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