Connective tissue disorders Systemic lupus erythematosus
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Transcript of Connective tissue disorders Systemic lupus erythematosus
Connective tissue disordersSystemic lupus erythematosus
Želmíra MacejováŽelmíra Macejová
Systemic lupus erythematosus
autoimmune, organ non specific diseasehyperreaction of B-lymphocyts with production of antibodies agains organ non-specific antibodiesmultisystem diseaseunknown etiologyvariable course and prognosis
Clinical features
Inflammation in various organ systems including skin, mucose membrane, joints, kidney, brain, serous membrane, lung, heart, gastrontestinal tract.
Involvement of organ- single or in combinationMortality: involvement of kidney and CNS General clinical features - fatigue, fever, malaise, weight loss
Ethiology
unknown, multifactorialunknown, multifactorial exogenousexogenous: virus infection: virus infection UV lightUV light stressstress drugs: hydralazindrugs: hydralazin endogenousendogenous: hormonal : hormonal genetic predisposition genetic predisposition immune reaction: immune reaction: hyperreactivity of B- cellshyperreactivity of B- cells
pathologic T cell , failure of remove immune complexespathologic T cell , failure of remove immune complexes
Clinical Features- general
Subfebrility, fever- 80%Subfebrility, fever- 80% Malaise, tirednessMalaise, tiredness SkinSkin Mental disorders, psychiatric manifestationMental disorders, psychiatric manifestation arthralgia, arthritisarthralgia, arthritis thrombosisthrombosis Changes of blood countChanges of blood count
The joint
Arthritis, arthralgiaArthritis, arthralgia – morning stiffness – morning stiffness stuhnutosťstuhnutosť
Involvement: small joints of hand, wrist, Involvement: small joints of hand, wrist, knees, knees,
arthritis- is not erosivearthritis- is not erosive MyositisMyositis- pain of involved muscles- pain of involved muscles
Skin manifestation
PhotosenzitivityPhotosenzitivity Butterfly erythemaButterfly erythema
Vaskulitic lesions: Vaskulitic lesions: fingertips, around nails, fingertips, around nails, purpura, urticariapurpura, urticaria
Raynaud s phenomenon, Raynaud s phenomenon, Livedo reticularisLivedo reticularis Palmar, plantar rashesPalmar, plantar rashes
Hematologic changes
Anaemia Anaemia Neutropenia, lymfopenia, trombocytopéniaNeutropenia, lymfopenia, trombocytopénia ESR – raisedESR – raised CRP normalCRP normal ANA : positiv: dsDNA, DNP, RF, serum ANA : positiv: dsDNA, DNP, RF, serum
complement- redused, immunoglobulin complement- redused, immunoglobulin IgG, IgM - raised IgG, IgM - raised
The kidney Lupus nephritisLupus nephritis – 30-50% – 30-50% cause of deathcause of death immune complex deposits in the kidneyimmune complex deposits in the kidney hypertensionhypertension nephrotic syndromenephrotic syndrome renal failurerenal failure
Lungs
Recurent pleurisyRecurent pleurisy Pleural effusionsPleural effusions Pulmonary fibrosisPulmonary fibrosis Pulmonary hypertensionPulmonary hypertension
Serositis PleuritPleuritisis - - 30-60% 30-60% patientspatients - - clinicaly asymptomaticclinicaly asymptomatic - - more often in drug induced SLEmore often in drug induced SLE PeriPericcarditarditisis - 60% pa - 60% patientstients - - clinicaly asymptomaticclinicaly asymptomatic DifDiffusefuse peritonitperitonitisis with little amount of with little amount of
fluidfluid - clinicaly asymptomatic- clinicaly asymptomatic
The heart
- 40%- 40% PeriPericcarditarditisis MyoMyoccarditarditisis - -
ararrrytythghgmimiaa,, c cardiomegardiomegalyaly, tach, tachyykkaardiardia EndoEndoccarditarditisis - abakteriálna Libman-Sacks- abakteriálna Libman-Sacks
The gastrointestinal system
VasVascculitulitisis of art. mesentericaof art. mesenterica::
antiphospholipid syndrantiphospholipid syndromeome Lupus enteritLupus enteritisis PanPanccreatitreatitisis AutoimunAutoimunee hepatit hepatitisis
Symptoms: nausea, vomiting, anorexia, Symptoms: nausea, vomiting, anorexia, diarrhoediarrhoe
The nervous system- neuropsychiatric lupus
N-P lupus : 60-70% N-P lupus : 60-70% Main cause of deathMain cause of death Mild depressionMild depression Severe psychiatric disturbancesSevere psychiatric disturbances EpilepsyEpilepsy Cerebellar ataxiaCerebellar ataxia Aseptic meningitisAseptic meningitis Peripheral neuropathyPeripheral neuropathy Cause: vasculitis, immune-complex depositionCause: vasculitis, immune-complex deposition
