Rheumatic Disorders Part II

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


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 MHC proteins

What causes autoimmune disease?


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)

Butterfly rash of lupus

Damaged kidney (left) caused by

immunoglobulin deposits (right)


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


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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Rheumatic Disorders Part II