Med-Surg: Chapter 20: Scleroderma Flashcards
Scleroderma
- 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
Pathophysiology
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
Scleroderma is divided into 2 categories
- Localized
- Systemic
Localized Scleroderma Manifestations
- 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
Systemic Scleroderma manifestations
- 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
Three Important Manifestations of Systemic Sclerosis
- 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
Systemic Scleroderma: Limited vs Diffuse
> 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
Raynaud’s Phenomenon
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
-
Scleroderma Renal Crisis
sudden onset of severe hypertension, proteinuria, and progressive renal failure
-even with tx, mortality rate and morbidity rate is high
Pulmonary Artery Hypertension
-symptoms develop insidiously, with a feeling of generalized weakness on exertion, then later dyspnea
Common Clinical Manifestations of Systemic Scleroderma
> 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
Diagnosis
- 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
Radiographic testing shows
- subcutaneous calcification
- distal esophageal hypomotility
- pulmonary fibrosis
Pulmonary function test
-assess the vital lung capacity and lung compliance
Echocardiogram
done routinely to assess for pulmonary artery hypertension and myocardial involvement
Treatment
- 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
Prednisone
systemic steroid
-suppress the immune response by reducing the migration of leukocytes and reducing the activity of the lymphatic system
Methotrexate
immunosuppressant
-work by modulating the immune response to decrease inflammation
Antihistamines
-Loratadine (Claritin)
-Cetirizine (Zyrtec)
>may alleviate pruritis by blocking H1 receptors on effector cells
Vasodilators
-Amlodipine (Norvasc)
-Nifedipine (Procardia)
>used to improve circulation and treat Raynaud’s Phenomenon, pulmonary hypertension, and renal impairment
Nursing Management: Assessment and Analysis
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
Nursing Diagnoses
- altered tissue perfusion r/t chronic ischemia
- fatigue r/t the disease process
- altered self-image r/t disfiguring skin lesions
Nursing Assessments
> 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
Nursing Actions
> 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
Teaching
> 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
Evaluating Care Outcomes
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
