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GP2 CH6 Autoimmune and rheumatic diseases 自身免疫性疾病和風濕性疾病 (Overview…

- - - - Most common persistent inflammatory arthritis關節炎
      - 40％的患者在發病後2年內就診
      - Genetic, epigenetic and environmental factors involved
      - Periodontal disease and oral pathogens have been implicated涉及
    - - Infiltration of the synovial membrane浸潤滑膜 with lymphocytes and other cells of the immune system.
      - New blood vessel formation that will perpetuate永久化 the inflammatory process
      - Inflammatory granulation tissue formed spreads under articular cartilague關節軟骨 (eroded侵蝕 and destroyed)
      - Maturation of osteoclasts破骨細胞 erodes bony estructures
      - Fibrous or bony ankylosis骨性強直 may occur
      - Muscles adjacent to inflamed joints atrophy鄰近發炎關節的肌肉萎縮
      - All leads to progressive biochemical dysfunction and may further amply destruction
      - Rheumatic nodules
        
        consist of a central area of fibrinoid material 纖維蛋白
        
        surrounded by a palisade of proliferating mononuclear cells周圍有增殖的單核細胞柵欄
    - - Presentation
        
        stiffness affecting small joints of the hands, feet and wrist
        
        joint swelling
        
        pain
        
        Large joint involvement
        
        systemic symptom(Rheumatoid arthritis is a systemic disease )
        
        extra-articular關節外 features
    - - Temporo-mandibular joint disturbances
      - Periodontal disease(related)
      - Secondary Sjögren’s disease (xerostomia口腔乾燥症)
    - - Early use of small-molecule disease-modifying
        antirheumatic drugs (DMARDs)
      - Corticosteroids皮質類固醇
- - - - unknown cause
      - lymphocytic infiltration of salivary and lachrimal glands淚腺
      - Age of onset between 40 and 50
      - 9:1 female preponderence
    - - primary Sjögren ́s syndrome:when disease is isolation
      - Secondary Sjögren ́s syndrome: When disease with other autoimmune diseases
    - - Keratoconjunctivitis sicca乾燥性角膜結膜炎 (lack of lubricating tears)
      - Xerostomia口腔乾燥症
    - - Arthralgias關節痛, chronic fatigue and MALT lymphoma淋巴瘤
      - Xerostomia
        
        • Salivary gland swelling
        
        • Reduced taste
        
        • Difficult swallowing & speech
        
        • Poor denture retention
        
        • Malodor惡臭
        
        • Dry, sticky mucosa
        
        • Angular cheilitis
        
        • Dry flaky乾燥的片狀 lips
        
        • Longer clearance times
        
        • Caries
        
        • Mucosal atrophy
        
        • Tongue fissuring
        
        • Candidiasis
        
        • Persistence of debris殘留物殘留
      - Dryness in mucosal surfaces
    - - Schirmer tear test
        
        Staining with rose Bengal....show punctate epithelial abnormalities顯示點狀上皮異常
        
        Not clear, confirm with lip biopsy
        
        Absorbent paper strips in
        lower eyelid
    - - – Symptomatic
      - – Lacrhymal substitutes淚液替代品, soft contact lenses
      - – Artificial saliva and oral gels人造唾液和口服凝膠, sugarfree chewing gum
      - – Adequate postpandrial oral hygiene
- - - - Disorders of the musculoskeletal system
      - affecting
        
        connective tissue
        
        muscles
        
        joints
        
        bones
- - - - Formation of soluble immune complexes
      - involving autoantibodies against antigens present within the cell or within the nucleus
      - Theory of defects in apoptosis
    - - Butterfly rash(malar rash)
      - Discoid rash盤狀皮疹
      - Photosensitivity : reaction of sunlight
      - Oral ulcers
      - Arthritis關節炎
      - Serositis漿膜炎
      - Renal disorder
      - Neurologic disorder
      - Hematologic disorder
      - Immunologic disorder
      - Antinuclear antibody
    - - Raynaud ́s phenomenon
      - Rash is common and is classically precipitated by
        exposure to UV light
        
