Rheumatic Disorders: SLE, PA, SSc, Fibromyalgia, Lyme Disease

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

Maria Carmela L. Domocmat, RN, MSN
Instructor, Curative and Rehabilitative Nursing Care II
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


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).


This is a picture of a
systemic lupus
erythematosis rash on the
face. Lupus
erythematosis often
produces a "butterfly
rash" or malar rash.
Typically, the rash also
appears on the nose.


The rash of lupis is round or
disk shaped (discoid) and is
characterized by red, raised
patches with adherent scales.
The skin pores (follicles) may
be plugged, and scarring often
occurs in older lesions.
Approximately 90% of
individuals with discoid lupus
have only skin involvement as
compared to more generalized
involvement in systemic lupus
erythematosis (SLE).


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)

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


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
(such as 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


Psoriasis
is a common, chronic skin condition that causes
red patches on the body.


Skin cells grow deep in the skin and normally
rise to the surface about once a month. In
persons with psoriasis, this process is too fast
(about 2 weeks instead of 4 weeks) and dead
skin cells build up on the skin's surface.


Psoriasis on the knuckles
This is a picture of a typical case of psoriasis,
with small lesions on the knuckles. Note the
changes in the fingernails.


The following may trigger an attack of psoriasis or make
the condition more difficult to treat:
Bacteria or viral infections, including strep throat and upper
respiratory infections
Dry air or dry skin
Injury to the skin, including cuts, burns, and insect bites
Some medicines, including antimalaria drugs, beta-blockers,
and lithium
Stress
Too little sunlight
Too much sunlight (sunburn)
Too much alcohol


In general, psoriasis may be severe in people
who have a weakened immune system. This
may include persons who have:
AIDS
Autoimmune disorders (such as rheumatoid
arthritis)
Cancer chemotherapy
Up to one-third of people with psoriasis may also
have arthritis, a condition known as psoriatic
arthritis.


Symptoms
Psoriasis can appear suddenly or slowly. In
many cases, psoriasis goes away and then
flares up again repeatedly over time.
People with psoriasis have irritated patches of
skin. The redness is most often seen on the
elbows, knees, and trunk, but it can appear
anywhere on the body. For example, there may
be flaky patches on the scalp.


The skin patches or dots may be:
Itchy
Dry and covered with silver, flaky skin (scales)
Pink-red in color (like the color of salmon)
Raised and thick


Other symptoms may include:
Genital lesions in males
Joint pain or aching (psoriatic arthritis)
Nail changes, including nail thickening, yellow-
brown spots, dents (pits) on the nail surface, and
separation of the nail from the base
Severe dandruff on the scalp


five main types of psoriasis
Psoriasis may affect any or all parts of the skin.
Erythrodermic -- The skin redness is very intense and
covers a large area.
Guttate -- Small, pink-red spots appear on the skin.
Inverse -- Skin redness and irritation occurs in the
armpits, groin, and in between overlapping skin.
Plaque -- Thick, red patches of skin are covered by
flaky, silver-white scales. This is the most common
type of psoriasis.
Pustular -- White blisters are surrounded by red,
irritated skin.


Scalp psoriasis Nail Psoriasis


Plaque Psoriasis Pictures
(Vulgaris/Circular)


Guttate Psoriasis


Pustular Psoriasis


Inverse Psoriasis


Erythrodermic Psoriasis
particularly inflammatory form of psoriasis that
often affects most of the body surface.
least common type of psoriasis
may occur once or more during a lifetime in 1 to 2
percent of people who develop psoriasis


Psoriatic Arthritis


Treatment
The goal of treatment is to control symptoms
and prevent infections.
In general, three treatment options are used for
patients with psoriasis:
Topical medications such as lotions, ointments,
creams, and shampoos
Body-wide (systemic) medications, which are pills or
injections that affect the whole body, not just the skin
Phototherapy, which uses light to treat psoriasis


Treatment
Topical medications
such as lotions, ointments, creams, and shampoos
Topical steroids
Tar preparations
UV Light therapy


Topical meds
Cortisone creams and ointments
Suppress cell division when applied to psoriatic
lesions
More effective when penetrate the skin
How to enhance absorption: apply to skin, wrap with
warm, moist dressings and occlusive outer wrap of
plastic (film, gloves, booties, or similar garments)
Note: when large surface areas are involved – limit
occlusive therapy to 12 hrs/day
Bcoz increased risk of localand systemic S/E


Topical meds
Tar preparations
Creams ,ointments, solutions, lotions, gels, shampoos
that contain coal tar or anthralin
Coal tar – inpatient
messy, cause staining, and have unpleasant odor


Topical meds
Tar preparations
Anthralin (Anthraforte, Drithocreme, Lasan)
A hydrocarbon similar in action to tar
Relieves chronic psoriasis
Can be used alone or in combination with coal tar baths and
UV light
Apply to each lesion for short periods (not exceeeding 2 hrs)