Diagnostic criteria for SLE
Diagnostic criteria SLE 1. 1. Facial skin erythemaFacial skin erythema 2. Dis2. Disccoid erytoid erythemahema 3. Fotosenzitivit3. Fotosenzitivityy 4. 4. Mouth ulcersMouth ulcers At least 4!!At least 4!! 5. Arthralgia, arthritis5. Arthralgia, arthritis 6. Serozitis6. Serozitis 7. 7. Involvement of kidneyInvolvement of kidney 8. Neurologic 8. Neurologic manifestationmanifestation 9. Hematologic 9. Hematologic manifestationmanifestation 10. Imunologic 10. Imunologic manifestationmanifestation 11. Antinukle11. Antinuklearar antibodiesantibodies
Treatment
NSANSA corticosteroidscorticosteroids Antimalarial drugs - hydroxychloroquineAntimalarial drugs - hydroxychloroquine AzathioprinAzathioprin CyklofosfamidCyklofosfamid Cyklosporin ACyklosporin A PlazmaferesisPlazmaferesis
Systemic sclerosis
Multisystem disease that predominantly Multisystem disease that predominantly affect the skin, locomotion system, organsaffect the skin, locomotion system, organs
Women : men 4:1Women : men 4:1 Aetiology: unknownAetiology: unknown HLA B8, DR3HLA B8, DR3
Clinical features
Skin involvement – edema štádiumSkin involvement – edema štádium
sklerosis skinsklerosis skin
skin atrofiaskin atrofia GIT involvement: GIT involvement:
hypomotility of oesophagushypomotility of oesophagus
hypomotility of small bowelhypomotility of small bowel
malabsorbtionmalabsorbtion
primárna biliar cirhosisprimárna biliar cirhosis
Lung involvement Lung involvement alveolitisalveolitis fibrosisfibrosis pulmonary hypertensionpulmonary hypertension Heart involvementHeart involvement fibrosisfibrosis perikarditisperikarditis arrythmiasarrythmias Vascular involvement:Vascular involvement: Raynod s phenomenonRaynod s phenomenon Joints and muscles: arthralgia, arthritisJoints and muscles: arthralgia, arthritis Kidney involvement: renal failure, nephrotic Kidney involvement: renal failure, nephrotic
syndrome syndrome
Treatment D-penicilamínD-penicilamín CyklofosfamidCyklofosfamid MethotrexateMethotrexate CorticosteroidsCorticosteroids Cyklosporín ACyklosporín A
Dermatomyositis Associated with carcinoma- paraneoplastic!!!Associated with carcinoma- paraneoplastic!!! Inflammatory disease of muscles – polymyoszitisInflammatory disease of muscles – polymyoszitis + skin dermatomyositis+ skin dermatomyositisAetiology: unknownAetiology: unknownClinical features: febrility, Raynaud s phenomenon, Clinical features: febrility, Raynaud s phenomenon,
arthritisarthritisMuscle weakness, atrophy of musclesMuscle weakness, atrophy of musclesSkin involvement: inflammation of eyelids, Skin involvement: inflammation of eyelids,
heliotrope coloration, ragged cuticleheliotrope coloration, ragged cuticleJoint involvementJoint involvement
Dysphagy, pulmonary manifestation: alveolitis, Dysphagy, pulmonary manifestation: alveolitis, pulmonary hypertensionpulmonary hypertension
Heart involvementHeart involvement Investigation: muscle enzymes: CK, aldolaseInvestigation: muscle enzymes: CK, aldolase
EMGEMG
muscle biopsymuscle biopsy
Treatment: corticosteroidsTreatment: corticosteroids
methotrexatemethotrexate
azathioprin azathioprin
Poor prognosisPoor prognosis
Sjogren s syndrome
Sicca syndrome: dry eyes, dry mouthSicca syndrome: dry eyes, dry mouth HLA B8, DR3HLA B8, DR3 Primary SjSPrimary SjS
Secundary SjS –RA, SLESecundary SjS –RA, SLE
KO: xerostomyKO: xerostomy
xerophtalmy: keratoconjunctivitis siccaxerophtalmy: keratoconjunctivitis sicca
Involvement: kidney, lungs, jointsInvolvement: kidney, lungs, joints
Myalgia, artralgia, arthritis Myalgia, artralgia, arthritis
Biopsies of the salivary gland or of the Biopsies of the salivary gland or of the lipshow a focal infiltration of lymphocytes lipshow a focal infiltration of lymphocytes and plasma cells.and plasma cells.
Laboratory abnormalities: ESR, RF,ANA, Laboratory abnormalities: ESR, RF,ANA, anti-Ro (SSA), anti La (SSB) anti-Ro (SSA), anti La (SSB) trombocytopaeniatrombocytopaenia
Treatment: symptomaticTreatment: symptomatic
antimalaric: hydroxychlorochineantimalaric: hydroxychlorochine
corticosteroid drugscorticosteroid drugs