        classic butterfly facial rash
        
        skin
        
        alopecia脫髮
        
        Discoid lupus rash
        
        hyperkeratosis
        
        follicular plugging濾泡堵塞
        
        Subacute cutaneous lupus erythematosus rash亞急性皮膚紅斑狼瘡皮疹
        
        migratory遷移
        
        non scarring 非瘢痕
      - Arthritis, joint deformities
      - involved
        
        kidney
        
        cardiovascular
        
        lung
        
        neurological
        
        haematological血液
        
        gastroinstetinal
    - - incompletely understood
      - Genetic factors play important role
    - - – Malar rash (butterfly rash)
      - – Oral and nasopharyngeal ulcers
      - – Erythematous areas
      - – Leukoplakicplaques
      - – Cheilitis唇炎
      - – ”Honeycomb” plaques
      - – Secondary Sjögren’s disease
      - – Discoid lesions盤狀病變
    - - – Educate the patient about the nature of the illness
      - – Control symptoms
      - – Prevent organ damage
      - – Patient should be advised to avoid sun and UV light exposure, and to employ sun blocks
- - - - Generalised disorder of connective tissue affecting
        
        internal organs
        
        vasculature
        
        skin
      - combination with Raynaud ́s phenomenon and digital ischaemia.
      - variety of forms and severities
      - Cause
        
        genetic component
        
        STAT4
        
        CD19
        
        II-1B
        
        CTGF
        
        HLA
        
        SPARC
        
        Fibirlin
        
        environment
        
        chemicals
        
        Infection
      - T lymphocytes infiltrate the skin, increased production of extracelular matrix in the dermis
    - - Symmetrical thickening, tightening and induration of the skin硬化皮膚
      - Arterial and arteriolar narrowing動脈和小動脈狹窄
    - - Raynaud ́s phenomenon
        
        small blood vessels in the extremities may cause critical tissue ischaemia
      - Musculoskeletal features
        
        Artralgia神經痛
        
        morning stiffness
        
        flexor tenosynovitis屈肌腱鞘炎
        
        restricted hand function
        
        muscle weakness
      - Skin
        
        edema of fingers and flexor tendon sheaths屈肌腱鞘
        
        Shiny and distal skin creases disappear皺褶消失
        
        Thinning of the lips and radial furrowing橈骨溝皺紋
        
        Face and neck usually involved next
        
        Limit disease
        
        only sites distal to the elbow or knee
        
        apart from face
        
        Diffuse disease
        
        5 years survival of 70%
        
        involvement proximal to the knee and elbow and on the trunk
      - Gastrointestinal, Pulmonary and renal involvement
    - - No treatments are available
      - Ameliorate改善 the effects of the disease on target organs.
    - - – Facial mask-like changes
      - – Reduction of oral opening (microstomia)
      - – Trismus牙關緊閉
      - – Telangiectasia毛細血管擴張
      - – Mandibular resorption
      - – Periodontal ligament widened on x-ray
      - – Xerostomia (secondary Sjögren’s syndrome)
      - – Stiffnessoflips,tongue,etc...
- - - - acute attack should be treated
        
        oral NSAIDs
        
        rest
        
        analgesics鎮痛藥
      - Intra-articular steroids關節內類固醇 may be required in patients with severe sinovytis滑膜炎
    - - Considerable systemic disturbance相當大的全身性紊亂, with fever and weight loss
      - Lower limb joints
      - First atack of arthitis is usually self-limiting
      - onset is typically acute, with an inflammatory oligoarthritis炎性少關節炎
      - Most common cause of inflammatory arthritis
      - recurrent復發 or chronic arthritis develops in more than 60% of patients
      - Symtoms
        
