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Connective tissue disorders Systemic lupus erythematosus

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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 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


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

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

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

11 Lungs Recurent pleurisy Pleural effusions Pulmonary fibrosis
Pulmonary hypertension

12 Serositis Pleuritis - 30-60% patients - clinicaly asymptomatic
- more often in drug induced SLE Pericarditis - 60% patients Diffuse peritonitis with little amount of fluid

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

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

15 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

16 Diagnostic criteria for SLE

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

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

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

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

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

22 Treatment D-penicilamín Cyklofosfamid Methotrexate Corticosteroids
Cyklosporín A


24 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

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




29 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

30 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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