Connective Tissue Disease Flashcards

1
Q

Diffuse Connective Tissue Diseases

A
  • A group of chronic disorders characterized by diffuse inflammation and degeneration in the connective tissue
  • Cause is unknown but thought to have an immunologic basis
  • Characterized by a clinical course of exacerbations and remissions
  • Includes rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), scleroderma, polymyositis, Sjogren Syndrome, polymyalgia rheumatica, and Giant Cell arteritis
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2
Q

SLE Systemic Effects

A
  • Fever
  • Fatigue
  • Anemia
  • Lymph node enlargement
  • Raynaud’s phenomenon (half hand turns blue) and Sjoren Syndrome
  • Any organ system may be involved; arteritis, neuropathy and other neurologic manifestations, scleritis, pericarditis, pleural effusion, splenomegaly, renal involvement
  • Skin and mucosal manifestations
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3
Q

Systemic lupus erythematosus (SLE)

A
  • Thought to be auto-immune…possible combination of genetic and environmental factors…
  • Antinuclear Antibodies affect DNA, RNA and nucleus.
    — Antinuclear Antibodies first test done to see if you have lupus
  • Chronic/progressive, inflammatory connective tissue disorder
  • Causes major body organs and systems to fail via formed autoimmune complexes in serum and tissues which cause inflammation and damage.
  • Vasculitis – Vessel inflammation deprives organs of blood and O2
  • Flareups …remissions and exacerbations
  • Onset acute or insidious
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4
Q

*Lupus Erythematosus (LE) risk factors

A
  • Affects women 6-10 times more than men
  • 3 x more in African Americans
  • 20-40 years of age (child bearing age)
  • Many forms besides SLE
  • Discoid lupus erythematosus (skin)
  • Subacute cutaneous LE
  • Drug induced LE
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5
Q

Lupus Erythematosus (LE) (cont.)

A
  • Main Classification:
    — SLE - Systemic lupus erythematosus
  • Other Forms:
    — DLE - Discoid lupus erythematosus (skin/ face)
    — Subacute – Sun exposed areas affected with sores
    — Drug Induced- rarely includes brain or kidney, Temporary
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6
Q

*Lupus Erythematosus (LE) Triggers

A
  • *Sunlight - Exposure to the sun may bring on lupus skin lesions or trigger an internal response in susceptible people.
  • *Infections - Having an infection can initiate lupus or cause a relapse in some people.
    — stay away from sick people/ crowds
    — wash hands
    — take temperature
  • Medications - Lupus can be triggered by certain types of blood pressure medications, anti-seizure medications and antibiotics. People who have drug- induced lupus usually get better when they stop taking the medication.
    — take meds
    — drug induced lupus can be reversed
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7
Q

Assessment of Auto- Immune Disease

A
  • Health history and physical assessment focus on:
    include onset of and evolution of symptoms, family history, past health history, and contributing factors
    — when did it start?
  • Current and past symptoms
  • Patient’s psychological and mental status
  • Social support systems
  • *Ability to participate in daily activities or Functional Assessment
  • Compliance with treatment regimen
  • Management of self-care
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8
Q

Diagnostic Findings Chart 38-5 Criteria for Classification

A
  • Full Assessment including all body systems
  • Skin Assessment
  • Laboratory:
    — *Skin biopsy (only significant test to confirm diagnosis)
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9
Q

SLE Clinical Manifestations

A
  • Muscles Involvement
  • Polyarthritis – joint pain
  • Osteonecrosis (avascular necrosis)
  • Muscle atrophy
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10
Q

SLE Key Features

A
  • Any organ system may be involved
  • Cardiac – Myocardial fibrosis
  • Musculoskeletal – Joint Inflammation
  • Renal – Kidney Failure
  • Pulmonary –Pulmonary HTN
  • Neuro Not common
  • GI – Esophagitis , Ulcers
  • Other: Fever Fatigue Anorexia Vasculitis
  • Lymph node enlargement
  • Raynaud’s syndrome
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11
Q

SLE—Clinical Manifestations

A
  • Renal involvement
    — Nephritis, Kidney Failure
  • Pulmonary
    — Pleural effusions
  • Cardiac
    — Pericarditis, endocarditis, myocarditis
    — Raynaud’s phenomenon
  • Neurologic manifestation
    — *Fever first sign of flare up (take temperature)
  • Fatigue
  • Anorexia
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12
Q

Pericarditis Assessment

A
  • Pain
  • ST elevation
  • Signs of heart failure
  • Assess for hx of systemic lupus erythematosus (SLE) or any systemic connective tissue disease
  • Acute pericarditis is most commonly associated with acute exacerbations of systemic connective tissue disease, including SLE.
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13
Q