Topical meds
Tar preparations
Anthralin (Anthraforte, Drithocreme, Lasan)
Not used to treat acute, spreading psoriasis – bcoz tend to
induce Koebner’s phenomenon
Koebner’s phenomenon – are psoriasis plaques that form at the
site of a skin injury.
Occurs when trauma to the skin causes a skin lesion. The
amount of trauma required can be very small -- sometimes just
rubbing the skin can cause a lesion to develop.
http://www.psoriasis.org/page.aspx?pid=1660


Topical meds
Prescription medicines containing vitamin D or
vitamin A (retinoids)
Cream, ointment, lotion
For mild to moderate
Calcipotriene (Dovonex)
Tazarotene (Tazorac)


Topical meds
Calcipotriene (Dovonex)
A synthetic form of vitamin D – regulates skin cell
division
Tazarotene (Tazorac)
Vit A derivative
Teratogenic - even when applied topically


Topical meds
Creams to remove the scaling (usually salicylic
acid or lactic acid)
Dandruff shampoos (over-the-counter or
prescription)
Moisturizers


UV light therapy
Or Phototherapy
uses light to treat psoriasis
Note: must wear eye protection during treatment
Types: UVA, UVB
Psoralen and UVA (PUVA)
Common in OPD
Client ingest psoralen – a photosensitizing agent- 2
hrs b4 exposure
2-3 times a week; not on consecutive days


UV light therapy


Observe for generalized redness with edema
and tenderness
Wear dark glasses during treatment and for the
remainder of the day – bcoz of the strong
photosensitizing properties of psoralen
Long term S/E of UVB and PUVA
Premature aging of skin
Actinic keratosis
Increased risk skin Ca


Systemic medications for severe
psoriasis
Immunosuppressants
Retinoids (acitretin)
DMARDs - Cytotoxic agents
Methotrexate or cyclosporine (Folex, Mexate)
C/I – liver damage, bone marrow supression, impaired function
Azathioprine (Imuran)
Cyclosporine (Neoral, Sandimmune)


BRMs (Biologics)
Alefacept (Amevive) – IM weekly x 12 wks
Efalizumab (Raptiva) – subq once/week
Adalimumab (Humira)
Etanercept (Enbrel)
Infliximab (Remicade)
Stelara


Self-care at home
Oatmeal baths
may be soothing and may help to loosen scales.
can use over-the-counter oatmeal bath products.
Or can mix 1 cup of oatmeal into a tub of warm water.
Sunlight may help symptoms go away. Be
careful not to get sunburned.
Relaxation and antistress techniques may be
helpful.


Psoriasis
Enhance body image
Provide emotional support


Tar - not only look dirty but unpleasant odor;
bed linens, pajamas become stained – further
discouraging social interaction
Encourage contact with other client with similar
problems
Group discussion with family members or SO –
can increase socialization process


Touch - more than any gesture communicates
acceptance of the person and their skin problem
Shake hands during introduction
Place hand on client’s shoulder when explaining
procedure
Do not wear gloves during social interactions


Expectations (prognosis)
Psoriasis is a life-long condition that can be
controlled with treatment.
may go away for a long time and then return.
With appropriate treatment - usually does not
affect general physical health.


Complications
Arthritis
Pain
Severe itching
Secondary skin infections
Side effects from medicines used to treat
psoriasis
Skin cancer from light therapy
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001470/


Psoriatic arthritis is an arthritis that is often
associated with psoriasis of the skin
20-50 years old
Women


Psoriatic arthritis
The cause of is not known
In general, people who have psoriasis have a
higher rate of arthritis than the general
population.


Symptoms
arthritis - may be mild and involve only a few
joints, particularly those at the end of the fingers
or toes.
In some - may be severe and affect many joints,
including the spine.
When spine is affected,
symptoms are stiffness, burning, and pain, most often
in the lower spine and sacrum.


Symptoms
People who also have arthritis usually have the
skin and nail changes of psoriasis. Often, the
skin gets worse at the same time as the arthritis.

Nail pitting in a patient with
psoriatic arthritis.


http://images.emedicinehealth.com/images/4453/4453-13248-30912-31165.jpg


Manage joint pain and inflammation
Same with RA
Control skin lesions
Same with SLE
Slow progression of disease


Treatment
NSAIDS
More severe arthritis - DMARDs, TNF inhibitors

Rest and exercise
Physical therapy may help increase the
movement of specific joints
heat and cold therapy


Expectations (prognosis)
The course of the disease is often mild and
affects only a few joints.
A few people will have severe psoriatic arthritis
in their hands, feet, and spine that causes
deformities.
In those with severe arthritis, treatment can still
be successful in relieving the pain.


Complications
Repeated episodes may occur.


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.


30 to 50 years old.
Women
history of being around silica dust and polyvinyl
chloride
can occur with other autoimmune diseases,
including systemic lupus erythematosus
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:
Limited disease (CREST syndrome)
diffuse disease.


