Autoimmune Diseases Lecture Notes

Overview of Autoimmune and Neurodegenerative Diseases

• Subject: Complex-RNSG-1443 Nursing

• Presenters: Mason Loudermilk, MSN, RN and Ophelia Abankwah, DNP, FNP-BC, CCRN-CMC

• Core Focus: Pathophysiology, clinical manifestations, diagnostic studies, and nursing/collaborative care for Multiple Sclerosis (MS), Parkinson’s Disease (PD), Myasthenia Gravis (MG), Guillain-Barre Syndrome (GBS), and Systemic Lupus Erythematosus (SLE).

Multiple Sclerosis (MS)

• Pathophysiology and Etiology

  • Definition: A chronic, progressive, and degenerative disorder affecting the nerve cells in the brain and spinal cord (Central Nervous System).

  • Genetic Factor: There is a possible genetic link associated with the development of the disease.

  • The Three Pathological Processes:

    1. Chronic inflammation.

    2. Demyelination (loss of the protective myelin sheath around nerves).

    3. Gliosis in the CNS (scarring/plaque formation).

  • Outcome: Permanent loss of nerve functionality occurs over time.

• Clinical Manifestations

  • Onset: Typically slow and vague, often appearing between the ages of $20$ and $40$.

  • Disease Patterns: There are $4$ primary patterns of progression (referenced in Table 63.12).

  • Vision Symptoms (Often the First Sign):

    • Blurred vision.

    • Diplopia (double vision).

    • Nystagmus (involuntary eye movement).

  • Motor and Sensory Symptoms:

    • Muscle weakness which may progress to paralysis.

    • Muscle spasticity.

    • Ataxia (lack of muscle coordination) and vertigo.

    • Fatigue and coordination/balance issues.

    • Hearing loss or Tinnitus.

  • Cognitive and Speech Symptoms:

    • Dysarthria (difficulty speaking).

    • Dysphagia (difficulty swallowing).

    • Memory issues.

  • Elimination Symptoms:

    • Spastic bladder.

    • Urinary retention.

    • Constipation.

• Diagnostic Criteria

  • A diagnosis is made by ruling out all other possibilities and meeting specific physical criteria:

    1. Evidence of at least $2$ lesions in at least $2$ different locations within the CNS.

    2. Evidence that the damage or attacks occurred at different times.

  • Imaging: MRI is used to detect plaque, active inflammation, or chronic lesions.

• Treatment and Collaborative Care

  • Cure: There is no known cure for MS.

  • Disease-Modifying Medications:

    • Interferon B-1a: Rebif, Plegridy, Avonex.

    • Interferon B-1b: Betaseron, Extavia.

    • Glatiramer Acetate: Copaxone, Glatopa.

  • Acute Exacerbations: Treated primarily with Steroids.

  • Symptomatic Treatments:

    • Antidepressants for depression.

    • Muscle relaxers for spasticity.

    • Physical Therapy (PT) and Occupational Therapy (OT).

    • Regular exercise.

• Nursing Management

  • Monitor vital functions: vision, speech, swallowing, and fatigue levels.

  • Monitor fluid intake and bowel/bladder habits.

  • Assess cognitive changes and development of coping mechanisms.

  • Educate on energy conservation and planning for long-term disease progression.

Parkinson’s Disease (PD)

• Characteristics and Manifestations

  • Nature: A progressive and debilitating neurodegenerative disease.

  • The "TRAP" Acronym (Primary Characteristics):

    1. Tremors: Often the first sign; may occur at rest.

    2. Rigidity: Increased resistance to passive motion.

    3. Bradykinesia: Slowness of movement.

    4. Postural Instability: Difficulty maintaining balance or upright posture.

  • Physical Appearance:

    • Blank facial expression (masked facies).

    • Slow, monotonous, and slurred speech.

    • Forward tilt to posture.

    • Short, shuffling gait.

• Diagnosis

  • Criteria: Based on history and clinical presentation. The patient must exhibit at least $2$ of the $4$ "TRAP" characteristics.

  • Other Clinical Indicators: Asymmetric onset and a positive response to antiparkinsonian medications.

  • Confirmation: The disease is confirmed by a positive response to Levodopa or a Dopamine (DA) agonist.

• Treatment

  • Dopaminergics: Sinemet (Carbidopa/Levodopa) is the primary gold-standard treatment.

  • Dopamine Agonists: Ropinirole (Requip) and Pramipexole (Mirapex).

  • Surgical Therapy:

    • Deep Brain Stimulation (DBS).

    • Ablation.

    • Transplantation.

  • "Off Episodes": Management of periods when medications wear off (Refer to Drug Table 63.17).

• Nursing Management

  • Dietary Needs: Provide appetizing foods that are easy to chew and swallow. Implement $6$ small meals per day to manage fatigue and intake.

  • Maintenance: Focus on maintaining general health as there are no "acute exacerbations" like MS; the goal is to prevent decline.

  • Home Safety: Assess for fall risks and implement safety measures.

  • Psychosocial: Address depression and anxiety; encourage counseling and support groups.

  • Independence: Promote independence while providing PT and exercise regimens.

