Nursing Care of the Client with Connective Tissue Disease

Systemic

Lupus Systemic Sclerosis Polymyositis

Systemic Lupus Erythmatosus
Multisystem Inflammatory Disease

Etiology and Pathophysiology

Etiology – unknown
 Genetic

predisposition  Hormones  Environmental factors  Triggers:
Infection  Certain medications

Overaggressive Antibody Response

Immune complexes deposited in basement membranes of capillaries:
 Kidneys  Heart  Skin  Brain  Joints

* Specific symptoms of SLE depend on which cells or organs are involved

Manifestations & Complications
  

Variable depending on severity Any organ(s) may be affected Most commonly affected:
 Skin  Muscle  Lungs  Heart  Nervous  Kidneys

system

Manifestations & Complications

Skin
  

Butterfly rash – 50% of patients Ulcers – mouth or nose Alopecia Polyarthralgia – 90% of patients Non-erosive arthritis Tachypnea & cough Arrhythmias Accelerates CAD

Musculoskeletal
 

Cardiopulmonary
  

Manifestations & Complications

Renal problems – 50% of clients within one year

Mild proteinuria to glomerulonephritis Seizures Disordered thought processes Disorientation Memory deficits Depression Psychosis

Nervous system
     

Manifestations & Complications

Hematological problems
 Anemia  Mild

leukopenia  Thrombocytopenia

Infection
 Immunosuppresive

inflammatory drugs  Defects in ability to phagocytize invading bacteria

effect of anti-

Diagnosis of SLE
 

No specific diagnostic test Four or more of criteria are present serially or simultaneously Presence of criteria are not definitive for diagnosis “rule-out” other disease processes

Drug Therapy
  

 

NSAID’s hydroxychloroquine (Plaquenil) corticosteroids – especially during severe exacerbations of polyarthritis methotrexate azathioprine (Imuran)

Nursing Care

Assess severity of symptoms: fever, joint pain and limitations of movement, fatigability Weight and I&O – risk of fluid overload:
 renal

failure  corticosteroid therapy

Nursing Care

Observe S/S bleeding
    

Changes in vital signs Bruising Petechiae Tarry stools Pallor Visual disturbances Mental status Seizures Numbness, tingling, weakness of extremities

Assess neuro status
   

Patient Education

 

Avoid triggers: sun, infections, emotional stress, etc. Pre-conception counseling Correct use of medication(s) and importance of compliance Disease complications are “hidden”; physical limitations are not obvious

Patient Education
Web site co-sponsored by NIH and National Institute of Arthritis and Musculoskeletal and Skin Diseases

http://www.niams.nih.gov/hi/topics/lupus

Systemic Sclerosis
   

Fibrotic, degenerative changes Skin thickening/tightening Localized vs. systemic Causes:
 Immune

dysfunction  Vascular abnormalities  Environmental exposure

Systemic Sclerosis
 

Collagen is over produced CREST syndrome:
 Calcinosis  Raynaud’s

phenomenon  Esophageal dysfunction  Sclerodactyly  Telangiectasia

Systemic Sclerosis

Internal organ involvement
 Esophageal

fibrosis  Lung involvement  Heart disease (CHF)  Kidney disease with malignant hypertension

Nursing and Collaborative Care

Goal of medical regimen:
 Prevent

organs  Medications:

complications of involved

Corticosteroids  Vasodilators (Raynaud’s, hypertension)  H2 receptor blocker or proton pump inhibitor (GI symptoms)
 

Physical therapy Nursing care

Nursing Care

Assessment:
 Joint

ROM  Respiratory function  Nutrition: swallowing, weight, I&O  Circulation: Raynaud’s  Body image

Nursing Care

Nursing Interventions:
 ROM

exercises, mouth excursion  Moist heat/parafifin  Protect hands and feet:
Keep warm  Gloves  No finger-stick blood testing  No smoking – vasoconstriction  Inspect for injury/infection

Nursing Care
 

Special attention to oral care Nutrition and hydration
 Small,

frequent meals  Carefull/complete chewing  Dysphagia precautions
Thickened liquids  High fowlers 2 hours after eating

 Antacids

45 minutes after eating if gastric reflux

Resources for clients and health professionals
http://www.scleroderma.org/

Polymyositis

 

Diffuse, inflammatory myopathies of striated muscle Relatively rare Produces bilateral weakness of shoulders, legs, arms and pelvic girdle Joint pain, reddness, inflammation

Collaborative Care

Patient teaching:
     

Prescribed therapies Tests Importance of follow-up care Delay in response to therapy Patient safety Paced activity

  

Physical therapy High-dose corticosteroids Immunosuppressive drugs

methotrexate

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