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Connective tissue disorders Systemic lupus erythematosus Želmíra Macejová

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Presentation on theme: "Connective tissue disorders Systemic lupus erythematosus Želmíra Macejová"— Presentation transcript:

1 Connective tissue disorders Systemic lupus erythematosus Želmíra Macejová

2 Systemic lupus erythematosus autoimmune, organ non specific disease hyperreaction of B-lymphocyts with production of antibodies agains organ non- specific antibodies multisystem disease unknown etiology variable course and prognosis

3 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 combination Mortality: involvement of kidney and CNS General clinical features - fatigue, fever, malaise, weight loss

4 Ethiology unknown, multifactorial unknown, multifactorial exogenous: virus infection exogenous: virus infection UV light UV light stress stress drugs: hydralazin drugs: hydralazin endogenous: hormonal endogenous: hormonal genetic predisposition genetic predisposition immune reaction: immune reaction: hyperreactivity of B- cells hyperreactivity of B- cells pathologic T cell, failure of remove immune complexes pathologic T cell, failure of remove immune complexes

5 Clinical Features- general Subfebrility, fever- 80% Subfebrility, fever- 80% Malaise, tiredness Malaise, tiredness Skin Skin Mental disorders, psychiatric manifestation Mental disorders, psychiatric manifestation arthralgia, arthritis arthralgia, arthritis thrombosis thrombosis Changes of blood count Changes of blood count

6 The joint Arthritis, arthralgia – morning stiffness stuhnutosť Arthritis, arthralgia – morning stiffness stuhnutosť Involvement: small joints of hand, wrist, knees, Involvement: small joints of hand, wrist, knees, arthritis- is not erosive arthritis- is not erosive Myositis- pain of involved muscles Myositis- pain of involved muscles

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8 Skin manifestation Photosenzitivity Photosenzitivity Butterfly erythema Butterfly erythema Vaskulitic lesions: fingertips, around nails, purpura, urticaria Vaskulitic lesions: fingertips, around nails, purpura, urticaria Raynaud s phenomenon, Raynaud s phenomenon, Livedo reticularis Livedo reticularis Palmar, plantar rashes Palmar, plantar rashes

9 Hematologic changes Anaemia Anaemia Neutropenia, lymfopenia, trombocytopénia Neutropenia, lymfopenia, trombocytopénia ESR – raised ESR – raised CRP normal CRP normal ANA : positiv: dsDNA, DNP, RF, serum complement- redused, immunoglobulin IgG, IgM - raised ANA : positiv: dsDNA, DNP, RF, serum complement- redused, immunoglobulin IgG, IgM - raised

10 The kidney Lupus nephritis – 30-50% Lupus nephritis – 30-50% cause of death cause of death immune complex deposits in the kidney immune complex deposits in the kidney hypertension hypertension nephrotic syndrome nephrotic syndrome renal failure renal failure

11 Lungs Recurent pleurisy Recurent pleurisy Pleural effusions Pleural effusions Pulmonary fibrosis Pulmonary fibrosis Pulmonary hypertension Pulmonary hypertension

12 Serositis Pleuritis % patients Pleuritis % patients - clinicaly asymptomatic - clinicaly asymptomatic - more often in drug induced SLE - more often in drug induced SLE Pericarditis - 60% patients Pericarditis - 60% patients - clinicaly asymptomatic - clinicaly asymptomatic Diffuse peritonitis with little amount of fluid Diffuse peritonitis with little amount of fluid - clinicaly asymptomatic

13 The heart - 40% - 40% Pericarditis Pericarditis Myocarditis - arrythgmia, cardiomegaly, tachykardia Myocarditis - arrythgmia, cardiomegaly, tachykardia Endocarditis - abakteriálna Libman-Sacks Endocarditis - abakteriálna Libman-Sacks

14 The gastrointestinal system Vasculitis of art. mesenterica: Vasculitis of art. mesenterica: antiphospholipid syndrome Lupus enteritis Lupus enteritis Pancreatitis Pancreatitis Autoimune hepatitis Autoimune hepatitis Symptoms: nausea, vomiting, anorexia, diarrhoe

15 The nervous system- neuropsychiatric lupus N-P lupus : 60-70% N-P lupus : 60-70% Main cause of death Main cause of death Mild depression Mild depression Severe psychiatric disturbances Severe psychiatric disturbances Epilepsy Epilepsy Cerebellar ataxia Cerebellar ataxia Aseptic meningitis Aseptic meningitis Peripheral neuropathy Peripheral neuropathy Cause: vasculitis, immune-complex deposition Cause: vasculitis, immune-complex deposition

