Diffuse Connective Tissue Diseases
- 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
SLE Systemic Effects
- 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
Systemic lupus erythematosus (SLE)
- 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
*Lupus Erythematosus (LE) risk factors
- 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
Lupus Erythematosus (LE) (cont.)
- 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
*Lupus Erythematosus (LE) Triggers
- *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
Assessment of Auto- Immune Disease
- 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
Diagnostic Findings Chart 38-5 Criteria for Classification
- Full Assessment including all body systems
- Skin Assessment
- Laboratory:
— *Skin biopsy (only significant test to confirm diagnosis)
SLE Clinical Manifestations
- Muscles Involvement
- Polyarthritis – joint pain
- Osteonecrosis (avascular necrosis)
- Muscle atrophy
SLE Key Features
- 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
SLE—Clinical Manifestations
- 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
Pericarditis Assessment
- 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.
Raynaud’s Disease
Raynaud’s Disease Drug Therapy
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
SLE Nursing Interventions
- 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.
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,B,C,E
Care Plan of the Patient With an auto-immune
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
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.”
B, C, E
Systemic Sclerosis/ Scleroderma
DISEASE OF OVERPRODUCTION AND ACCUMULATION OF COLLAGEN
- Starts off as reynuads
Chronic & progressive hardening of the skin
- affects other body systems
*CREST Syndrome
Scleroderma?
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)
PROGRESSIVE SYSTEMIC SCLEROSIS
- 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
Scleroderma—Interventions
- 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
Scleroderma Treatment
- 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.