Skin symptoms
Fingers or toes that turn blue or white in response to hot
and cold temperatures
Hair loss
Skin hardness
Skin that is abnormally dark or light
Skin thickening, stiffness, and tightness of fingers,
hands, and forearm
Small white lumps beneath the skin, sometimes oozing a
white substance that looks like toothpaste
Sores (ulcers) on the fingertips or toes
Tight and mask-like skin on the face

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.


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.


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.


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
Problems controlling stools (fecal incontinence)


Dx tests
Blood tests may include:
Antinuclear antibody (ANA) panel
Antibody testing
ESR (sed rate)
Rheumatoid factor
Other tests may include:
Chest x-ray
CT scan of the lungs
Echocardiogram
Urinalysis
Tests to see how well your lungs and gastrointestinal
(GI) tract are working
Skin biopsy Maria Carmela L. Domocmat, RN, MSN

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


Other treatments for specific symptoms may
include:
Medicines for heartburn or swallowing problems
Blood pressure medications (particularly ACE
inhibitors) for high blood pressure or kidney problems
Light therapy to relieve skin thickening
Medicines to improve breathing
Medications to treat Raynaud's phenomenon
Treatment usually also involves physical
therapy.


Medicines used to treat scleroderma include:
Power anti-inflammatory medicines called
corticosteroids
Immune-suppressing medications such as
methotrexate and Cytoxan
Nonsteroidal anti-inflammatory drugs (NSAIDs)


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


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.

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

http://www.nlm.nih.gov/medlineplus/ency/ar
ticle/000429.htm

Ankylosing Spondylitis
a form of arthritis that primarily affects the spine,
although other joints can become involved.
It causes inflammation of the spinal joints
(vertebrae) that can lead to severe, chronic pain
and discomfort.


In the most advanced cases, this inflammation can
lead to new bone formation on the spine, causing
the spine to fuse in a fixed, immobile position,
sometimes creating a forward-stooped posture
(Kyphosis)


Causes and risk factors
20 and 40, but may begin before age 10.
Risk factors include:
Family history of ankylosing spondylitis
Male gender


hallmark feature
the involvement of the sacroiliac (SI) joints during the
progression of the disease, which are the joints at the
base of the spine, where the spine joins the pelvis.


S/S
The disease starts with low back pain that
comes and goes.
Pain and stiffness are worse at night, in the
morning, or when you are not active. It may
wake you from your sleep.
The pain typically gets better with activity or
exercise.
Back pain may begin in the sacroiliac joints
(between the pelvis and the spine). Over time, it
may involve all or part of the spine.

S/S
Fatigue
less common symptoms include:
Eye inflammation or uveitis
Heel pain
Hip pain and stiffness
Joint pain and joint swelling in the shoulders, knees,
and ankles
Loss of appetite
Slight fever
Weight loss


Ankylosing Spondylitis


Complications
Rarely, people may have problems with the
aortic heart valve (aortic insufficiency) and heart
rhythm problems.
Some patients may have pulmonary fibrosis or
restrictive lung disease


Treatment
NSAIDs
Corticosteroid therapy
TNF-inhibitors
cytotoxic drugs
If do not respond well to corticosteroids or who are
dependent on high doses of corticosteroids.
Surgery


Nursing management
Provide education
Promote effective breathing
Exercises can help improve posture and
breathing.
Lying flat on the back at night can help maintain
normal posture.


Reactive arthritis
is a group of inflammatory conditions that
involves the joints, urethra, and eyes.


Triad of symptoms
Nongonococcal urethritis
Conjunctivitis
Arthritis


Causes, incidence, and risk
factors
men before the age of 40.
may follow an infection withChlamydia,
Campylobacter, Salmonella, or Yersinia.


Symptoms
Urinary symptoms usually appear within days or
weeks of an infection. Low-grade fever,
inflammation of the conjunctiva of the eye
(conjunctivitis), and arthritis develop over the
next several weeks. The arthritis may be mild or
severe, and may affect only one side of the body
or more than one joint.


Muscle and joint symptoms include:
Achilles tendon pain
Heel pain
Joint pain in the large joints (hip pain, knee pain,
and ankle pain are common)
Low back pain


Eye and skin symptoms include:
Eye discharge
Eye pain - burning
Eye redness
Skin lesions on the palms and soles that may
resemble psoriasis
Small, painless ulcers in the mouth, tongue, and
glans penis
Urinary and genital symptoms may include:


Urinary and genital symptoms may include:
Genital lesions (male)
Incontinence
Penis pain
Skin redness or inflammation
Urethral discharge
Urinary hesitancy
Urinary urgency
Urination - burning or stinging


Signs and tests
The diagnosis is based on symptoms.
Since the symptoms may occur at different
times, the diagnosis may be delayed.
A physical examination may reveal conjunctivitis
or typical skin lesions.
Tests that may be performed include:
HLA-B27 antigen
Joint x-rays
Urinalysis


Treatment
goal - to relieve symptoms and treat any
underlying infection.
Conjunctivitis and skin lesions associated with
the syndrome – self-limiintg
antibiotics if have an infection.
NSAIDS and pain relievers
Intraarticular corticosteroid
DMARDs


Management
Self-limited course - 3 to12 months
Administer meds
Same with AS
Antibiotic therapy – still controversial
Steroid eyedrops or subconjunctival prep
Provide PT
make adjustments if job requires heavy lifting or
strenuous use of the back.