16 Diagnostic criteria for SLE

17 Diagnostic criteria SLE 1. Facial skin erythema 1. Facial skin erythema 2. Discoid erythema 2. Discoid erythema 3. Fotosenzitivity 3. Fotosenzitivity 4. Mouth ulcers At least 4!! 4. Mouth ulcers At least 4!! 5. Arthralgia, arthritis 5. Arthralgia, arthritis 6. Serozitis 6. Serozitis 7. Involvement of kidney 7. Involvement of kidney 8. Neurologic manifestation 8. Neurologic manifestation 9. Hematologic manifestation 9. Hematologic manifestation 10. Imunologic manifestation 10. Imunologic manifestation 11. Antinuklear antibodies 11. Antinuklear antibodies

18 Treatment NSA NSA corticosteroids corticosteroids Antimalarial drugs - hydroxychloroquine Antimalarial drugs - hydroxychloroquine Azathioprin Azathioprin Cyklofosfamid Cyklofosfamid Cyklosporin A Cyklosporin A Plazmaferesis Plazmaferesis

19 Systemic sclerosis Multisystem disease that predominantly affect the skin, locomotion system, organs Multisystem disease that predominantly affect the skin, locomotion system, organs Women : men 4:1 Women : men 4:1 Aetiology: unknown Aetiology: unknown HLA B8, DR3 HLA B8, DR3

20 Clinical features Skin involvement – edema štádium Skin involvement – edema štádium sklerosis skin sklerosis skin skin atrofia skin atrofia GIT involvement: GIT involvement: hypomotility of oesophagus hypomotility of oesophagus hypomotility of small bowel hypomotility of small bowel malabsorbtion malabsorbtion primárna biliar cirhosis primárna biliar cirhosis

21 Lung involvement Lung involvement alveolitis alveolitis fibrosis fibrosis pulmonary hypertension pulmonary hypertension Heart involvement Heart involvement fibrosis fibrosis perikarditis perikarditis arrythmias arrythmias Vascular involvement: Vascular involvement: Raynod s phenomenon Raynod s phenomenon Joints and muscles: arthralgia, arthritis Joints and muscles: arthralgia, arthritis Kidney involvement: renal failure, nephrotic syndrome Kidney involvement: renal failure, nephrotic syndrome

22 Treatment D-penicilamín D-penicilamín Cyklofosfamid Cyklofosfamid Methotrexate Methotrexate Corticosteroids Corticosteroids Cyklosporín A Cyklosporín A

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24 Dermatomyositis Associated with carcinoma- paraneoplastic!!! Associated with carcinoma- paraneoplastic!!! Inflammatory disease of muscles – polymyoszitis Inflammatory disease of muscles – polymyoszitis + skin dermatomyositis + skin dermatomyositis Aetiology: unknown Clinical features: febrility, Raynaud s phenomenon, arthritis Muscle weakness, atrophy of muscles Skin involvement: inflammation of eyelids, heliotrope coloration, ragged cuticle Joint involvement

25 Dysphagy, pulmonary manifestation: alveolitis, pulmonary hypertension Dysphagy, pulmonary manifestation: alveolitis, pulmonary hypertension Heart involvement Heart involvement Investigation: muscle enzymes: CK, aldolase Investigation: muscle enzymes: CK, aldolase EMG EMG muscle biopsy muscle biopsy Treatment: corticosteroids methotrexate methotrexate azathioprin azathioprin Poor prognosis

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29 Sjogren s syndrome Sicca syndrome: dry eyes, dry mouth Sicca syndrome: dry eyes, dry mouth HLA B8, DR3 HLA B8, DR3 Primary SjS Primary SjS Secundary SjS –RA, SLE KO: xerostomy xerophtalmy: keratoconjunctivitis sicca xerophtalmy: keratoconjunctivitis sicca Involvement: kidney, lungs, joints Myalgia, artralgia, arthritis

30 Biopsies of the salivary gland or of the lipshow a focal infiltration of lymphocytes and plasma cells. Biopsies of the salivary gland or of the lipshow a focal infiltration of lymphocytes and plasma cells. Laboratory abnormalities: ESR, RF,ANA, anti-Ro (SSA), anti La (SSB) trombocytopaenia Laboratory abnormalities: ESR, RF,ANA, anti-Ro (SSA), anti La (SSB) trombocytopaenia Treatment: symptomatic Treatment: symptomatic antimalaric: hydroxychlorochine antimalaric: hydroxychlorochine corticosteroid drugs corticosteroid drugs

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