Inflammatory or Autoimmune

and Rheumatic Disorders

INFLAMMATORY DISORDERS PATHOPHYSIOLOGY (RHEUMATIC DISEASE)
→ AKA Autoimmune Disease.
→ The immune system causes inflammation 3 DISTINCT CHARACTERISTICS:
by mistakenly attacking your body's own 1. INFLAMMATION
cells or tissues. 2. AUTOIMMUNITY
→ There are several ways that our immune 3. DEGENERATION
system can go wrong and cause
inflammation.
INFLAMMATION
AUTOIMMUNE DISORDERS
→ Happens when the body's natural defense 1. Stimuli: Antigen
system can't tell the difference between 2. Activation of Immune System
your own cells and foreign cells, causing 3. Antigen-Antibody Complexes
the body to mistakenly attack normal cells. 4. Phagocytosis -> Inflammatory Reaction
5. Leukotrienes & Prostaglandins
ETIOLOGY: Unknown, exact etiology. 6. Enzymes (Collagenase)
7. Collagen breakdown
In most cases, a combination of factors is 8. Pannus formation
probably at work, such as: 9. Bone Erosion
• Genes, which may make you more likely to Pannus formation – proliferation of newly
develop the disease. formed synovial tissue infiltrated with
• Environment, such as a virus that triggers inflammatory cells.
the disease if you have the gene(s).

RHEUMATIC DISORDERS
→ An umbrella that refers to arthritis and
several other conditions that affect the
joints, tendons, muscle, ligaments, bones,
and muscles.
→ Most common clinical feature: arthritis
(inflammation of a joint) & pain.
→ Female (more likely) than men.

ONSET: acute or insidious

OCCURING PERIODS:
➢ Remission a period when disease
symptoms are reduced or absent.
➢ Exacerbation a period when symptoms
occur or increase.

RHEUMATIC DISEASES

CLASSIFICATION:
• MONOARTICULAR – affecting a single
joint.
• MONOARTICULAR – affecting multiple
joints.
OR
• INFLAMMATORY
• NONINFLAMMATORY (osteoarthritis)
 AUTOIMMUNITY
• Hallmark of inflammatory rheumatic
diseases.
• Body mistakenly recognizes its own
tissue as a foreign antigen.

Human Leukocyte Antigen (HLA) Genes

▪ Large group of genes.
▪ Has been linked to the immune response
and the development of multiple
rheumatic diseases.

DEGENERATION
• Degenerative rheumatic diseases,
inflammation also occurs, but as a
secondary process.
• More accurately called degradation.

Theory
Genetic or hormonal influences, mechanical
factors, and prior joint damage ->
degradation of cartilage -> increased
mechanical stress on bone ends -> stiffening
of bone tissue.

CLINICAL MANIFESTATIONS
Most common symptom: pain
• Joint swelling
• Limited movement
• Stiffness
• Weakness
• Fatigue

ASSESSMENT & DIAGNOSTIC FINDINGS

➢ Complete health history

➢ Complete physical assessment

INSPECTION IMAGING STUDIES

▪ Gait, posture, and general musculoskeletal
size and structure.
▪ Gross deformities and abnormalities in
movement.
▪ Symmetry, size, and contour of other
connective tissues (skin & adipose tissue).
• X-ray studies
• Computed tomography (CT) scans
• Magnetic resonance imaging (MRI) scans
• Arthrography

FUNCTIONAL ASSESSMENT
▪ Combination of history (what the patient
reports that they can and cannot do).
▪ Examination (observation of activities, in
which the patient demonstrates what they
can and cannot do, such as dressing and
getting in and out of a chair).
 MEDICAL MANAGEMENT ▪ Sulindac

1. Pharmacologic Therapy ACTION/USES:

2. Nonpharmacologic Pain Management Anti-inflammatory, analgesic, antipyretic,
3. Exercise and Activity platelet aggregation inhibitor.
4. Sleep
NURSING CONSIDERATIONS:
PHARMACOLOGIC THERAPY ▪ Administer NSAIDs with food.
▪ Monitor for GI, CNS, cardiovascular, renal,
INDICATIONS: hematologic, and dermatologic adverse
• Manage symptoms effects.
• Control inflammation ▪ Avoid salicylates; use acetaminophen for
• To modify the disease additional analgesia.