Prevention
Preventing sexually transmitted diseases and
gastrointestinal infection may help prevent this
disease.
Wearing a condom during intercourse can
reduce the risks of sexually transmitted disease.
Wash hands and surface areas thoroughly
before and after preparing food.


Fibromyalgia (FM)

is a disorder of chronic widespread pain
with associated fatigue, poor sleep, stiffness, cognitive difficulties,
multiple somatic symptoms, and, not infrequently, anxiety and/or
depression.


Fibromyalgia (FM)

Pain - radiates diffusely from the axial skeleton
over large areas of the body,
predominantly involving muscles and musculoskeletal
junctions,
but also in joints (arthralgia without actual synovitis)
described as exhausting, burning, miserable, or unbearable.
may also be multifocal and can wax and wane in a migratory
fashion.
Described as "pain all over."
However, multifocal pain or recurrent episodes of regional pain are
essentially equivalent to the classic "pain all over" description.


Algometer or dolorimeter
A useful device
for rough
quantitation of
pain sensitivity
is a pressure
algometer, or
dolorimeter.


Tender points in fibromyalgia.


Fibromyalgia (FM)

Fatigue and poor sleep
Most patients with fibromyalgia also meet the classification criteria
for chronic fatigue syndrome.


Fibromyalgia (FM)

Cognitive problems
(known as "fibrofog") - primary symptom of
fibromyalgia,
reflecting impairments in working, episodic, and
semantic memory that are roughly equivalent to 20
years of aging.
Cognitive symptoms associated with
fibromyalgia are exacerbated by pain, mood
and anxiety disorders, and poor sleep.


Other common symptoms
Weight fluctuations
Allergic symptoms (eg, nasal congestion) and
hypersensitivity to environmental stimuli (eg, odors, bright
lights, loud noises) and medications
Regional pains, including noncardiac chest pain,
dyspepsia, headache, abdominal cramping (irritable bowel
syndrome), temporomandibular pain, chronic pelvic pain,
and others (Patients with fibromyalgia may meet criteria for
3 or more central sensitivity syndromes.)


Other common symptoms
Syncope or dizziness
Shortness of breath
Urinary frequency and urgency (female urethral syndrome,
interstitial cystitis)


Causes
multifactorial.
Engel's biopsychosocial model of chronic illness
(ie, health status and outcomes in chronic illness
are influenced by the interaction of biologic,
psychologic, and sociologic factors) provides a
useful way to conceptualize fibromyalgia


Laboratory Studies
do not have characteristic or consistent abnormalities as
determined by laboratory test results.
Laboratory studies - important to help rule out diseases
with similar manifestations


Laboratory Studies
Thyroid-stimulating hormone: H
ypothyroidism shares many clinical features with fibromyalgia,
especially diffuse muscle pain and fatigue.
Creatinine phosphokinase (CPK)
to exclude inflammatory myopathies
Erythrocyte sedimentation rate (ESR):
The normal ESR in patients with fibromyalgia contrasts with the
high ESR in elderly patients with polymyalgia rheumatica.
Obtaining an ESR can assist in identifying an underlying
inflammatory disorder or occult malignancy.


Laboratory Studies
Antinuclear antibodies (ANAs):
Many patients with SLE have comorbid fibromyalgia. A low-titer
ANA is common in the general population and may be of no clinical
significance if diagnostic features of SLE or related autoimmune
disorders are absent.
Rheumatoid factor:
Many patients with RA have comorbid fibromyalgia. However, a
positive result for rheumatoid factor does not support a diagnosis of
RA in the absence of objective evidence of characteristic joint
inflammation. A positive result for rheumatoid factor is
diagnostically nonspecific in other clinical settings.


Treatment
validation of the patient’s illness
empathetic listening and acknowledgment that the
patient is indeed experiencing pain
first crucial element in the treatment of pain, fatigue,
and other diverse symptomatology in patients with
fibromyalgia (FM) I


Treatment
Accurately assess possible causal or perpetuating
factors,
including attention to psychologic and sociocultural
factors
and identification of specific regional sources of ongoing
nociceptive pain (eg, degenerative spondylosis,
bursitis).


Comments such as "it’s all in your mind" or "I
cannot find anything wrong with you" only add to
the patient's frustration.


Psychologic and behavioral
approaches
Depression must be treated aggressively.
Depression, anxiety, stress, sleep disturbance, pain
beliefs and coping strategies, and self-efficacy all are
central to the pain experience in many patients and
frequently determine the outcome of chronic pain.
Unless psychosocial and behavioral variables are
recognized and approached, strictly
pharmacologic interventions are of limited benefit.


