The lack of response to the body’s own cells is known as tolerance
autoimmunity occurs when individuals develop antibodies called autoantibodies to their own tissues or self antigens
1.1 Lupus 狼疮
- chronic, affect skin, joints, heart, lungs, blood, kidneys, brain
- risk factor: 90% woman, 15~44
- types: systematic, cutaneous, drug-induced, neonatal
- systematic lupus erythematosus (SLE) 系统性红斑狼疮:
- 70%
- 1/2 SLE develop severe heart lungs kidneys brain diseases
- signs & symptoms: fatigue, arthritis, fever, butterfly rash across cheeks and nose, photosensitivity, mouth or nose ulcers, fingers turn white/blue when cold (Secondary Raynaud’s phenomenon)
- Cutaneous or discoid lupus 皮肤或盘状狼疮:
- 10%
- only affect skin
- signs & symptoms: discoid rash 盘状体皮疹: raised, scaly, red rash, but not itchy, circular; butterfly rash across cheeks and nose; rash on face neck scalp mouth nose vagina
- 10% of cutaneous lupus will develop SLE
- drug-induced lupus erythematosus (DILE) 药物引起的狼疮:
- 10%
- 70+ prescription drugs: hydralazine (for high blood pressure), procainamide and quinidine (for irregular heart rythm), certain drugs entail a high risk
- signs & symptoms: similar to SLE, but rarely affects organs, will disappear within days to months after discontinuing
- Neonatal Lupus 新生儿红斑狼疮:
- acquired from maternal autoantibodies, affect skin heart blood
- signs & symptoms: rash within several weeks of life, persists about 6 months
- etiology: idiopathic, environmental (infections, sulfa antibiotics, penicillin, UV, stress, certain drugs, hormones(ss grow severe before menstrual periods or during pregnancy, estrogen may)) and genetic may
- diagnosis:
- no testing will confirm
- CBC, urinalysis, autoantibody testing, general level of inflammation, biopsy
- characters:
- chronic, relapsing, often with symptom-free remissions that can last for years
- incurable, challenging management
- most patients live a normal lifespan
- patient care:
- limit exposure to sunlight, tobacco, stress and fatigue
- health diet, exercise, take care of fevers over 100 (37.8)
- treatment:
- for control
- NSAIDs, corticosteroids, antimalarial medications, immunosuppressive drugs, monoclonal antibodies to inhibit autoantibody and antibody production
1.2 Scleroderma 硬皮病
- chronic disease of connective tissue
- risk: women, 40s
- localized form (LSc): affects only the skin
- signs & symptoms: waxy patches (morphea 硬斑) or streaks on the skin
- tends to regress or stop progressing without treatment
- normal lifespan
- systemic form (SSc): involves internal organs and the skin
- limited cutaneous SSc (lcSSc)
- skin tightening confined to fingers, hands, arms below the elbows; may involve feet and legs below the knees
- 10yr survival rate: 71%
- diffuse cutaneous SSc (dcSSc)
- skin tightening lcSSc+trunk, above the elbows, on the knees
- 10yr survival rate: 21%
- both affect internal organs, but dcSSc are at a greater risk for significant organ dysfunction
- limited cutaneous SSc (lcSSc)
- common initial ss: secondary raynaud’s, thickening & tightening of the skin of the fingers, pain in 2+ joints
- early symptoms: heartburn, difficulty swallowing, shortness of breath
- etiology: idiopathic
- immune: stimulate fibroblasts to produce too much collagen
- environmental & genetic: exposure to viral infections & chemicals to who are genetically predisposed
- hormonal: women 4x (estrogen)
- diagnosis:
- history, physical exam
- autoantibody testing and biopsy
- treatment: no cure
- no tobacco, bo cold & stress
- exercise
- small frequent meals
- moisture skin
- cannot be prevented
- control: anti-inflammatory drugs, immunosuppressive drugs, vasodilators to treat raynaud’s
1.3 Sjogren’s Syndrome 干燥综合征
- chronic, slow progressive, affects the exocrine glands
- risk factor: 90% women
- primary Sjogren’s syndrome
- secondary Sjogren’s syndrome: occur along with rheumatoid arthritis, lupus, scleroderma
- signs & symptoms:
- dry eyes and mouth
- complications in kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas, and CNS
- etiology: idiopathic
- environmental: viral infections
- genetic: first-degree relative with autoimmunity disease
- hormone: women during childbearing years
- diagnosis:
- treatment: