L21 Autoimmune Disorders Systemic Lupus Erythematosus (SLE) & Myasthenia Gravis (MG) Flashcards
Immune system except protect body from disease and compromise, what else does it trigger?
Temp elevation and mucus production
What are the 3 immune system disorders?
1.Hypersensitivity disorder (e.g. Asthma, Allergic Rhinitis, Food Allergies)
2.Immunodeficiency disease (e.g. AIDS)
3.Autoimmune disorder (Rheumatoid arthritis,
Type 1 DM, Systemic Lupus Erythematosus, multiple sclerosis, Myasthenia Gravis)
What is an autoimmune disorder?
immune system MISTAKENLY attacks the body’s own healthy cells and tissues, mistakenly recognise them as foreign invaders. Leading to inflammation, tissue damage(extensive) and impaired function of the affected organs
What could be the causes of autoimmune disorders?
Genetic factors, environmental changes, hormonal influences, immune system dysregulation, age
Common s/s of autoimmune disorders:
Fatigue
Joint pain and swelling
Muscle weakness
Skin changes(e.g. dry)
Fever
Numbness or tingling
Digestive issues
Hair loss(alopecia)
Swollen lymph nodes
Weight change
Difficulty concentrating/
Brain fog
Myasthenia Gravis is an acute or chronic autoimmune disease?
Chronic
For MG, what receptors are blocked or destroyed? And where do the receptors located?
nicotinic acetylcholine receptors at the neuromuscular junction
What is the consequence of blocked or destroyed receptors? (MG)
The ability of acetylcholine to stimulate muscle contraction is impaired, causing muscle weakness
What are the common population of MG?
Women under 40 years of age(especially during childbearing years) and men over 60
What is the pathophysiology of MG?
Antibodies attack the Acetylcholine (ACh) receptors, decreased the number of ACh receptor site at neuromuscular junction, making it unable to stimulate muscle contraction.
What are the two risk factors of MG (cause is still unknown)
Thymus gland abnormalities(e.g. thymus tumor) and hyperythyrodism
For the clinical manifestations of MG, what are the ocular symptoms?
Diplopia (double vision)
Ptosis(upper eyelid droop 下垂)
Dysarthia(slurring speech) and dysphagia that leads to probs of swallowing, choking and aspiration belong to which clinical manifestation of MG?
Bulbar symptoms, due to the weakness of bulbar muscle
Except for respiratory weakness(shallow and decreased vital capacity, accessory muscles using, cough difficulty), What else is the clinical manifestations of MG
Bowel and bladder sphincter weakness
MG does NOT affect…
Sensory
What are the diagnosis of MG?
Physical exams;
Electromyography EMG;
Blood test;
CT/MRI;
Screening for other autoimmune disease
What is the function of Electromyography/EMG?
It detects the speed and strength of electrical signals sent from nerves to the muscles
Why are CT/MRI used for diagnosing MG?
Check any thymoma/thymic hyperplasia
To check for ACh antibodies and thyroid function in MG patient, what test is useful?
Blood test
What is the reason of myasthenic crisis?
Complication of myasthenia graves result from insufficient medication, emotional stress, trauma, infection or surgery
What are the s/s of Myasthenia crisis?
‑Sudden ↑BP and Pulse rate;
‑Cyanosis∵hypoxia
‑Respiratory distress
→intubation/
mechanical ventilation
‑Absent cough& gag reflex
‑Restlessness
‑Diaphoresis
‑↓urine output
‑Incontinence
(both bowel and bladder)
‑Dysarthria
(speaking difficulty)
What is the opposite condition of myasthenia crisis?
Cholinergic crisis, which is due to excessive medication given
For cholinergic crisis, what are the s/s related to muscle?