SALICYLATES COX-2 ENZYME BLOCCKERS

Example: Celecoxib
Acetylated:
▪ Aspirin ACTION/USES:
Nonacetylated: Inhibit only COX-2 enzymes, which are
▪ Choline Trilisalicylate produced during inflammation, and spare
▪ Salsalate COX-1 enzymes, which can be protective to
▪ Sodium Salicylate the stomach.

ACTION/USES: NURSING CONSIDERATIONS:

Anti-inflammatory, analgesic, antipyretic. ▪ Watch for possible confusion in older
Acetylated salicylates are platelet aggregation adults.
inhibitors. ▪ Appropriate for older adults and
patients who are at high risk for gastric
NURSING CONSIDERATIONS: ulcers.
▪ Administer with food, milk, antacids or
large glass of water to reduce GI effects.
▪ Assess for tinnitus, gastric intolerance, GI
bleeding, and purpura.

NONSTEROIDAL ANTI-INFLAMMATORY
DRUGS (NSAIDs)

Example:
▪ Diclofenac
▪ Diflunisal
▪ Etodolac DISEASE-MODIFYING ANTIRHEUMATIC
▪ Ibuprofen DRUG (DMARDs)
▪ Ketoprofen
▪ Meloxicam ANTIMALARIALS:
▪ Nabumetone ▪ Hydroxychloroquine
▪ Naproxen ▪ Chloroquine
▪ Piroxicam
ACTION/USES: IMMUNOSUPPRESSIVES
Anti-inflammatory and inhibits lysosomal ▪ Methotrexate (1st line agent)
enzymes. ▪ Azathioprine
▪ Cyclophosphamide
NURSING CONSIDERATIONS: ▪ Cyclosporine
▪ May be administered concurrently with
NSAIDs. ACTION/USES:
▪ Assess for visual changes, GI upset, skin Nonbiologic immune suppression by
rash, headaches, photosensitivity, inhibiting T lymphocytes.
bleaching of hair.
▪ Emphasize need for ophthalmologic NURSING CONSIDERATIONS:
examinations (every 6–12 months). ▪ Assess for bone marrow suppression,
GI ulcerations, alopecia, increased
infections.
▪ Monitor CBC, liver enzymes, creatinine.

IMMUNOMODULATORS

TNF-BLOCKING AGENTS
▪ Adalimumab
▪ Etanercept
▪ Infliximab
▪ Golimumab

ACTION/USES:
Biologic response modifier that binds to TNF,
a cytokine involved in inflammatory and
immune responses.

NURSING CONSIDERATIONS:
JANUS KINASE (JAK) INHIBITORS ▪ Patient should be tested for
tuberculosis before beginning this
TOFACITINIBIC, BARICITINIC medication.

ACTION/USES: T-CELL COSTIMULATION MODULATOR

Enters the cell and binds to the active JAK site ▪ Abatacept
-> inhibits autophosphorylation and JAK
activation that inhibits cytokine production. ACTION/USES:
Blocks one of the pathways needed to fully
NURSING CONSIDERATIONS: activate T cells, decreasing inflammatory and
▪ Test for latent TB before initiation of immunologic responses.
therapy.
▪ Monitor liver enzymes routinely. NURSING CONSIDERATIONS:
▪ Educate patient about increased risk of
SULFASALAZINE infection.

