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Connective tissue disorders Systemic lupus erythematosus

Connective tissue disorders Systemic lupus erythematosus. Želmíra Macejová. 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

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Connective tissue disorders Systemic lupus erythematosus

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  1. Connective tissue disordersSystemic 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 • exogenous: virus infection • UV light • stress • drugs: hydralazin • endogenous: hormonal • genetic predisposition • immune reaction: hyperreactivity of B- cells pathologic T cell , failure of remove immune complexes

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

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

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

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

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

  10. Lungs • Recurent pleurisy • Pleural effusions • Pulmonary fibrosis • Pulmonary hypertension

  11. Serositis • Pleuritis - 30-60% patients - clinicaly asymptomatic - more often in drug induced SLE • Pericarditis - 60% patients - clinicaly asymptomatic • Diffuseperitonitiswith little amount of fluid - clinicaly asymptomatic

  12. The heart • - 40% • Pericarditis • Myocarditis - arrythgmia, cardiomegaly, tachykardia • Endocarditis- abakteriálna Libman-Sacks

  13. The gastrointestinal system • Vasculitisof art. mesenterica: antiphospholipid syndrome • Lupus enteritis • Pancreatitis • Autoimune hepatitis Symptoms: nausea, vomiting, anorexia, diarrhoe

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

  15. Diagnostic criteria for SLE

  16. Diagnostic criteria SLE • 1. Facial skin erythema • 2. Discoiderythema • 3. Fotosenzitivity • 4. MouthulcersAtleast 4!! • 5. Arthralgia, arthritis • 6. Serozitis • 7. Involvementofkidney • 8. Neurologic manifestation • 9. Hematologic manifestation • 10. Imunologicmanifestation • 11. Antinuklearantibodies

  17. Treatment • NSA • corticosteroids • Antimalarial drugs - hydroxychloroquine • Azathioprin • Cyklofosfamid • Cyklosporin A • Plazmaferesis

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

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

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

  21. Treatment • D-penicilamín • Cyklofosfamid • Methotrexate • Corticosteroids • Cyklosporín A

  22. Dermatomyositis • Associated with carcinoma- paraneoplastic!!! • Inflammatory disease of muscles – polymyoszitis + 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

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

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

  25. 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 • Treatment: symptomatic antimalaric: hydroxychlorochine corticosteroid drugs

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