- Muscle twitching(esp. around mouth)
-Difficulty chewing, swallowing and speaking(muscle keep contracting)
-Advancing muscle weakness ~ 1hr after ANTIcholinesterase medication)
What are the other s/s for Cholinergic crisis (except for muscle-relating)
-nausea&vomiting
-diarrhea, cramp
-Increased secretion(e.g.salivary,
perspiration汗,lacrimal淚,bronchial)
-Headache
-Confusion
-Irritability, anxiety
-Syncope and respiratory distress–>respiratory arrest
What medications could improve MC and CC respectively?
MM: Edrophonium(Tensilon)[Temporary]
CC:Anticholinergics(Atropine)
How does MC and CC affect HR, BP, Pupil, secretion?
MC:↑HR,↑BP,Mydriasis
(dilation of pupil);
CC:↓HR,↓BP, miosis(constriction of pupil), ↑secretion
What is Tensilon Test?
IVI anticholinesterase agent edrophonium chloride(Tensilon)–> Differentiate MG to other conditions causing muscle weakness
+ve:Notable improvement in muscle strength(↓ptosis, better facial expression and enhance chewing/swallowing)
-ve: No improve
How long should the improvement occur for Tensilon Test?
within 30s
What is the nursing care of tensilon test?
Emergency:resuscitation trolley, with atropine–>counteract
What can atropine do for CC?
It can mitigate overactivity of cardiac muscarinic receptors
What are the treatment of MG?
Drugs: Anticholinesterase, corticosteriods
Surgical:Thymectomy
IV:Immunoglobulin G
Adjunctive: immunosuppressant(Cyclosporine),
plasmapheresis
What does anticholinesterase do?
1.Inhibit Ach breakdown
2.Increase synaptic Ach
3.Enhance neuromuscular transmission and restore muscle strength and endurance
What are the s/e of Anticholinesterase?
Excessive salivation sweating, nausea, diarrhoea, abdominal cramps, bradycardia, cholinergic crisis
What are Prostigmin (Neostigmine bromide) and Mestinon (Pyridostigmine)?
They are short-acting anticholinesterase
What can IV immunoglobulin G do on MG patient?
Decrease the antibody production
What is a plasmapheresis?
-An adjunctive therapy for patient with refractory(hard to cure) MG
-Remove plasma proteins containing antibodies
-3 to 5 treatments required over 2-3 months
What are the assessments for MG?
Severity of muscle weakness
ADL
Strength
Choking, gag reflex, speech
Respiratory distress: vital signs, accessory muscle, ABG, Lung function test
What could be the impacts of MG?
Resp: Altered breathing pattern[diaphragm and intercostal muscle weakness] and airway clearance
[intercostal weakness, impaired cough and gag reflex]
Muscle: Risk of injury[weakness of bulbar muscle, swallowing difficulty] and Altered activity tolerance[fatigue and muscle weakness]
Other:
Altered nutrition
[impaired swallowing],
↓self-esteem,
body image disturbance[eyelid/facial dropping]
What goals are needed to be achieved for MG patients?
-Prevent complications due to weakness of muscle
-Optimal status of muscle endurance
-manage fatigue
-Nutrition status(protein, maintained BW)
-Emotional and psychological needs are satisfied
For MG Patient, safety, choking and aspiration prevention includes (implementation):
1.Assess GAG Reflex
2.Observe choking, aspiration, nasal regurgitation
3. Head o bed elevated during meals for 30-60mins after eating
4.Thickening liquids
5.Small bites/soft diet
6.Oral hygiene
7.Mon Resp. status–>suction?