ACTION/USES: B-CELL PRODUCTION MODULATOR

Anti-inflammatory, reduces lymphocyte ▪ Rituximab
response, inhibits angiogenesis.
ACTION/USES:
NURSING CONSIDERATIONS: Binds to B-lymphocyte CD20 surface
▪ Do not use in patients with allergy to antigens.
sulfa medications or salicylates.
▪ Emphasize adequate fluid intake. NURSING CONSIDERATIONS:
▪ Assess for GI upset, skin rash, ▪ Educate patient about increased risk of
headache, liver abnormalities, anemia. infection.
 HUMAN IL-1 RECEPTOR ANTAGONIST PHARMACOLOGIC THERAPY (SUMMARY)
▪ Anakinra
1. SALICYLATES
ACTION/USES: 2. NSAIDs
Blocks IL-1 receptors, decreasing ▪ COX-2 enzyme blockers (lesser
inflammatory and immunologic responses. effect on stomach)
3. DMARDs
NURSING CONSIDERATIONS: ▪ ANTIMALARIALS
▪ Educate patient about increased risk of ▪ JAK INHIBITORS
infection, given SQ/SC. ➢ Tofacitinib
➢ Sulfasalazine
➢ Immunosuppressives
HUMAN IL-6 RECEPTOR ANTAGONIST ▪ IMMUNOMODULATORS
▪ Tocilizumab ➢ Pyrimidine Synthesis
Inhibitors
ACTION/USES: ➢ TNF-Blocking agents
Binds to and inhibits IL-6 receptors, ➢ T-Cell costimulation
decreasing inflammatory and immunologic modulators
responses. ➢ B-Cell production blocker
➢ Human IL-1 Receptor
NURSING CONSIDERATIONS: ➢ Human IL-6 Receptor
▪ Educate patient about increased risk of Antagonist
infection. 4. CORTICOSTEROIDS

CORTICOSTEROIDS CLASSIFICATION

Prednisone, Prednisolone, Hydrocortisone A. SYNTHETIC DMARDs

ACTION/USES: B. BIOLOGICAL DMARDs

Anti-inflammatory, fast acting; onset in days,
intra-articular injections useful for joints
unresponsive to NSAIDs.

NURSING CONSIDERATIONS:
▪ Assess for toxicity: Cataracts, GI irritation,
hyperglycemia, hypertension, fractures,
avascular necrosis, hirsutism, psychosis.
▪ Joints most amenable to injections (ankles,
knees, hips, shoulders, and hands).
▪ Repeated injections can cause joint
damage.
DRUGS USED IN RHEUMATOID ARTHRITIS

CORTICOSTERIODS
▪ Used as bridge therapy to reduce disease
activity until slower acting DMARDs take
effect.
▪ Can be used as an adjunctive therapy for
active disease that persists despite
treatment with DMARDs.

SYNTHETIC DMARDs
▪ Methotrexate
▪ Sulfasalazine
▪ Leflunomide
▪ Hydroxychloroquine
 ▪ Minocycline → Short-term use of low-dose antidepressant
▪ Tofacitinib medications.
▪ Gold salts
▪ Penicillamine SLEEP HYGIENE STRATEGIES
• Establishing a set time to sleep and a
BIOLOGICAL DMARDs regular wake-up time.
▪ TNF-a inhibitors • Creating a quiet sleep environment with
- Etanercept a comfortable room temperature.
- Infliximab
• Avoiding factors that interfere with sleep
- Adalimumab (e.g., the use of alcohol and caffeine).
- Golimumab
• Using relaxation exercises and getting out
- Certolizumab
of bed and engaging in another activity
▪ Co-stimulation inhibitors
(e.g., reading) if unable to sleep.
- Abatacept
- Belatacept
▪ IL-6 inhibitors
- Tocilizumab DIFFUSE CONNECTIVE TISSUE DISEASES
▪ B-cell depleter → Refers to a group of systemic disorders.
- Rituximab → Chronic in nature.
→ Characterized by diffuse inflammation and
NONPHARMACOLOGIC PAIN MANAGEMENT degeneration in the connective tissues.
→ Have unknown causes -> r/t immunologic
HEAT APPLICATIONS abnormalities.
→ Helpful in relieving pain, stiffness, and
muscle spasm (maximum benefit: 20 mins). A. RHEUMATOID ARTHRITIS
→ Warm tub baths or showers and warm B. SYSTEMIC LUPUS ERYTHEMATOSUS
moist compresses. C. SCELODERMA
→ Paraffin baths (dips) -> offers concentrated
heat. RHEUMATIC ARTHRITIS

DEVICES Rheumatoid Arthritis

→ Eases the pain by limiting the affected → An autoimmune disease of unknown origin.
joint/area. → Commonly occurs between 30s to 60s.
→ Braces, splints, and assistive devices for
ambulation (e.g., canes, crutches, walkers).
→ Cervical collars, metatarsal bar or special
pads.