        conjunctivitis
        
        Urethritis尿道炎
        
        Mouth ulcers
        
        Achillobursitis 跟腱附近的滑囊炎症
- - - - Pain and swelling affecting the joints and entheses
      - generally one of intermittent exacerbation followed by varying periods of complete or near-complete remission通常是間歇性惡化，然後是不同時期的完全緩解或接近完全緩解
      - Occurs in 7-20% of patients with psoriasis牛皮癬 and in
        up to 0.6% of the general population
- - - - Can progress to bony fusion of the spine
      - Interaction between environmental pathogens and the host inmune system in genetically susceptible individuals
      - Chronic inflammatory arthritis predominantly
        affecting the sacroiliac joints骶髂關節 and spine
    - - low back pain and early morning stiffness with radiation to the buttocks or posterior thighs
      - Tends to ascend slowly, ultimately involving the whole spine
      - Chest pain, plantar fasciitis足底筋膜炎, Achilles tendinitis腱炎
      - spine becomes increasingly rigid as ankylosis occurs
    - - Maintain a maximal range of skeletal mobility
      - avoid the development of deformities避免畸形的發生
      - Relieve pain and stiffness
      - Education and appropriate physical activity
- - - - Claudication
      - fever
      - aethalgia
      - weight loss
    - - pulmonary arteries
      - major branches
- - - - folliculitis毛囊炎
      - erythema nodosum結節性紅斑
      - uveitis葡萄膜炎
      - genital ulcerations
      - neurological involvement神經系統受累
      - arthropathies關節病
      - acneiform eruptions痤瘡樣疹
      - gastrointestinal involvement.
      - aneurysm formation 動脈瘤形
    - - Minor aphthous ulceration
        
        <10 mm
        
        Clusters <10 ulcers
        
        Heal without scar in 7-14 days
      - Major aphthous ulceration
        
        10 mm
        
        Clusters 2-3 ulcers
        
        May cause necrosis & scar
      - most frequent and often initial presenting sign
      - Herpetiform aphthous ulceration皰疹性口瘡性潰瘍
        
        Multiple 1-2 mm ulcers that tend to coalesce凝聚
        
        Scarring is unusual 疤痕很不尋常
- - - - particularly the extracranial branches of the carotid artery
      - most prevalent vasculitis in >50 years old patients in western world.
      - Systemic vasculitis affecting large vessels
    - - depression
      - weight loss
      - malaise全身乏力
      - fever
      - Frequent association with polymyalgia rheumatica風濕性多肌痛
    - - Pain on mastication (jaw claudication); due to ischemia of the masticatory muscles咀嚼肌缺血
      - Symtoms
        
        numbness of chain
        
        inflammation and enlargement of the tongue
        
        facial swelling
        
        submandibular masses
        
        dysphagia吞嚥困難
        
        dysarthria構音障礙
        
        dental pain
        
        rarely necrosis of the lip and tongue.
        
        Trismus牙關緊閉
- - - - Lingual necrosis
      - Labial swelling
      - Osteonecrosis骨壞死 and mucosal ulceration due to progressive destruction of nasal cartilage and septum
      - Mucosal ulceration
      - “Strawberry-like” appearance of the gingiva
        
        reddened
        
        swollen
        
        granular gingiva
    - - involving the respiratory system and kidney, lungs
        
        90% manifestations in the upper respiratory system
      - Necrotizing granulomatous inflammation affecting small to medium- sized vessels
    - - granulomatous sinusitis肉芽腫性鼻竇炎
      - necrotizing glomerulonephritis壞死性腎小球腎炎
      - nasal discharge鼻涕
    - - Autoimmune disease that causes inflammation of blood vessels(vasculitis)
- - - - Acute systemic disorder
      - Mostly affects the coronary vessels
      - Japan and other Asian countries
      - usually affecting children under 5 years
    - - erythema and desquamation of hands and feet手足紅斑和脫屑
      - bilateral conjunctivitis雙側結膜炎
      - polymorphous rash多形性皮疹
      - ervical lymphadenopathy頸淋巴結腫大
      - fever
    - - Strawberry-like” appearance of the tongue
        
        Erythema
        
        prominent lingual papillae突出的舌乳頭
      - Inflamed or cracked lips
      - Erythema of the oropharynx口咽的紅斑
- - - - painless
      - firm to the touch, with normal overlying mucous membrane.
      - diffuse enlargement at the submucous level or a nodular lesion粘膜下層瀰漫性腫大或結節性病變