Psychologic and behavioral
approaches
Cognitive-behavioral therapy (CBT) and operant-
behavioral therapy (OBT)
both effect clinically meaningful improvements in pain
intensity and physical impairment in approximately one
third to on half of patients with fibromyalgia.


Patient Education
Education is an essential element in therapy for
fibromyalgia.
It begins with an empathetic manner on the part of
the nurse/physician, who must affirm the patient's
pain, explore social and behavioral variables (both
in childhood and current) that influence this
illness, and explain to the patient how stress and
distress can amplify pain and fatigue.


Medication
Anxiolytics/hypnotics
Antidepressants
Tricyclics antidepressants
Selective Serotonin-reuptake Inhibitors (SSRIs)
Serotonin Norepinephrine Reuptake Inhibitors (SNRIs)
Central Nervous System Depressants
Opioids
Anticonvulsants
Analgesics
Nonsteroidal anti-inflammatory drugs (NSAIDs)


often used in combination with antidepressants
and anticonvulsant drugs (both of which also have
efficacy for anxiety and insomnia)
Benzodiazepines
alprazolam [Xanax]
temazepam [Restoril
clonazepam [Klonopin]
buspirone [BuSpar]
trazodone [Desyrel])


Tricyclic antidepressant
Amitriptyline (Elavil)
Desipramine (Norpramin)
Doxepin (Sinequan)
Imipramine (Tofranil)
Trazodone (Desyrel)
Nortriptyline (Pamelor)


Selective Serotonin-reuptake
Inhibitors (SSRIs)
Fluoxetine (Prozac)
Sertraline (Zoloft)
Paroxetine (Paxil)
Fluvoxamine (Luvox)
Citalopram (Celexa)


Serotonin Norepinephrine
Reuptake Inhibitors (SNRIs)
Milnacipran (Savella)
Duloxetine (Cymbalta)


Central Nervous System
Depressants
Zolpidem (Ambien)
Zaleplon (Sonata)
Sodium oxybate (Xyrem)


Opioids
Morphine
Oxycodone (OxyContin)
Hydrocodone (Vicodin, Percocet)
Hydromorphone (Dilaudid)
Meperidine (Demerol)


Anticonvulsants
Gabapentin (Neurontin)
Pregabalin (Lyrica)


Pain Relievers
Acetaminophen (Tylenol)


Nonsteroidal anti-inflammatory
drugs (NSAIDs)
Aspirin
Ibuprofen (Advil)
Naproxen (Aleve)


Sodium oxybate (Xyrem)
a sedative hypnotic, prolongs stage III/IV
restorative sleep, which is essential to awaken
rested and refreshed.


Anticonvulsants
Pregabalin (Lyrica)
Gabapentin (Neurontin)
Clonidine (Catapres)


Polymyositis
a persistent inflammatory muscle disease that
causes weakness of the skeletal muscles, which
control movement.
Medically, polymyositis is classified as a chronic
inflammatory myopathy — one of only three such
diseases.


Polymyositis
can occur at any age,
adults -30s, 40s or 50s.
Blacks
Women


Polymyositis
signs and symptoms usually develop gradually,
over weeks or months.
Remissions - rare

Remissions: periods during which symptoms spontaneously
disappear

Signs and symptoms
appear gradually,
Progressive muscle weakness
Difficulty swallowing (dysphagia)
Difficulty speaking
Mild joint or muscle tenderness
Fatigue
Shortness of breath


Signs and symptoms
affects the muscles closest to the trunk,
particularly hips, thighs, shoulders, upper arms
and neck.
weakness is symmetrical
worsens over time.
As muscle weakness progresses,
difficult to climb stairs, rise from a seated position, lift
objects or reach overhead.


Complications
Dysphagia
Which in turn may cause weight loss and malnutrition.
Aspiration pneumonia
Shortness of breath or respiratory failure.
Calcinosis
Calcium deposits in muscles, skin and connective
tissues


Associated conditions
polymyositis is often associated with other
conditions that may cause further complications
of their own, or in combination with polymyositis
symptoms. Associated conditions include:
Raynaud's phenomenon.
Other connective tissue diseases.
Cardiovascular disease.
Lung disease.


