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- Advances in the Understanding of Etiology (I)
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1. Infection and autoimmunity: a two-way relationship
- Dr. Ricard Cervera
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2. Autoimmunity and atherosclerosis
- Prof. Johan Frostegard
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3. Endothelium and autoimmunity
- Prof. Pier Luigi Meroni
- Advances in the Understanding of Etiology (II)
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4. Prolactin has a pathogenic role in systemic lupus erythematosus
- Prof. Luis Jara-Quezada
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5. Sjögren's syndrome: current knowledge and future prospects
- Prof. Athanasios G. Tzioufas
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6. Genetic and environmental triggers of autoimmune thyroid diseases
- Prof. Yaron Tomer
- Advances in Pathogenesis and Clinical Manifestation
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8. Pathogenic mechanisms of autoantibodies: circulating and local antibodies that form immune complexes
- Dr. Luís Eduardo Coelho Andrade
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9. Pathogenic mechanisms of autoantibodies: antibodies to intracellular and membrane-bound antigens
- Dr. Luís Eduardo Coelho Andrade
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10. What causes primary biliary cholangitis?
- Dr. M. Eric Gershwin
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11. Capillaroscopy in autoimmune rheumatic diseases
- Prof. Maurizio Cutolo
- Advances in Diagnostic Methodologies (I)
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12. The past, present & future of ANA testing: history and challenges of ANA
- Prof. Marvin J. Fritzler
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13. The past, present & future of ANA testing: changing bandwidth and future of ANA
- Prof. Marvin J. Fritzler
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14. Diagnostic methods in autoimmunity
- Mr. Steven Binder
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15. Systemic lupus erythematosus: novel aspects of pathogenesis and treatment 1
- Prof. Chaim Putterman
- Dr. Noa Schwartz
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16. Systemic lupus erythematosus: novel aspects of pathogenesis and treatment 2
- Prof. Chaim Putterman
- Dr. Noa Schwartz
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17. Novel aspects of systemic sclerosis
- Prof. Gabriele Valentini
- Advances in Diagnostic Methodologies (II)
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18. An update on the multiple faces of celiac disease
- Prof. Aaron Lerner
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19. Protective autoantibodies
- Prof. Elias Toubi
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21. Rheumatic fever: a model of a post-infectious autoimmune disease
- Prof. Jorge Kalil
- Treatments of autoimmune diseases
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22. