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Systemic Diseases of Systemic Diseases of Connective Tissue Connective Tissue
M.ValešováM.Valešová
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Systemic Diseases of Connective Systemic Diseases of Connective TissueTissue
• The term „connective tissue diseases“ The term „connective tissue diseases“ describe a group of conditions characterised describe a group of conditions characterised by the occurrence of:by the occurrence of:
• vasculitisvasculitis
• multisystem involvementmultisystem involvement
• arthritis or artralgiaarthritis or artralgia
• abnormal immunological featuresabnormal immunological features
• (autoantibodies, immune complexes (autoantibodies, immune complexes depositiondeposition))
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Systemic Diseases of Connective Systemic Diseases of Connective TissueTissue
• Systemic lupus erythematosus (SLE)Systemic lupus erythematosus (SLE)
• Rheumatoid arthritisRheumatoid arthritis
• SjSjőőgren´s syndromagren´s syndroma
• Systemic sclerosisSystemic sclerosis
• Dermatomyositis, MyositisDermatomyositis, Myositis
• VasculitisVasculitis
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Systemic Diseases of Connective Systemic Diseases of Connective TissueTissue
The aetiology is unknownThe aetiology is unknown
• Environmetal factorsEnvironmetal factors
- exposure to sunlight- exposure to sunlight
- viral infections- viral infections
- drugs - lupus-like syndroma- drugs - lupus-like syndroma
(hydrallazine, isoniazid, (hydrallazine, isoniazid, contraceptives)contraceptives)
• Genetics-markers DR2 and DR3Genetics-markers DR2 and DR3
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Systemic Diseases of Systemic Diseases of Connective TissueConnective TissueConnective tissue iscomposed essentially Connective tissue iscomposed essentially
of collagen,elastic fibres and fibroblasts of collagen,elastic fibres and fibroblasts in anamorphous ground substancein anamorphous ground substance
Common features of this diseases:Common features of this diseases:
• Vascular pathology and fibrinoid Vascular pathology and fibrinoid changes in the ground substancechanges in the ground substance
• Many clinical features in common, Many clinical features in common, difficulty to make a diagnosisdifficulty to make a diagnosis
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Systemic Diseases of Systemic Diseases of Connective TissueConnective Tissue
• Deposition of immune complex in Deposition of immune complex in tissue results in:tissue results in:
- glomerulonefritisglomerulonefritis- in glomerular - in glomerular basementbasement
membranemembrane
- vasculitisvasculitis – in blood vessels – in blood vessels
- arthritisarthritis – in joint synovium – in joint synovium
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Systemic Diseases of Systemic Diseases of Connective TissueConnective TissueComplement activation results in:Complement activation results in:
• Depresion of circulating complementDepresion of circulating complement
• Activation of coagulation system and Activation of coagulation system and depression of circulating plateled levelsdepression of circulating plateled levels
• Chemotaxis of PMN(polymorphonuclear Chemotaxis of PMN(polymorphonuclear leukocytese)leukocytese)
• Release of vasoactive Release of vasoactive substances(histaminesubstances(histamine))
• CytolysisCytolysis
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Systemic lupus erythematosus Systemic lupus erythematosus (SLE)(SLE)
SLE SLE is a complex multi-system autoimmune diseaseis a complex multi-system autoimmune disease
Involved organs:Involved organs:
• SkinSkin
• HeartHeart
• VesselsVessels
• KidneyKidney
• CNSCNS
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Systemic lupus erythematosus Systemic lupus erythematosus (SLE)(SLE)
• The primary pathology is that of The primary pathology is that of multisystem inflammatory process, multisystem inflammatory process, probably secondary to probably secondary to
antigen-antibody reactionsantigen-antibody reactions
• Hyperactivity of B lymphocytes-Hyperactivity of B lymphocytes-production of antibodies againts nuclear production of antibodies againts nuclear antigens.: ANA, anti-ds DNA, anti-Smantigens.: ANA, anti-ds DNA, anti-Sm
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SLE-AetiologySLE-Aetiology
• UnknownUnknown• Aetiological role of enviromental Aetiological role of enviromental
factors,such as -exposure to sunlightfactors,such as -exposure to sunlight• Viral infection (EBV, CMV, retroviruses)Viral infection (EBV, CMV, retroviruses)• Increased concentration of prolactin Increased concentration of prolactin andand
estrogensestrogens• Some drugs produces lupus-like Some drugs produces lupus-like
syndrome (hydrallazin,isoniazid,oral syndrome (hydrallazin,isoniazid,oral contraceptives)contraceptives)
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SLE -pathogenesesSLE -pathogeneses
• The primary pathology is that of The primary pathology is that of multisystem inflammatory process multisystem inflammatory process probably secondary to antigen-antibody probably secondary to antigen-antibody reaction,autoimmunityreaction,autoimmunity
• vasculitis and vasculopathyvasculitis and vasculopathy
• Almost all patients possess an antibody Almost all patients possess an antibody against nuclear antigens(ANA)-nonspecific against nuclear antigens(ANA)-nonspecific marker,antibodies anti –dsDNA,antiSm-marker,antibodies anti –dsDNA,antiSm-more specific for SLEmore specific for SLE
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Systemic lupus erythematosus Systemic lupus erythematosus (SLE) (SLE) - - clinical findingsclinical findings
The most constant features:The most constant features:
• Fever,Fever,
• Arthritis or arthralgiaArthritis or arthralgia
• Skin rashesSkin rashes
• PolyserositisPolyserositis
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Systemic lupus erythematosus Systemic lupus erythematosus (SLE)- clinical findings(SLE)- clinical findings
• Constitutional syptoms-Constitutional syptoms-anorexia,tiredness,fever,weight lossanorexia,tiredness,fever,weight loss
• ManyMany organ systems may be organ systems may be involvedinvolved
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Systemic lupus erythematosus Systemic lupus erythematosus (SLE)(SLE)
Skin involvementSkin involvement
• rash rash – erythematosus lessions– erythematosus lessions
- discoid lessions- discoid lessions
• photosensitivityphotosensitivity
• urticaurtica
• various exanthemasvarious exanthemas
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Systemic lupus erythematosus Systemic lupus erythematosus Skin involvementSkin involvement
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Systemic lupus erythematosus Systemic lupus erythematosus Skin involvementSkin involvement
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Systemic lupus erythematosus Systemic lupus erythematosus Skin involvementSkin involvement ( (80%80% patients)patients)
Rash may occur in Rash may occur in sun exposed areassun exposed areas
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Systemic lupus erythematosus Systemic lupus erythematosus (SLE)(SLE)
Heart involvement Heart involvement • Endo,myo,peri carditisEndo,myo,peri carditis
• Pericarditis with efusion- ECHOPericarditis with efusion- ECHO
• Vasculopathy of coronary arterias ( atherosclerosis)Vasculopathy of coronary arterias ( atherosclerosis)
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Systemic lupus erythematosus Systemic lupus erythematosus (SLE)(SLE)
• PleuriPleurisysy ( (effusioneffusion))
• AcuteAcute pneumonitid pneumonitidis is –– vasvascculitiulitis of s of lung lung
vesselsvessels
• Pulmonary infarctsPulmonary infarcts
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Systemic lupus erythematosus Systemic lupus erythematosus (SLE)(SLE)
Central nervous system involvement.Central nervous system involvement.Increasingly important manifestation develops later Increasingly important manifestation develops later
than renal diseasethan renal disease
• SeizuresSeizures
• Focal neurologic signsFocal neurologic signs
• Organic brain syndromeOrganic brain syndrome
• Non organic mental disordersNon organic mental disorders
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Systemic lupus erythematosus Systemic lupus erythematosus (SLE)(SLE)
Renal involvement :Renal involvement :
glomerulonefritisglomerulonefritis• Mesangial typeMesangial type-mesangial deposition of immune -mesangial deposition of immune
complexescomplexes
• Focal proliferativeFocal proliferative type –local thickeningtype –local thickening
• Diffuse proliferative type Diffuse proliferative type
thickening of glomerular basement membrane,thickening of glomerular basement membrane,
masive deposition of immunocomplexes masive deposition of immunocomplexes subepithelialysubepithelialy
• Membranous typeMembranous type
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Systemic lupus erythematosus Systemic lupus erythematosus (SLE)(SLE)
Renal involvementRenal involvement• Proteinurie, miProteinurie, miccrohematurierohematurie
• NefroticNefrotic syndromsyndromaa
• Renal hypertensionRenal hypertension
• Renal insuficiencyRenal insuficiency
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Systemic lupus erythematosus Systemic lupus erythematosus (SLE)(SLE)
Joint involvementJoint involvement
•ArthralgiasArthralgias
•Arthritis- may resemble RAArthritis- may resemble RA
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Systemic lupus Systemic lupus erythematosus(SLE)erythematosus(SLE)
Blood involvement
•Hemolytic anemia
•Leukopenia or lymphopenia
•Trombocytopenia
•Lymfadenopthy
•Trombotic tendency
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Systemic lupus Systemic lupus erythematosus(SLE)erythematosus(SLE)
Blood involvement
Trombotic tendency are more marked in patients with positive
•Anticardiolipin antibodies ACLA
•Anti-phospholipid antibodies
•„Lupus anticoagulans“
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Systemic lupus Systemic lupus erythematosus(SLE)erythematosus(SLE)Investigations: increased : ESR,CRP immunoglobulinspositivity : ANA ( 90% pts) anti ds- DNA sublclasses of ANA- anti - Ro anti - Sm
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Systemic lupus Systemic lupus erythematosus(SLE)erythematosus(SLE)
Theraphy:
• Mild disease – skin, joints
´ – hydroxychloroqine (Plaquenil)
• Active disease
– systemic steroids
- immunosupressive agents
(Methotrexate, cyclophosphamide
azathioprim,
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Systemic Systemic Sclerosis Sclerosis (S(SScSc))
Uncommon idiophatic multisystem disease
Predominantly affects the skin and blood vessels
Female preponderance 3:1
Main pathological feature-progressive fibrosis and atrophy
2 types – diffuse
localised
Typhical autoantibody
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Systemic Systemic Sclerosis Sclerosis (S(SScSc))
• The skin is thickned The skin is thickned and tight and this and tight and this results in contracturesresults in contractures
• Raynaud´s Raynaud´s phenomenon in 95% phenomenon in 95% ptspts
• Organs involvement- Organs involvement- GIT, lung,kidneyGIT, lung,kidney
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Systemic Systemic Sclerosis Sclerosis (S(SScSc))
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Systemic Systemic Sclerosis Sclerosis (S(SScSc))
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DDermatomyoermatomyositis andsitis and PolymyoiPolymyoisitssits
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DDermatomyoermatomyositis andsitis and PolymyoiPolymyoisitssits
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SjSjőőgren´s syndromgren´s syndromee
• Dryness of the eyes(sicca syndrome)Dryness of the eyes(sicca syndrome)
• Dryness of the mouth (xerostomia)Dryness of the mouth (xerostomia)
Caused by chronic dysfunction of the Caused by chronic dysfunction of the exocrine glandsexocrine glands
It may occur alone or in assotiation with RA It may occur alone or in assotiation with RA or other connective tissue disordersor other connective tissue disorders