Treatment
corticosteroid
When muscle strength improves, usually in 4 to 6
weeks, the medication is slowly tapered off.
Maintenance therapy with prednisone may be
continued indefinitely.
DMARDs - If unresponsive to corticosteroids
methotrexate and azathioprine,


Treatment
Intravenous gamma globulin
IVIG
is a purified blood product that contains healthy
antibodies from thousands of blood donors.
The healthy antibodies in IVIG can block the
damaging antibodies that attack muscle in
polymyositis.
Repeat infusions q 6-8 weeks


Other immunosuppressive
medicine
Tacrolimus (Prograf)
is a transplant-rejection drug that may work to inhibit
the immune system.
Immunosuppressants,
cyclophosphamide (Cytoxan) and cyclosporine
(Gengraf, Neoral, Sandimmune),


Biological therapies
Rituximab (Rituxan)
Tumor necrosis factor (TNF) inhibitors
etanercept (Enbrel) and infliximab (Remicade),


Other treatment approaches
Physical therapy
Dietetic assessment
Speech therapy


Nursing management
Coping and support
Educate about the illness
Balance Rest and exercise


Sources
http://www.mayoclinic.com/health/polymyositis/DS00334/METHOD=
print&DSECTION=all
http://www.mayoclinic.com/health/polymyositis/DS00334


Dermatomyositis
a muscle disease characterized by inflammation
and a skin rash. It is a type of inflammatory
myopathy.
5 - 15 and adults age 40 - 60.
Women
Polymyositis is a similar condition, but the
symptoms occur without a skin rash.


Symptoms
Dysphagia
Muscle weakness, stiffness, or soreness
Purple or violet colored upper eyelids
Purple-red skin (violaceous) rash
SOB


Symptoms
The muscle weakness may appear suddenly or develop
slowly over weeks or months. may have difficulty raising
arms over head, rising from a sitting position, and
climbing stairs.
The rash may appear over the face, knuckles, neck,
shoulders, upper chest, and back.


reddish-purple (violaceous) rash
reddish-purple
(violaceous) rash.
The rash is named after
the tendency of plants to
grow toward the sun
(heliotropic) and is
characteristic of
dermatomyositis.


purple (violaceous) plaques
The appearance of purple
(violaceous) plaques on
the knees may be
associated with
dermatomyositis.


Gottron's sign
Red, thickened, scaly skin over the knuckles


Heliotrope eyelids
eyelids develop a brown
(violaceous - rather than
red) color.
Heliotrope eyelids and
Gottron's papules on the
knuckles are
characteristic findings in
dermatomyositis.


violet-colored inflammation (erythema) over the
knuckles


periungual erythema
Candida paronychia produced periungual
erythema, edema and nail fold maceration.


Dx Exams
CPK & aldolase
ECG
Electromyography
Magnetic resonance imaging (MRI)
Muscle biopsy


Treatment
Corticosteroids
Immunosuppressants
When muscle strength gets better – taper off
corticos
However, most people take prednisone
indefinitely.
If the condition is associated with a tumor, the
muscle weakness and rash may improve when
the tumor is removed.


Outlook (Prognosis)
Some recover and have symptoms completely
disappear - especially in children.
In adults, death may result from severe and
prolonged muscle weakness,
malnutrition, pneumonia, or lung failure. The
major causes of death are cancer (malignancy)
and lung disease.


Acute renal failure
Cancer (malignancy)
Inflammation of the heart
Joint pain
Lung disease


http://www.nlm.nih.gov/medlineplus/ency/article/
000839.htm
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH
0001842/


Bursitis
A painful inflammation of the bursae


Bursitis
bursae
closed, minimally fluid-filled sacs that are
lined with a synovium similar to the lining of
joint spaces
function: to reduce friction between adjacent
tissues (tendon and bones or tendon and
ligaments) by lubricating these enclosed
structures with synovial fluid from bursal sac


Bursitis
Bursae
there are 150 bursae in human body
cover bony prominences (e.g., olecranon, trochanter,
and patella)
or provide protection between the skin and other
structures (e.g., calcaneal bursa)
usually thin, but with repeated stress – can become
thickened and fluid-filled secondary to inflammation


Bursitis
peaks – 40 to 50 yrs
affected areas – shoulder joints (most
common), elbow, knee, hip; dominant arm


Etiology and risk factors
acute or chronic trauma (mechanical, highly
repetitive activities)
arthritic conditions (e.g., RA), gout, tumors,
degenerative changes
occupational or avocational activities (e.g., wood
carver – acute subacromial bursitis;
businesswoman walking long distance on high
heels – retrocalcaneal bursitis)


Clinical manifestations
exquisite localized pain in target area
point tenderness (can specifically point the spot
of greatest discomfort)
diffuse soreness radiating to the tendons at the
site
interrupted sleep (e.g., with subacromial bursitis,
calcaneal bursitis)


difficulty walking (e.g., trochanteric bursitis,
calcaneal bursitis)
difficulty performing ADL (e.g., with subacromial
or olecranon bursitis)


Dx
diagnosis is based on PE and history
radiographs – usually normal in acute bursitis;
calcium deposits in chronic
lab tests and synovial fluid analysis – normal
unless bursa become infected


Management
Goals
Rests and immobilization of affected joint
Non-opoiod analgesics
ROM exercises
NSAIDs


Nursing Management
Client education
Focus on causes and prevention of additional attacks
by avoiding activities that cause constraint friction or
pressure
correct application of moist heat
medication
exercise instruction
intra-articular injections of cortisone


Nursing Diagnoses
Acute or Chronic Pain
Impaired Physical Immobility
Temporary Self-Care Deficits


Interventions
Goal: pain reduction (without pain reduction –
joint mobility is impaired thru guarding,
protective measures)
Teach purpose, dose and side effects of anti-
inflammatory meds
Resting or immobilizing joint or elevating or
compressing involved area to control edema
Teach about correct application of ice ad heat


Teach postinjection flare of intra-articular
cortisone
Self-care: oversized garment, especially those
with long sleeves or wide pant legs
Minimize shoulder or elbow pain – by putting
clothing on affected arm first and by taking it off
the affected arm last.


