed at controlling symptoms


Why does the immune system attack the body that
it‟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


Systemic Lupus Erythematosus (SLE)

a chronic systemic autoimmune disease
Complexes of anti-self antibodies and antigen deposit in, and
cause damage to, tissue
1 million sufferers in the U.S.
Strikes women nine times more often than men
symptoms may include butterfly-shaped rash on face,
fatigue, headaches
triggered by environmental effects in persons who are
genetically susceptible


Lupus sufferers develop IgG antibodies to
double stranded DNA and nuclear antigens.
Antibodies to T cells may also be produced.
Immune complexes formed during the disease
process may end up in the kidneys and then
cause damage by initiating an inflammatory
response that starts with complement fixation.
This may ultimately cause kidney failure.
Blood vessels and skin may also be attacked by
antibodies. Antibodies to erythrocytes are
sometimes produced.

s/s
Painful or swollen joints and muscle pain
Unexplained fever
Red rashes, most commonly on the face
Chest pain upon deep breathing
Unusual loss of hair
Pale or purple fingers or toes from cold or stress (Raynaud's
phenomenon)
Sensitivity to the sun
Swelling (edema) in legs or around eyes
Mouth ulcers
Swollen glands
Extreme fatigue


Diagnostic Tools for Lupus
Medical history
Complete physical examination
Laboratory 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 test
Skin biopsy
Kidney biopsy


Dx
Most specific tests
Seen exclusively in SLE
Antibodies to double-stranded DNA
Antibodies to Sm and ribosomal P proteins
Most sensitive test
ANA (anti-nuclear antibody) assay fluorescent


Systemic Lupus Erythematosus (SLE)

Damaged kidney (left) caused by
immunoglobulin deposits (right)

Butterfly rash of lupus

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.


The round or disk shaped
(discoid) rash of lupus
produces red, raised patches
with scales. The pores (hair
follicles) may be plugged.
Scarring often occurs in older
lesions. The majority
(approximately 90%) of
individuals with discoid lupus
have only skin involvement as
compared to more generalized
involvement in systemic lupus
erythematosis (SLE).


Lupus erythematosis
often produces a
"butterfly rash" or malar
rash.
Typically, the rash also
appears on the nose.


Lupus


exact trigger - not known
research has shown that the environmental
factors include
infectious agents
excess UV light
physical trauma
emotional stress
and some drugs (anticonvulsants, some forms of
penicillin, and drugs used in estrogen therapy)


Management
no cure for SLE.
goal of treatment - control symptoms


Management
Reduce inflammation and minimize
complications
NSAIDs - Aspirin
Glucoccorticoids
Hydroxychloroquine
Manage exacerbations
Promote orientation and decision-making
Provide seizure precaution
Monitor fluid status


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 symptoms

http://www.nlm.nih.gov/medlineplus/ency/article/000435.htm


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)



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


Drugs in clinical trial
Useful for maintenance in lupus nephritis and
other serious lupus cases; Inhibits antibody
production
Mycophenolate (CellCept)
Toterogens
Dehydroepiandrosterone (DHEA)
Stem cell transplant


Maintain skin integrity
Avoid prolonged exposure to sunlight and other
forms of UV lighting including tanning beds
wear protective clothing (long sleeves, large-
brimmed hat, long pants), sunglasses, and
sunscreen (SPF 30 or higher) when in the sun.
Clean skin with mild soap (e.g., ivory)
avoid harsh, perfumed substances
Skin is rinsed and dried well, apply lotion


Avoid excess powder and other drying
substances
Dry skin thoroughly by patting rather than
rubbing
Apply lotion liberally to dry skin areas
Avoid powder or other drying agents (e.g.,
rubbing alcohol)
Use cosmetics that contain moisturizers
Inspect skin daily for open areas and rashes


For alopecia
Recommend use of mild protein shampoos
Avoid harsh treatments (e.g., permanents or
highlights) until hair regrows during remission


Severe or life-threatening
symptoms
Hemolytic anemia, extensive heart or lung
involvement, kidney disease, or central nervous
system involvement
often require more aggressive treatment by
doctor specialists.


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.


Outlook (Prognosis)
How well a person does depends on the severity
of the disease.
The outcome for people with SLE has improved
in recent years. Many people with SLE have mild
symptoms.


Outlook (Prognosis)
Women with SLE who become pregnant are
often able to carry safely to term and deliver a
healthy infant, as long as they do not have
severe kidney or heart disease and the SLE is
being treated appropriately. However, the
presence of SLE antibodies may increase the
risk of pregnancy loss.


