Med-Surg: Chapter 20: Scleroderma

Question 1

Scleroderma

Answer 1

• autoimmune disorder
• affects skin and may involve internal organs
• small-vessel vasculopathy (disease affecting blood vessels), pathological accumulation of collagen, and autoimmunity cause the disease manifestations
• excess collagen formation

Question 2

Pathophysiology

Answer 2

tissue damage occurs when mononuclear cells cluster in tissues such as the skin, blood vessels, and organs, stimulating lymphokines that stimulate procollagen, causing insoluble collagen formation in excess

• the inflammatory response causes edema, which leads to fibrotic changes, causing loss of elasticity and movement
• eventually the tissue degenerates and becomes nonfunctional

Question 3

Scleroderma is divided into 2 categories

Answer 3

• Localized

- Systemic

Question 4

Localized Scleroderma Manifestations

Answer 4

• morphea (isolated patches of hardened skin)
• linear scleroderma (lines of thickened skin affecting the tissues beneath)
• scleroderma en coup de saber (thickened skin on head)
• localized does not affect internal organs

Question 5

Systemic Scleroderma manifestations

Answer 5

• divided into diffuse and limited
• may involve the skin, lungs, heart, kidneys, and musculoskeletal system
• Skin: patches of thickened skin; can be itchy
• Pulmonary: dyspnea, nonproductive cough, pulmonary fibrosis; increased risk of lung cancer
• -Cardiac: pericarditis, pericardial effusion, myocardial fibrosis, heart failure, and arrhythmia
• Renal: proteinuria and impaired renal function
• Musculoskeletal: swelling of the hands, joint pain, muscle pain, fatigue

Question 6

Three Important Manifestations of Systemic Sclerosis

Answer 6

• Raynaud’s Phenomenon
• Scleroderma Renal Crisis
• Pulmonary Artery Hypertension

> all due to abnormal vasoconstriction of small vessels as a result of functional changes in the arteries

Question 7

Systemic Scleroderma: Limited vs Diffuse

Answer 7

> Limited:

• insidious onset
• involves the skin of extremities distal to the elbows and knees
• internal organ involvement less likely and with late onset
• Raynaud’s Phenomenon may precede disease diagnosis by years

> Diffuse:

• rapid onset
• involves skin of extremities and trunk
• affects internal organs, typically 2 years of onset
• Raynaud’s Phenomenon may occur concurrently or after diagnosis

Question 8

Raynaud’s Phenomenon

Answer 8

results from vasospasm of the small vessels in the hands caused by exposure to cold
-vasospasm of the small vessels causes the fingers to blanch white, become blue from cyanosis, and then become red from reactive hyperemia
-is secondary to scleroderma and should not be confused with primary Raynaud’s Disease (primary is common in the general population and does not result in permanent tissue damage)
-Raynaud’s Phenomenon causes permanent tissue damage and loss of digits due to chronic ischemia
-

Question 9

Scleroderma Renal Crisis

Answer 9

sudden onset of severe hypertension, proteinuria, and progressive renal failure
-even with tx, mortality rate and morbidity rate is high

Question 10

Pulmonary Artery Hypertension

Answer 10

-symptoms develop insidiously, with a feeling of generalized weakness on exertion, then later dyspnea

Question 11

Common Clinical Manifestations of Systemic Scleroderma

Answer 11

> Skin:

• thickened skin
• digital ulcers
• puffy hands
• superficial skin tenderness
• diffuse hyperpigmentation

> GI

• frequent heartburn
• dysphagia
• esophageal stricture
• erosive esophagitis
• postprandial bloating
• constipation
• malabsorptive diarrhea

> Pulmonary

• generalized fatigue
• SOB
• dry cough
• lung fibrosis

> Cardiac

• arrhythmias
• heart failure
• pericarditis

> Renal

• renal insufficiency
• renal failure

> Musculoskeletal

• joint contractures
• tendon friction rubs
• muscle pain and weakness
• synovitis

Question 12

Diagnosis

Answer 12

• presence of clinical manifestations and serum antibodies
• antinuclear antibody (ANA) screening
• specific antibodies such as antitopoisomerase I, anticentromere, anti-RNA polymerase III, and anti-beta2 glycoprotein I
• radiographic testing
• pulmonary function tests
• echocardiograms
• kidney biopsies for renal disease

Question 13

Radiographic testing shows

Answer 13

• subcutaneous calcification
• distal esophageal hypomotility
• pulmonary fibrosis

Question 14

Pulmonary function test

Answer 14

-assess the vital lung capacity and lung compliance

Question 15

Echocardiogram

Answer 15

done routinely to assess for pulmonary artery hypertension and myocardial involvement