Myasthenia Gravis (MG)

• Pathophysiology

  • An autoimmune process where antibodies attack acetylcholine receptors, preventing nerve impulses from stimulating muscle contraction.

• Clinical Manifestations

  • Fluctuating Weakness: Skeletal muscle weakness that increases with muscle use.

  • Diurnal Variation: Muscles are typically strongest in the morning; resting helps restore strength.

  • Exacerbation Triggers: Fatigue, pregnancy, illness, trauma, and stress.

  • Drug-Induced Worsening: Symptoms can be exacerbated by $\beta$-adrenergic blockers, quinidine, phenytoin, and certain antibiotics.

• Diagnostics

  • EMG: Shows a decreased response to repeated muscle stimuli.

  • Tensilon Test: IV injection of Tensilon results in a rapid, temporary improvement in muscle strength.

    • Warning: Atropine (the antidote) must be kept close during the test to reverse potential cholinergic effects.

• Crisis Comparison

  • Myasthenic Crisis:

    • Acute worsening triggered by infection, surgery, or distress.

    • Affects swallowing and breathing.

    • Response: Improves with anticholinesterase drugs.

  • Cholinergic Crisis:

    • Weakness occurring within $1$ hour of taking anticholinesterase drugs.

    • Features: Involuntary muscle contraction, sweating, excessive salivation, and constricted pupils.

    • Response: Worsens with more anticholinesterase drugs; requires immediate medical attention.

• Treatment and Nursing Care

  • Medications:

    • Anticholinesterase: Pyridostigmine (Mestinon).

    • Corticosteroids: Prednisone (alternate-day dosing).

    • Immunosuppressants: Azathioprine (Imuran), Mycophenolate (CellCept).

  • Invasive Procedures: Thymectomy (surgical removal of the thymus), Plasmapheresis, and IV Immunoglobulin G (IVIG).

  • Nursing Focus:

    • Schedule medications to peak during meal times or activities.

    • Ensure a balanced, easy-to-swallow diet.

    • Plan rest periods and utilize OT for ADLs.

Guillain-Barre Syndrome (GBS)

• Pathophysiology

  • Acute edema and inflammation to the peripheral nerves.

  • Usually follows a viral or bacterial infection.

  • Common Precursors: Cytomegalovirus (most common), Epstein-Barr virus, and respiratory or intestinal infections. It has an association with recent immunizations.

• Clinical Manifestations

  • Pattern: Acute, ascending (starts in feet and moves up), rapidly progressive, and symmetric weakness.

  • First Symptoms: Weakness, numbness, tingling (paresthesia), and reduced muscle tone.

  • Reflexes: Weak or absent (areflexia).

  • Progression: Reaches maximum weakness within $4$ weeks. It may progress to total paralysis.

  • Major Risks: Respiratory failure (most serious), problems with talking, swallowing, and bowel/bladder function.

• Diagnosis and Acute Care

  • Diagnostics: CSF analysis shows elevated protein levels; EMG and nerve conduction studies (usually $2$ weeks after onset).

  • Acute Nursing/Medical Care:

    • Ventilatory support is critical if respiratory muscles are involved.

    • Plasmapheresis or IV Immunoglobulin (IVIG) within the first $2$ weeks are effective.

    • Note: After $4$ weeks, plasmapheresis and IVIG are generally no longer helpful.

  • Recovery: Most patients experience spontaneous recovery over time.

Systemic Lupus Erythematosus (SLE)

• General Characteristics

  • A multi-system inflammatory autoimmune disease affecting skin, joints, and serous membranes (pleura, pericardium).

  • Course is chronic and unpredictable with alternating periods of remission and exacerbation.

  • Etiology is unknown but suspected to be genetic; hormonal changes often worsen the condition.

• Systemic Manifestations

  • Skin: Butterfly rash (over cheeks/bridge of nose), photosensitivity (rashes in sun-exposed areas), vascular lesions, cutaneous lesions, and alopecia.

  • Musculoskeletal (MSK): Polyarthralgia with early morning stiffness, joint swelling, and "swan neck" finger deformities.

  • Cardiopulmonary: Tachypnea, cough, pleurisy (pleural inflammation/effusion), HTN, Raynaud's phenomenon, pericarditis, and potential fibrosis of the SA and AV nodes.

  • Renal: Lupus Nephritis, characterized by proteinuria and hematuria.

  • Nervous System/Psychosocial: Seizures, neuropathy, cognitive problems, and emotional lability.

  • General: Weight loss, fatigue, fever, and increased risk of infection.

• Treatment and Management

  • Medications:

    • NSAIDs (important intervention for joint pain).

    • Antimalarial agents: Hydroxychloroquine.

    • Corticosteroids.

    • Immunosuppressive drugs.

  • Monitoring: $24$-hour urine collection to monitor protein and creatinine clearance.

  • Education: Teach the nature of the disease, compliance with treatment, and sun restrictions (UV protection).

• Pregnancy and Family Planning

  • Pregnancy must be planned and discussed with a physician.

  • Risks: Possible infertility, spontaneous abortion, stillbirth, and Intrauterine Growth Retardation (IUGR).

  • Contraindications: Counseling against pregnancy is recommended if there is severe Renal, CV, Lung, or CNS involvement.