For MG Patient nutrition improvement includes (implementation):
1.Collab withDietitian, speech therapist
2.High-calorie snacks/supplements
3.Mon calorie &food intake,BW weekly, serum albumin lv
When should the anticholinesterase drugs be given to patient/taken by the patients themselves(education)
~45-60mins b4 meals
–>maximise effect for chewing and swallowing
Except for diet modification, what are the other patient education:
1.Avoid triggering factors e.g. sauna/hot tubs/sunbath, crowds, overheating, erratic(irregular) sleep habit, emotional extremes
2.Avoid aggravate hypoxia, hypercapnia(resp. distress)
3.Balace rest and activity, compensate with extra rest when needed
4.Carry a card stating MG
5.Aware s/s of MC
Key points of anticholinesterase:
1.45-60mins b4 meals
2.Adjust dosage regarding pattern of weakness
3.No OTC/prescribed med unless approval from doc
4.Toxicity/S/E–>CC
5.Contraindicated Med:
Laxatives, Antidysrhythmics, Beta-Blockers, antibiotics, Opioids
What is Systemic Lupus Erythematous (SLE)?
Chronic, inflammatory, autoimmune disease, affecting almost all body system.
The pathophysiology of SLE:
Immune system failed to differentiate self and non-self antigens, so antibodies act against self-antigens
1.gender; 2.age; 3.Colour
What is the prevalence of SLE?
1.10:1 (women to men)
2. Peak from 15 to 40
3.Black>White
What is known about the etiology of SLE?
Exact etiology is unknown
This highlights the complexity and uncertainty surrounding the causes of SLE.
Except for genetic factors, give some examples of the environmental factors that can influence the risk of SLE.
UV light, smoking, stress, Infectious agent (e.g. EBV)
List some drugs associated with an increased risk of SLE.
- Procainamide(treat arrhythmias)
- Hydralazine(anti-hypertensive)
- Isoniazid/INH(antibiotics for TB)
- Estrogen-containing oral contraceptive/hormonal replacement
What are the most common symptoms of SLE?
Fever, nonspecific fatigue, arthralgia (joint pain) and weight change
Which organs may be involved in SLE?
- Mucus membranes (ulcers)
- Kidney (proteinuria)
- Blood (anemia)
- Gut (Abdominal pain, HBP disease)
- Neurologic system (seizures or psychosis)
- Eye (uveitis retinopathy)
- Skin (rashes and photosensitivity)
- Cardiovascular disease
- Lupus nephritis –>ESRF(end stage renal failure)
uveitis–>uvea(middle layer of eye)
What type of skin symptoms are associated with SLE?
Rashes and photosensitivity
Photosensitivity refers to an increased sensitivity of the skin to sunlight.
What neurological symptoms may occur in SLE?
Seizures or psychosis
Neurological involvement can significantly impact the quality of life in SLE patients.
Fill in the blank: SLE cause____ retinopathy
uveitis=inflammtion of uvea, middle layers of eye (including iris, ciliary muscle and choriod)
What is the characteristic skin manifestation of SLE?
A red butterfly rash(diffuse maculopapular) across the cheeks and bridge of the nose
This rash is often photosensitive.
What is a discoid lesion?(SLE manifestation)
Raised, scaly, circular lesion with an erythematous rim
https://www.everydayhealth.com/lupus/how-to-identify-lupus-rash-and-othe
Except butterfly rash and discoid lesion
What other skin manifestation can be obersved for a SLE patient?
Hives 蕁痲疹
Hives can appear as raised, itchy welts on the skin.
What other manifestation can be obersved on the fingers of a SLE patient?
Erythematous fingertip lesion
This type of lesion is characterized by redness and inflammation at the fingertips. With skin breakdown
What condition can be observed on the lips/mouth/nose of a SLE patient?
Painless mucous membrane ulceration on the lips or in the mouth or nose
What do we call the manifestation of blood clots running vertically under the nails of a SLE paitent?
Splinter haemorrhage
Psychological and physical:
What should we ask about the SLE patient from the patient & the family members
Any behavioural changes, signs of neurosis, psychosis and depression; any seizures/ other CNS manifestations
What should we assess and investigate for SLE patient reagrding to his/her health?
Systemic and multiorgan manifestation
What is the purpose of laboratory studies in this context?
Confirm or rule out other physical problems and evaluate the severity of the disease and organs involved.
What does a positive Antinuclear Antibody (ANA) indicate?