EXERCISE AND ACTIVITY

Purposes: Maintain flexibility and joint motion,
enhance synovial blood flow, promote strength
of bone and cartilage and improve muscle tone &
cardiovascular status.

➢ Range of motion
➢ Isometric exercise
➢ Dynamic exercise
➢ Aerobic exercise PATHOPHYSIOLOGY
➢ Pool exercise
Exact etiology: unknown
SLEEP Proposed etiology: genetic predisposition,
development of immunologically mediated joint
AMITRIPTYLINE inflammation.
→ May be used to reestablish adequate sleep
patterns and improve pain management.
 An autoimmune reaction occurs in the
synovial tissue -> synovium breaks down
collagen -> causing edema -> proliferation of
the synovial membrane -> pannus formation
-> pannus destroys cartilage and erodes the
bone.

Main consequence: loss of articular surfaces

and joint motion, muscle fibers undergo
degenerative changes, tendon and ligament
elasticity and contractile power are lost.

PERIPHERAL NERVOUS SYSTEM

INVOLVEMENT:

Synovial inflammation can compress the

adjacent nerve -> causing neuropathies and
paresthesias.

PATTERN OF JOINT INVOLVEMENT

Begins at small joints of the hands, wrists, and
feet -> knees, shoulders, hips elbows, ankles,
cervical spine, and temporomandibular joints.

ONSET: usually, acute

CHARACTERISTIC: symptoms are usually
bilateral and symmetric.
PAIN: painful to move -> immobilization ->
contractures -> soft tissue deformity

Deformities
→ Caused by misalignment resulting from
swelling, progressive joint destruction, or
the subluxation (partial dislocation) that
occurs when one bone slips over another
and eliminates the joint space.
CLINICAL MANIFESTATIONS
MOST COMMON: Deformities of the hands
INITIAL SYMPTOMS (ulnar deviation and swan neck deformity) and
• Symmetric joint pain feet.
• Morning joint stiffness (>1hour)
SWAN NECK DEFORMITY (HANDS & FEET)
CLASSIC SYMPTOMS
• Symmetric joint pain
• Swelling
• Warmth, erythema
• Lack of function

Upon PALPATION of JOINTS

• Reveals spongy or boggy tissue -> fluid
can be aspirated from the inflamed
joint.
 SYSTEMIC SYMPTOMS
• Fever, weight loss, fatigue, anemia, lymph
node enlargement.
• Reynaud’s phenomenon (cold and stress
induced vasospasm causing episodes of
digital blanching or cyanosis).
• Rheumatoid nodules -> more common in
advanced RA.
➢ Nontender and movable in the
subcutaneous tissue.
➢ Appear over bony prominences.

EXTRA-ARTICULAR FEATURES:
• Arteritis, neuropathy, pericarditis,
ULNAR DEVIATION splenomegaly, and Sjögren’s syndrome
(dry eyes and dry mucous membranes).