Possible Complications
Some people with SLE have abnormal deposits
in the kidney cells. This leads to a condition
called lupus nephritis. Patients with this
condition may eventually develop kidney
failure and need dialysis or a kidney transplant.


SLE causes damage to many different parts of
the body, including:
DVT or pulmonary embolism
hemolytic anemia
pericarditis, endocarditis, or myocarditis
Fluid around the lungs (pleural effusions) and
damage to lung tissue


Pregnancy complications, including miscarriage
Stroke
thrombocytopenia
Inflammation of the blood vessels


http://t1.gstatic.com/images?q=tbn:ANd9GcSNZJeyTzNT2-
n9x75SxwCiHpxGRsPy_cT_qqDvE5lc04P94kRW


Cause is unknown.
People with this condition have a buildup of a
substance called collagen in the skin and other
organs. This buildup leads to the symptoms of
the disease.


Incidence and risk factors
30 to 50 years old.
Women
history of being around silica dust and polyvinyl
chloride
can occur with other autoimmune diseases,
including SLE and polymyositis.
In such cases, the disorder is referred to as mixed
connective disease.


Manifestations
Types: localized and systemic

Localized scleroderma
affects only the skin on the hands and face.
develops slowly, and rarely, if ever, spreads
throughout the body or causes serious complications.


Manifestations
Systemic 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)


Diffuse, cutaneous scleroderma
early: bilateral symmetrical swelling of fingers,
face, feet
skin – tense, wrinkle-free appearance
as progress: skin- more thickened, hidebound,
shiny
face- mask-like
mouth- rigid
develop also in GIT, heart, lungs, kidneys
changes progress rapidly


Limited cutaneous scleroderma
skin changes confined to fingers and distal
portions of extremities and face
slow progression
Usually develops CREST


Raynaud's phenomenon
is characterized by
fingers becoming white
due to lack of blood flow,
then blue due to oxygen
consumption, and finally
red as blood flow returns.


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.


Telangiectasia
is the dilation of small
superficial vessels and
capillaries that cause
numerous flat red marks
on the hands, face and
tongue. Telangiectasia
can be a symptom of
scleroderma or other
systemic diseases.


Bone and muscle symptoms may include:
Joint pain
Numbness and pain in the feet
Pain, stiffness, and swelling of fingers and joints
Wrist pain


Breathing problems may result from scarring in
the lungs and can include:
Dry cough
Shortness of breath
Wheezing


Digestive tract problems may include:
Bloating after meals
Constipation
Diarrhea
Difficulty swallowing
Esophageal reflux or heartburn
fecal incontinence


Dx tests
mild hemolytic anemia – due to mechanical
damage of diseased small vessels
mild hypergammaglobulinemia (IgG)
(+)RF
proteinuria
(+) ANA titers
(+) SCL-70: serum anticentromere antibody


Dx tests
definitive dx requires presence of one major or 2
minor 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


Medical management
There is no specific treatment for scleroderma.
Reduce inflammation, sclerosis, vasospasm
Reduce renal complications
Treat pulmonary arterial hypertension (PAH)


Nursing management
Facilitate muscle and joint movement
Provide education
Promote adequate nutrition
Promote bowel elimination
Monitor for complications


Reduce inflammation, sclerosis,
vasospasm
vasoactive agents: calcium-channel blockers –
nifedipine 10-20 mg TID
anti-inflamm agents: Glucoccorticoids (low-dose
steroid therapy) – 10mg/day
immunosuppressive agents or
immunomodulating agent : penicillamine
125 mg/day start: gradual increase
has numerous toxic effects
explain importance of periodic lab: liver, renal function


Reduce inflammation, sclerosis,
vasospasm
minocycline: for diarrhea assoc with
malabsorption syndrome
Proton pump inhibitor: reduce acidity of gastric
reflux


Reduce renal complications
ACE inhibitors: control hyperreninemia; prevent HTN
and renal crisis


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


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


provide education
modify dress
protective clothing in cold weather
eliminate use of vasoconstrictive substances


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


promote bowel elimination
eat high-fiber foods
more fluids
exercise
eliminate food that precipitate diarrhea


monitor for complications
monitor potential complications
supportive therapy – O2
educate factors that exacerbate pulmo condition
(pollen, smoking, humidity)


Outlook (Prognosis)
Some people with scleroderma have symptoms
that develop quickly over the first few years and
continue to get worse. However, in most
patients, the disease slowly gets worse.
People who only have skin symptoms have a
better outlook. Widespread (systemic)
scleroderma can damage the heart, kidney,
lungs, or GI tract, which may cause death.
Lung problems are the most common cause of
death in patients with scleroderma.

Prognosis: 10 year survival rate – 65%


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 (malabsorption



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