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
Musculoskeletal
Butterfly rash – 50% of patients Ulcers – mouth or nose Alopecia Polyarthralgia – 90% of patients Non-erosive arthritis
Cardiopulmonary
Tachypnea & cough Arrhythmias Accelerates CAD
Manifestations & Complications
Renal problems – 50% of clients within one year
Mild proteinuria to glomerulonephritis
Nervous system
Seizures Disordered thought processes Disorientation Memory deficits Depression Psychosis
Manifestations & Complications
Hematological problems Anemia Mild
leukopenia Thrombocytopenia
Infection Immunosuppresive
effect of anti-
inflammatory drugs Defects in ability to phagocytize invading bacteria
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
Assess neuro status
Visual disturbances Mental status Seizures Numbness, tingling, weakness of extremities
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
complications of involved
organs Medications:
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