OTHER MANIFESTATIONS

CLINICAL MANIFESTATIONS
ASSESS FOR
• Complete health history
• Physical examination (focus on
manifestations)
• Extra-articular changes and systemic
manifestations.
• Assess for TB, hepatitis B and hepatitis C.
PALPATION
• Rheumatoid nodules, joint inflammation
A physical therapy).
DIAGNOSTIC FINDINGS
• (+) Rheumatoid factor – not accurate.
• Antibodies to cyclic citrullinated peptide
(anti-CCP) – more accurate.
• Elevated ESR & CRP
• CBC (anemia, platelets may be elevated
due to the inflammatory process)
• Tuberculin (TB) skin test
o Should be done prior to the initiation
of certain medications.
• Plain X-ray (most common to monitor
disease progression, inexpensive).
• MRI (detect small erosions, more detailed
than X-ray).
MEDICAL MANAGEMENT
GOAL OF TREATMENT (all phases)
• Decrease joint pain and swelling
• Achieve clinical remission
• Decrease the likelihood of joint deformity
• Minimize disability
A. EARLY RHEUMATOID ARTHRITIS
• Begins with: nonbiologic or biologic
DMARDs.
• Goal: preventing inflammation and joint
damage.
Nonbiologic DMARDs
▪ Methotrexate, preferred agent.
▪ WOF: liver and kidney function, CBC
(anemia).
Biologic DMARDs
▪ First targeted therapy for RA.
▪ Works more quickly, more expensive.
▪ Moderate to severe RA, unresponsive to
non-biologics.
Corticosteroids
▪ Recommended as a “bridge” in the early
treatment.
▪ Are not recommended for long-term
therapy due to side effects.
Janus Kinase (JAK) inhibitors
▪ Used in combination with nonbiologic
DMARDs or monotherapy.
NSAIDs
▪ Pain and inflammation.
B. ESTABLISHED RHEUMATOID ARTHRITIS
• Formal program (an occupational and
• Combination therapy (one non-biologic
DMARD and one biologic DMARD).
• Systemic Corticosteroids (bridge tx).
• Topical analgesic agents (capsaicin,
methylsalycylate).
• Reconstructive Surgery
Reconstructive Surgery
− Indicated when pain cannot be relieved
by conservative measures and the threat
of loss of independence is eminent.
NOT PERFORMED during EXACERBETIONS.
 Synovectomy
− Is the removal of the synovium from a
joint in the body.

ARTHRODESIS

A. The left ankle is exposed through a

lateral incision and the subtalar joint is
decorticated.

NURSING MANAGEMENT

NURSING CONCERNS FOR PATIENT WITH RA:

• Pain
• Sleep disturbance
• Fatigue
• Altered mood
• Limited mobility

NURSING RESPONSIBILITIES:
• Monitoring and managing potential
complications.
B. Multiple guide pins are placed across the • Promoting home, community-based, and
subtalar joint. 3 screws are placed for transitional care.
further fixation. • Nutrition therapy (should include grains,
fruits, vegetables, dairy and protein)

SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

Systemic Lupus Erythematosus

→ An inflammatory, autoimmune disorder
that affects nearly every organ of the body.
→ Overall estimated incidence is 1.8 to 7.6 per
100,000 persons (CDC).
→ 4 – 12 times more common in women than
men.
→ It occurs more in African American, Asians,
C. Bone graft is packed over the
Hispanics than in Caucasians.
decorticated bone to aid in the fusion
process.
PATHOPHYSIOLOGY
• The immune system inaccurately
recognized one or more components of
cell's nucleus as foreign, seeing it as an
antigen.
• Immune system starts to develop
antibodies to the nuclear antigen.
• The B cells begin to overproduce
antibodies, but instead of producing
protective antibodies, they produce
“autoantibodies,” antinuclear antibodies
 (ANA) which attack the patient's own • Lesions often worsen during exacerbation
tissue. of the systemic disease and provoked by
• Antibodies and antigens form antigen– sunlight.
antibody complexes -> trapped in the • Others: oral ulcers, alopecia
capillaries of visceral structures -> destroy
host cells. BUTTERFLY RASH (MALAR RASH)
Pathognomonic or hallmark sign of SLE
Hypothesized Risk Factors:
 Gender: Women
▪ Female sex hormones (estrogen) play
a role in the predisposition to SLE.
▪ Estrogen contributes to the body’s
response of overreacting to the body’s
own tissues.

 Exogenous or Environmental Triggers

▪ Cigarette smoke
▪ Ultraviolet rays, exposure from
sunlight and fluorescent light bulbs.
▪ Medications such as hydralazine,
minocycline, or procainamide.
▪ Viral infections
▪ Emotional stress
▪ Stress on the body (e.g., surgery,
pregnancy)
▪ Silica dust exposure in the
occupational setting.