Vasculitis
a group of disorders leading to inflammation and
necrosis of blood vessel walls
includes:
polyarteritis nodosa
systemic necrotizing vasculitis
allergic granulomatous angitis


Vasculitis
Pathophysiology
soluble immune complexes are deposited in blood
vessel walls in areas where capillaries have
increased permeability
after deposition, the immune system is activated and
the complex is destroyed along with the blood vessel
wall
inflammation and damage to large and small vessels
result in end-stage organ damage


vary
depending on organs affected


Management
Steroids


Polymyalgia rheumatica
a clinical syndrome
more common women
disease of aging, rarely occur before age 60
years


pain and stiffness in neck, shoulder, back, and
pelvic girdle esp in the morning
headaches or painful areas on head
low grade fever
temporal arteritis


Dx
Elevated ESR
mild anemia
elevated Ig


Management
steroids


Giant cell arteritis
AKA temporal or cranial arteritis
disease of aging


Giant cell arteritis
a clinical syndrome
more common women
disease of aging, rarely occur before age 60
years


polymyalgia rheumatic for months, then
suddenly experiences severe headaches assoc
with temporal arteritis
sudden onset with severe pain often appearing
in temporal area (can also be in occipital area,
face, or side of neck


hyperesthesia (unusual or pathological
sensitivity of the skin or of a particular sense of
stimulation) – makes any touch exquisitely
painful
visual changes – blindness in one or both eyes


Management
Corticosteroids


Mixed connective tissue disease
a combination of several connective tissue
diseases
frequent combinations are SLE and SSc and RA


Mixed connective tissue disease
clinical manifestations
have manifestations that are not typical of any one
disorder

management
according to manifestations


Complex multisystem disease
One of form of rheumatic joint disease with a
known cause
Included as a connective tissue disorder bcoz
the skin, joint, nervous system, and heart are
involved


Etiology and risk factors
cause: spirochete Borrelia burgdorferi

Male tick Female tick


Risk factors
Doing activities that increase tick exposure (for
example, gardening, hunting, or hiking)
Having a pet that may carry ticks home
Walking in high grasses

Tick imbedded in the skin
This is a close-up photograph of a
tick embedded in the skin. Ticks are
important because they can carry
diseases such as Rocky Mountain
spotted fever, tularemia, Colorado
tick fever, Lyme disease, and others.

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001690/bin/2060.jpg


Large “bull’s –eye” circular rash; red flat rash
that clears in the center
severe headache
severe malaise
stiff neck
fever chills
myalgias
joint pain
fatigue

3 stages of Lyme disease
Stage 1 : primary Lyme disease.
Stage 2 : secondary Lyme disease and early
disseminated Lyme disease.
Stage 3 : tertiary Lyme disease and chronic
persistent Lyme disease


Stage 1 :
primary Lyme disease.
Occurs 3 to 32 days after the bite
Flu-like symptoms , bull’s eye rash, pain and stiffness
in muscles and joints
For some – arthritis is the first and only sign of the
disease


Stage 2 :
secondary Lyme disease and early disseminated
Lyme disease.
Occurs 2-12 wks after bite
Carditis with dysrhtmia, dyspnea, dizziness,
palpitations
CNS disorders – meningitis, facial paralysis,
peripheral neuritis


Stage 3
tertiary Lyme disease and chronic persistent Lyme
disease
develop months or years after first develop Lyme
disease infection
Occurs when disease is not diagnosed and treated in
earlier stages
s/s - arthralgias, fatigue, memory/thinking problems


Management
Stage 1
Antibiotic therapy – PO, for 10-21 days
Doxycycline
Amoxicillin
Cefuroxime
Stage 2
IV Antibiotic therapy
ceftriaxone, cefotaxime


Management
Intra-articular steroids and NSAIDs
To reduce inflammation and pain


Client Education guide
Avoid heavily wooded areas or areas with thick
underbrush
Walk in the center of the trail
Avoid dark clothing. Lighter-colored clothing
makes spotting ticks easier
Use an insect repellent on your skin and clothes
when in an area where ticks are likely to be
found
Wear long-sleeved tops and long pants


Wear closed shoes and a hat or cap
Bathe immediately after being in an infested
area, and inspect your body for ticks (abt the
size of a pinhead); pay special attention to arms,
legs and hairline
Gently remove with tweezers, or finger any tick
that you find. Dispose of the tick by flushing it
down the toilet (burning could spread infection)


Wait 4-6 weeks after being bitten by a tick b4
being tested for Lyme disease (testing b4 this
time is not reliable)
Report symptoms, such as rash or influenza-like
illness, to the physician
Obtain a vaccine to prevent disease if you live
in a high-risk area


Complications
long-term joint inflammation (Lyme arthritis)
Arrhythmia
Brain and nervous system (neurological)
problems


Sarcoidosis is a disease in which swelling
(inflammation) occurs in the lymph nodes, lungs,
liver, eyes, skin, or other tissues.