Question 16

Treatment

Answer 16

• no single tx
• focused on specific organ involvement and clinical manifestations
• systemic steroids (prednisone) suppress the immune response by reducing the migration of leukocytes and reducing the activity of the lymphatic system
• immunosuppressants (methotrexate), work by modulating the immune response to decrease inflammation
• antihistamines (loratadine (claritin) and cetirizine (Zyrtec)) may alleviate pruritis by blocking H1 receptors on effector cells
• topical ointments and moisturizers are used to help soften the skin and relieve itching
• Vasodilators (amlodipine (Norvasc) or nifedipine (Procardia)) are used to improve circulation and treat Raynaud’s Phenomenon, pulmonary hypertension, and renal impairment

Question 17

Prednisone

Answer 17

systemic steroid

-suppress the immune response by reducing the migration of leukocytes and reducing the activity of the lymphatic system

Question 18

Methotrexate

Answer 18

immunosuppressant

-work by modulating the immune response to decrease inflammation

Question 19

Antihistamines

Answer 19

-Loratadine (Claritin)
-Cetirizine (Zyrtec)
>may alleviate pruritis by blocking H1 receptors on effector cells

Question 20

Vasodilators

Answer 20

-Amlodipine (Norvasc)
-Nifedipine (Procardia)
>used to improve circulation and treat Raynaud’s Phenomenon, pulmonary hypertension, and renal impairment

Question 21

Nursing Management: Assessment and Analysis

Answer 21

clinical manifestations of scleroderma are r/t multiple factors according to the extent of organ involvement as well as side effects of pharmacological therapy

• fatigue
• thickened or hardened skin
• digital ulcers
• dyspnea
• hypertension
• decreased range of motion of joints
• poor nutrition
• frequent heartburn
• dysphagia
• cough

Question 22

Nursing Diagnoses

Answer 22

• altered tissue perfusion r/t chronic ischemia
• fatigue r/t the disease process
• altered self-image r/t disfiguring skin lesions

Question 23

Nursing Assessments

Answer 23

> Vital signs at every assessment: BP, HR and rhythm, respiratory rate

• elevated BP may be a sign of renal crisis
• elevated HR and ventricular arrhythmias may be present due to HF
• increased respiratory rate and effort may = interstitial lung disease, pulmonary vascular disease, or pulmonary artery hypertension

> Auscultate Lung Sounds
presence of crackles may = interstitial lung disease

> Assess Skin Integrity and document skin thickening

• swelling of the hands, joint pain, muscle pain, and fatigue are common symptoms of scleroderma
• fibrosis (fibrotic scarring) around tendons and joint structures may lead to joint immobility

> Auscultate bowel sounds and gather info regarding gastroesophageal motility
-chronic gastric reflux, difficulty swallowing, choking, cough, bloating, and alternating constipation and diarrhea are manifestations of GI involvement

> Monitor serum creatinine and blood urea nitrogen (BUN)
-to assess renal function and possible renal failure

Question 24

Nursing Actions

Answer 24

> Administer medications as ordered: Steroids, immunosuppressants, antihistamines, vasodilators, steroids, topical ointment

• steroids reduce the inflammatory response
• immunosuppressants modulate the immune response to decrease inflammation
• antihistamines alleviate pruritis
• vasodilators dilate arterial vessels, improving circulation
• ointments soften skin and relieve itching

> Perform Range-of-Motion exercises
-improve joint mobility and help prevent contractures

> Assist in referring to a pulmonologist, gastroenterologist, plastic surgeons, and PT
-diffuse scleroderma may lead to decreased pulmonary function, GI dysfunction, disfiguring skin lesions, and decreased joint mobility

> Refer patients to counseling as needed
-many suffer from depression

Question 25

Teaching

Answer 25

> Scleroderma Disease process

• is a very complicated disease that varies from one patient to another
• patients should understand and monitor for possible complications but realize they may not experience all possible complications

> Protect skin from Trauma
-patients are at higher risk for infection and prolonged healing time r/t decreased tissue perfusion

Question 26

Evaluating Care Outcomes

Answer 26

diffuse systemic sclerosis is a complicated and serious condition that is difficult to treat and usually results in disability and shortened life span

• there is no cure
• maintaining stable cardiac, pulmonary, and renal functions is the goal of tx
• care focused on monitoring, preventing, and managing complications
• maintenance of skin integrity may help minimize tissue loss, infection, and contractures
• well-controlled patient maintains skin integrity and pulmonary, cardiac, GI, and renal functions