CLINICAL MANIFESTATIONS
SLE
→ Is autoimmune, systemic disease that can
affect any body system.
→ Commonly involved are:
• Mucocutaneous system SUBACUTE CUTANEOUS
• Musculoskeletal system LUPUS ERYTHEMATOSUS
• Renal system
• Nervous system
• Cardiovascular system
• Respiratory system

DISEASE PROCESSES:

Chronic state – symptoms are minimal/absent.

Acute state – symptoms and lab results are
elevated.
CLINICAL MANIFESTATIONS
SYSTEMIC SYMPTOMS
– fever, malaise, weight loss and anorexia. JOINT SYMPTOMS
• Arthralgia/arthritis (synovitis)
MUCOCUTANEOUS • Joint swelling, tenderness, pain on
• Butterfly rash (malar rash) movement, morning stiffness.
• Subacute cutaneous lupus erythematosus
CARDIOVASCULAR
• In some patients’, skin lesions are
precursor to more systemic involvement • Pericarditis – is most common cardiac
manifestation.
 • Myocarditis, hypertension, arrythmias CENTRAL NERVOUS SYSTEM
and valvular incompetence. • Widespread involvement
• Early onset atherosclerosis for women. • Cognitive impairment
• Psychosis
• Seizures
• Neuropathies
• Strokes

DIAGNOSTIC FINDINGS
• Based on complete history, physical
examination, and blood tests.
• The American College of Rheumatology
has established criteria for classification of
SLE involving 11 distinct elements.

KIDNEY
• Lupus Nephritis – due to build up of
antibodies and immune complexes that
cause damage to the nephron.
 American College or Rheumatology
Criteria for the Diagnosis of Systemic
Lupus Erythematosus (SLE)

Malar rash
A rash on the cheeks and nose, often in the
shape of a butterfly.

Discoid rash
A rash on that appears as red, raised, disk-
shaped patches.

Photosensitivity
A reaction to sunlight that causes a rash to
appear or get worse.

Oral ulcers
A rash on that appears as red, raised, disk-
shaped patches.

Arthritis
Joint pain and swelling of 2 or more joints.

Serositis
Inflammation of the lining around the lungs
(pleuritis) or inflammation of the lining
around the heart that causes chest pain,
which is worse with deep breathing
(pericarditis).

Kidney disorder
Persistent protein or cellular casts in the
urine.

Neurologic disorder
Seizures or psychosis.

Blood disorder
Anemia (low red-cell count), leukopenia
(low white-cell count), lymphopenia (low
level of specific white cells), or
thrombocytopenia (low platelet count).

Immunologic disorder
Positive test for anti-double-stranded DNA,
anti-Sm, or antiphospholipid antibodies.

Abnormal antinuclear antibodies

Positive antinuclear-antibody test.
 MEDICAL MANAGEMENT PATHOPHYSIOLOGY
• Poorly understood.
GOAL OF TREATMENT • Commonly begins with skin involvement.
• Preventing progressive loss of organ
function. Mononuclear cells cluster on the skin ->
• Reducing the likelihood of acute disease. stimulate lymphokines to stimulate
• Minimizing disease-related disabilities. procollagen -> insoluble collagen is formed
• Preventing complications from therapy. and accumulates excessively in the tissues.

MAINSTAY OF TREATMENT • Initially, the inflammatory response

• Pain management and
immunosuppression.
• NSAIDs, corticosteroids, antimalarial
agents, and cytotoxic agents.
PHARMACOLOGIC THERAPY
Belimumab (Benlysta), 2011
• A monoclonal antibodies, halts the
production of unnecessary antibodies and
decreases disease activity in SLE.
Antimalarial medications
• Effective for managing cutaneous,
musculoskeletal, and mild systemic
features of SLE.
➢ Hydroxychloroquine (Plaquenil)
➢ Chloroquine (Aralin)
NSAIDs
• Used for minor clinical manifestations are
often used with corticosteroids to
minimize corticosteroids requirements.
➢ Diclofenac
➢ Celecoxib
causes edema, with a resulting taut,
smooth, and shiny skin appearance.
• The skin then undergoes fibrotic changes,
leading to loss of elasticity and movement.
• Eventually, the tissue degenerates and
becomes nonfunctional.
• This chain of events, from inflammation to
degeneration, also occurs in blood vessels,
major organs, and body systems.
CLIICAL MANIFESTATIONS
(EXTERNAL CHANGES)
• The skin and subcutaneous tissues
become increasingly hard and rigid due to
excess collagen and cannot be pinched up
from the underlying structures.
• Wrinkles and lines are obliterated.
• The skin is dry because sweat secretion
over the involved region is suppressed.
• The extremities stiffen and lose mobility.
• The face appears masklike, immobile, and
expressionless, and the mouth becomes
rigid; referred to as “stone facies”

