Slide 1 Organ-specific autoimmune diseases Prof Peter Gergely Slide 2 Ssystemic autoimmune diseases systemic lupus erythematosus (SLE) antiphospholipid syndrome (APS) rheumatoid arthritis (RA) Sjögren’s syndrome (SS) scleroderma group mixed connective tissue disease (MCTD) myositis group UCTD and overlap Organ-specific Slide 3 1. ENDOCRINE SYSTEM 1. ENDOCRINE SYSTEM thyroid thyroid parathyroid parathyroid pancreas pancreas adrenals adrenals gonads gonads hypophysis hypophysis 2. DIGESTIVE SYSTEM 2. DIGESTIVE SYSTEM stomach stomach intestines intestines liver liver 3. EYE 3. EYE 4. NERVOUS SYSTEM 4. NERVOUS SYSTEM 5. HEART 5. HEART 6. KIDNEY 6. KIDNEY 7. LUNG 7. LUNG 8. SKIN 8. SKIN 9. BLOOD 9. BLOOD
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· Rheumatic fever (febris rheumatica) Rheumatic fever (RF) develops following Group A Streptococcus (usually throat) infection in 3-4% of genetically susceptible ...
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Rheumatic fever (RF) develops following Group A Streptococcus (usually throat) infection in 3-4% of genetically susceptible children, not treated by antibiotics. The disease can be detected in cca. 30% of patients within 3- 4 weeks after infection.The basis of disease is an immune reaction between heart antigens and cross-reacting streptococcal antigens.
Prevalence: 100/100,000 in non-developed countries2/100,000 in developed countries
Slide 25 Cross-reacting epitopes between streptococcus N-
Slide 26 Diagnostic Criteria for RF:The diagnosis of RF may be established by the modified Jones criteria (AHA, 1992): a) Major criteria: carditis, polyarthritis, Sydenham’s chorea, subcutaneous nodules, erythema marginatumb) Minor criteria:- clinical: fever, arthralgia - laboratory: high ESR, high CRP, prolonged PR distance on ECG (in the absence of manifest carditis)c) Essential to prove underlying infection:- High or increasing ASO, or- Positive culture or bacterial quick-test for Streptococcus-Astrain.Diagnosis can be established in the presence of 2 major or 1 major + 2 minor criteria. The presence of streptococcal infection is mandatory!
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Histology of Aschoff body (inflammatory nodule)
Slide 28 Mitralstenosis
Slide 29 Erythema marginatum
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Erythema marginatum
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Subcutaneous nodules
Slide 32 Treatment and Prevention
Antibiotics (penicillin or macrolid) – prophylaxis for at least 10 years
Nonsteroid antiinflammatory drugs
Slide 33 b
GBM antibodies in Goodpasture syndrome
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Pemphigus vulgaris
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Bullous pemphigoid
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Dermatitis herpetiformis
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Herpes gestationis
Slide 38 a b
a) Pemphigus vulgaris – antibodies against keratinocytes (desmoglein 3)b) Bullous pemphigoid – antibodies against skin basal membrane
Slide 39 Vitiligo
- frequent 1-16%- autoimmune raction against melanocytes- genetic predisposition
Slide 40
Alopecia areata- sometimes totalis (1-2%)- lymphocytes attack hair follicles
A nonblanching, nonpalpable petechial rash in a patient with immune thrombocytopenia. ITP is the most frequent cause of acquired thrombocytopenia in children. It is caused by platelet destruction by autoantibodies. An episode may be preceded by a viral infection. Thrombocytopenia is not associated with significant lymphadenopathy and hepatosplenomegaly. Anemia and neutropenia are absent. Approximately 80% to 90% of cases of acute ITP resolve without recurrence.
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Thrombocytopenia and large platelets in a patient with ITP. The increased platelet size is thought to reflect increased megakaryopoiesis. Giant platelets and thrombocytopenia are also observed in Bernard-Soulier syndrome, a hereditary bleeding disorder with defective platelet glycoprotein Ib/IX surface receptors
Slide 46
A normal or increased number of megakaryocytes in the bone marrow of patients with ITP. Megakaryocytes are easily identified as the largest cell type in the bone marrow and by their finely granular cytoplasm and multilobed nuclei. A low megakaryocyte count, decreased cellularity, and the presence of abnormal cells suggests a diagnosis other than ITP. In typical cases of ITP, a bone marrow aspirate is not mandatory
Slide 48 APS is a thrombophilic disorder caused by antiphospholipid (APL) antibodies (actually the antibodies are directed against phospholipid –glycoprotein complexes); characterised by arterial and/or venous thrombosis and/or pregnancy loss.
There are two forms:
a) primary APS (without underlying disease), andb) secondary APS (associated with other autoimmune disease (most frequently SLE, AIHA, ITP. etc)
Slide 49 Epidemiology:No reliable data.
Antibodies occur in 1-5% of normal population (increases with age)
APS is more frequent in females (ratio 4-10:1). In SLE the occurrence of APS is 20-30%. The APS (i.e. positive LA and/or ACL) in other thromboembolic diseases (e.g. stroke, AMI) varies around 10%. In pregnancy loss the likelihood of APS is 10-20%.
Slide 50 Pathomechanism:a) endothel and thrombocyte activation → thrombus
formation
b) inhibition of binding of endogenous anticoagulants (e,g,: activated protein C, protein S, annexin V, etc)
One or more clinical episodes of arterial, venous, or small vessel thrombosis, in any tissue or organ .
Pregnancy Morbidity:a) One or more unexplained deaths of a morphologically normal fetus at or beyond the 10th week of gestation, ORb) One or more premature births of a morphologically normal neonate at or before the 34th week of gestation because of severe preeclampsia or eclampsia, or severe placental insufficiency, ORc) Three or more unexplained consecutive spontaneous abortions before the 10th week of gestation .
Laboratory criteria a) Lupus anticoagulant present in plasma, on 2 or more occasions at least
12 weeks apart, OR b) Anticardiolipin antibody of IgG and/or IgM isotype in blood, present in
medium or high titer, on 2 or more occasions, at least 12 weeks apart, OR c) Anti-beta2-glycoprotein I antibody of IgG/IgM isotype in blood, present
in>99% percentile titre, on 2 or more occasions at least 12 weeks apart.
For a definitive APS diagnosis the presence of at least 1 clinical and at least 1 laboratory criterion is required.
Slide 56 Therapy Acute: heparin, then long-term warfarin (coumarine)
Long-term: warfarin - INR between 2-3
Pregnancy: NO warfarin, LMW heparin (+ low-dose aspirin
In milder cases: aspirin alone (or other platelet-aggregation inhibitor)