Raynaud’s Disease

A
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14
Q

Raynaud’s Disease Drug Therapy

A

Know signs and symptoms, adverse effects of each.
- Monoclonal antibodies – First drug approved in 60 years for SLE
— *Belimumab (Benlysta)
- allows B cells to undergo apoptosis as normal to avoid inflamation from antibody production 
— stimulate B cells to produce antibodies against the body’s own nuclei
- Corticosteroids – risk of osteoporosis and fractures
— Take meds early in the morning before breakfast when body’s natural corticosteroid level is low and reduces toxicity.
— Chronic steroid use >5 years - Osteonecrosis (avascular necrosis)
Antimalarial-
- hydroxychloroquine (Plaquenil) – anti-malarial
— check eyes
- NSAIDS/Tylenol - joint and muscle pain and inflammation.
- Immunosuppressive Agents

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15
Q

SLE Nursing Interventions

A
  • Understanding the underlying disease process guides the nurse’s critical thinking to provide interventions
  • Administer Medication
  • Protect skin – mild soap avoid perfume, cosmetics, watch for alopecia, hair will grow back during remission.
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16
Q

What self-management education by the nurse is important for clients diagnosed with systemic lupus erythematosus who are taking prednisone? Select all that apply.
- A “Take calcium supplements to prevent osteoporosis from the steroid.”
- B “Stay away from crowds and people with infections.”
- C “Avoid being in the sun to prevent disease flare-ups.”
- D “Get up slowly to prevent dizziness from orthostatic hypotension.”
- E “Take your prednisone early in the morning before breakfast.”

A

A,B,C,E

17
Q

Care Plan of the Patient With an auto-immune

A

Major goals may include:
- Relief of pain and discomfort
- Relief of fatigue
- Promotion of restorative sleep
- Increased mobility
- Maintenance of self-care
- Improved body image
- Effective coping
- Absence of complications

18
Q

The health care provider prescribes celecoxib (Celebrex) (NSAID) for a client with SLE. What health teaching will the nurse provide for this client regarding this drug? Select all that apply.
- A. “Take the drug on an empty stomach before breakfast.”
- B. “Stop taking the drug if unusual bleeding occurs and call your health care provider.”
- C. “Report frequent episodes of indigestion to your health care provider.”
- D. “Expect fluid accumulation in your legs and feet that usually gets worse during the day.”
- E. “Call 911 immediately if chest pain occurs.”

A

B, C, E

19
Q

Systemic Sclerosis/ Scleroderma

A

DISEASE OF OVERPRODUCTION AND ACCUMULATION OF COLLAGEN
- Starts off as reynuads
Chronic & progressive hardening of the skin
- affects other body systems

20
Q

*CREST Syndrome
Scleroderma?

A

Patients with the limited form of the disease often have the CREST syndrome
- *C—calcinosis….ca. deposits in the skin
- *R—Raynaud’s phenomenon; spasms of blood vessels in response to cold or stress; sometimes the first symptom
- *E—esophageal dysmotility/ dysfunction; acid reflux and decrease in motility of esophagus
- *S—sclerodactyly…(scleroderma of digits); thickening and tightening of the skin on the fingers and hands
- *T—telangiectasia..(capillary dilation that forms a vascular lesion ); dilation of capillaries causing red marks on the surface of the skin (on face)

21
Q

PROGRESSIVE SYSTEMIC SCLEROSIS

A
  • AKA: Systemic Scleroderma
  • Little is known about this disease, but autoimmunity is suspected
  • Scleroderma means “hardening” of the skin
  • Chronic connective tissue disease characterized by inflammation, fibrosis and sclerosis of the skin and vital organs
  • The inflammatory process is so similar to SLE, many are often diagnosed as having probably SLE.
  • The tissue is most obviously involved, but renal involvement is the leading cause of death
22
Q

Scleroderma—Interventions

A
  • Drug therapy – No specific Drug
    — Blood Pressure Meds to dilate blood vessels
    — Anti inflammatory’s
    — Proton Pump Inhibitor for GI manifestation of Esophagitis
  • Immunosprresives
    — Cclophosphamide and methotrexate
  • Identify early organ involvement
  • Skin protective measures
  • Comfort
  • Mobility
23
Q

Scleroderma Treatment

A
  • New treatments that are currently under investigation:
    — Interferons
    — Tumor necrosis factor alpha blockers
    — Halofuginone
    — Plasmapheresis
    — Photopheresis
    — Autologous stem cell transplantation
  • The prognosis for people with scleroderma and the important factors that have a significant impact in predicting the overall chances of recovery.
  • Quality of life issues that often confront people with scleroderma and how to minimize their impact and cope better with these issues.