Immunosuppressive Agents
• Reserved for patients with serious forms
of SLE that have not responded to
conservative therapy.
➢ Cyclophosphamide
➢ Azathioprine
➢ Methotrexate

SCLERODERMA

Scleroderma
→ Rare autoimmune disease affecting the
connective tissue of the skin, blood vessel
walls, and internal organs.

2 GENERAL TYPES:
a. Localized affecting only the cutaneous
system.
b. Diffuse routinely referred to as systemic
sclerosis & affecting multiple organ systems.
 CLIICAL MANIFESTATIONS Sclerodactyly
(SYSTEMIC CHANGES) In scleroderma, the abnormal build-up of fibrous
• More serious than external changes. tissue in the skin can cause the skin to tighten so
• Esophagus hardens, interfering with severely that the fingers curl and lose their
swallowing. mobility.
• Lungs become scarred, impeding
respiration.
• Digestive disturbances -> sclerosing
(hardening) of the intestinal mucosa.
• Vascular involvement of the kidneys
leads to malignant hypertension and renal
insufficiency.
• Cardiac disorders include pericarditis,
heart block, and myocardial fibrosis

CREST SYNDROME

Calcinosis (calcium deposits in the tissues)

Raynaud’s phenomenon
Esophageal dysmobility
Sclerodactyly (scleroderma of the digits)
Telangiectasia (capillary dilation that forms
a vascular lesion)

The limited symptoms of scleroderma are

referred to as CREST.

Calcinosis – calcium deposits in the skin.

Raynaud’s phenomenon – spasm of blood
vessels in response to cold or stress.
Esophageal dysfunction – acid reflux and
decrease in motility of esophagus.
Sclerodactyly – thickening and tightening
of the skin on the fingers and hands.
Telangiectasias – dilation of capillaries Telangiectasia
causing red marks on surface of skin. Dilation (widening) of small vessels and
capillaries cause flat red marks to appear on the
skin.

DIAGNOSTIC FINDINGS
• Assessment focuses on sclerotic changes
in the skin, contractures in the fingers, and
color changes, or lesions on fingertips.
• Systemic manifestations, CREST.
• CT, echocardiography, esophageal studies.
• (+) antinuclear antibodies (ANA) ->
indicates connective tissue disorder
 MEDICAL MANAGEMENT
• Strategies to decrease pain or limit
disability.
• Moderate exercise program.
• Avoid extreme temperatures and lotion to
minimize skin dryness.

PHARMACOLOGIC THERAPY
• NO MEDICATION REGIMEN is effective in
modifying scleroderma, it is mainly
symptomatic and supportive.
• There are various medications to treat
organ system involvement.

Calcium Channel Blockers -> (e.g., Nifedipine)

• Improvement of Raynaud’s phenomenon.

Angiotensin-converting Enzyme Inhibitors

(ACE Inhibitors) -> (e.g., Captopril)
• For hypertensive kidney disease.

Proton Pump Inhibitor

• For gastric reflux.

Vasoactive Medications
• Epoprostenol (Flolan)
• Sildenafil (Viagra)

Immunosuppressive Agents
• Methotrexate

Antifibrotic Agent -> (e.g., Imatinib Mesylate)

• To decrease fibrosis in various organs.

NURSING MANAGEMENT
NURSING DIAGNOSES
• Impaired skin integrity
• Self-care deficits
• Imbalanced nutrition: less than the body
requirements
• Disturbed body image

MAJOR NURSING CHALLENGES

• Providing meticulous skin care
• Preventing the effects of Raynaud’s
phenomenon