Found in clients with certain types of autoimmune diseases, positive in more than 97% clients with SLE.
Is the Antinuclear Antibody (ANA) test specific?
No, it is not specific and may also be positive in RA, scleroderma(hard skin), carcinoma, tuberculosis, and hepatitis.
Which antibody is better for confirming SLE?
Anti-DNA antibody, as it is rarely found in any other disorder
What do elevated Erythrocyte Sedimentation Rate (ESR) and C-reactive protein (CRP) indicate?
They indicate chronic inflammation.
What abnormalities might be found in a Complete Blood Count (CBC)?
Moderate to severe anaemia, leukopenia, lymphocytopenia, and possible thrombocytopenia.
What findings might be observed in a urinalysis during exacerbation?
Mild proteinuria, haematuria, and blood cell casts(cylindric 圓柱urinary particles).
What tes is used for indicating increase in serum creatinine, blood urea nitrogen (BUN), and eGFR. And what does the result suggest?
Renal Function Test.
It suggests the extent of renal disease.
What is the purpose of a kidney biopsy?
To assess the severity of renal lesions and guide therapy.
What are the 11 distinct elements of SLE suggested by American College of Rheumatology ACR? Name 4 of them
- Antinuclear antibody:abnormal titer
2.Discoid rash:
raised patches with scaling follicular plugging; scarring in older lesions
3.Hematologic disorder: hemolytic anemia, leukopenia, lymphopenia, or thrombocytopenia
- Immunologic disorder: anti-DNA antibody or antibody to Sm(Smith) nuclear antigen o rpositive antiphospholipid antibodies
5.Malar rash: fixed erythema, flat or raised(butterflyrash) - Oral ulcers: usually painless
7.Nonerosive arthritis: 2 or more peripheral joints with tenderness, swelling, effusion
8.CVS: Pleuritis or pericarditis
- Neurologic disorder: seizures or psychosis(in the absence of causative drugs or known metabolic disorders)
- Photosensitivity: skin rash as unusual reaction to light
- Renal disorder: persistent proteinuria or cellular casts in urine
Diagnosis of SLE, if 4 of the 11 criteria are present
As there is no cure for SLE, our goals include:
- Preventing progressive loss of organ function
- Treat flare up
- Minimizing disease-related disabilities
- Preventing complication from therapy
What is a flare? (SLE)
Warning Signs of a Flare
* Increase fatigue
* Pain, abdominal discomfort
* Rash
* Headache
* Fever
* Dizziness
Why NSAIDS and corticosteriods are given to SLE pt?
Reduce inflammation
NSAIDS can also reduce pain
To treat fatigue, moderate skin and join problems, what should be administered? (SLE)
Antimalarial agents
e.g. hydroxychloroquine (Plaquenil)
SLE is an autoimmune disease
For severe form of SLE that have not responded to conservative therapies, what should be administered?
Immunosuppressive agents
What are the nursing management during disease flare?
-Assess fever pattern and vital signs, joint, degree of discomfort/fatigue
-Monitor laboratory values
-Monitor for potential adverse effects of medications(e.g.bleeding)
- Collect 24-hour urine samples 廿四味for protein and creatinine clearance
-Monitor the patient’s weight and fluid intake and output–>fluid retention?
What are the education for SLE pt/family?
- s/s of SLE and flare up
- progression of SLE
- Lifestyle change
- Good lifestyle
- Provide support
- Skin protection(sunblock/avoid sunlight, long sleeves, moisturizers, inspect skin)
How could SLE affect pregnancy?
*Infertility
∵renal/corticosteroid(high dose)
- ↑stillbirth, intrauterine growth retardation, spontaneous abortion
∵immune complexes deposit at placenta/Inflammatory responses in placenta blood vessels
What should SLE patient to be aware for pregnancy?
Plan pregnancy when disease activity is minimal, understand exacerbation is common during postpartum產後 period