Causes
The cause of the disease is unknown. In
sarcoidosis, clumps of abnnormal tissue
(granulomas) form in certain organs of the body.
Granulomas are clusters of immune cells.
The disease can affect almost any organ of the
body, but it most commonly affects the lungs.


Possible causes of sarcoidosis include:
Excess sensitivity to environmental factors
Genetics
Extreme immune response to infection


The condition is more common in African
Americans than Caucasians. Females are
usually affected more often than males. The
disease typically begins between the ages of 20
and 40. Sarcoidosis is very rare in young
children.


Symptoms
There may be no symptoms. When symptoms
occur, they can involve almost any part or organ
system in your body.
Almost all patients have lung or chest
symptoms:
Dry cough
Shortness of breath
Discomfort behind your breast bone
Abnormal breath sounds (such as rales)


Symptoms of general discomfort or uneasiness
often occur:
malaise
Fatigue (one of the most common symptoms in
children)
Fever
Weight loss (one of the most common symptoms
in children)
Joint achiness or pain (arthralgia)


Skin symptoms:
Skin rashes
Old scars become more raised
Raised, red, firm skin sores (erythema nodosum,
almost always on the front part of the lower legs
Skin lesions
Hair loss


Nervous system (neurological) and vision
changes:
Headache
Seizures
Weakness or paralysis (palsy) on one side of the
face
Eye burning, itching, and discharge
Symptoms of uveitis
Decreased tearing


Other symptoms of this disease:
Enlarged lymph glands - armpit lump
Enlarged liver
Enlarged spleen
Dry mouth
Nosebleed

http://www.nlm.nih.gov/medlineplus/ency/ar
ticle/000076.htm

Exams and Tests
Often the disease is found in patients with no
symptoms who have an abnormal chest x-ray.
Different imaging tests may help diagnose
sarcoidosis:
Chest x-ray to see if the lungs are involved or
lymph nodes are enlarged
CT scan
Lung gallium (Ga.) scan


Biopsies of different tissues may be done:
Lymph node biopsy
Skin lesion biopsy
Bronchoscopy to perform a biopsy
Open lung biopsy
Mediastinoscopy with biopsy
Liver biopsy
Kidney biopsy
Nerve biopsy
Heart biopsy


This disease may also alter the results of the following
lab tests:
CBC
Chem-7 or chem-20
Quantitative immunoglobulins (nephelometry
PTH
Serum phosphorus
Immunoelectrophoresis - serum
Calcium - urine
Calcium - ionized
Calcium - serum
Liver function tests Carmela L. Domocmat, RN, MSN
Maria

Treatment
Sarcoidosis symptoms often get better on their own gradually without
treatment.
Severely affected patients may need treatment with corticosteroids
(prednisone or methylprednisolone). This includes people who have
involvement of the eyes, heart, nervous system, and some with lung
involvement. Therapy may continue for 1 or 2 years. Some of the most
severely affected patients may require life-long therapy.
Drugs that suppress the immune system (immunosuppressive medicines),
such as methotrexate, azathioprine, and cyclophosphamide, are sometimes
used in addition to corticosteroids. Rarely, some people with irreversible
organ failure require an organ transplant.
Although these treatments may temporarily improve the symptoms of the
disease, long-term treatment has not been proven to prevent sarcoidosis
from slowly getting worse.


Outlook (Prognosis)
Many people are not seriously ill, and the
disease may get better without treatment. About
30 - 50% of cases get better without treatment in
3 years. About 20% of those whose lungs are
involved will develop lung damage.
The overall death rate from sarcoidosis is less
than 5%. Causes of death include:
Scarring of lung tissue (pulmonary fibrosis)
Bleeding from the lung tissue
Involvement of the heart (rarely)

Osteoporosis and other complications of taking
corticosteroids for longer periods of time.
Diffuse interstitial pulmonary fibrosis
Pulmonary hypertension
Fungal lung infections (aspergilloma
Anterior uveitis
Glaucoma and blindness (rare)
Cardiac arrhythmias
Cranial or peripheral nerve palsies
High calcium levels (hypercalcemia
Kidney stones
Organ failure, leading to the needRN, MSN transplant
Maria Carmela L. Domocmat, for a

THANK YOU!!!


Rheumatic Disorders: SLE, PA, SSc, Fibromyalgia, Lyme Disease

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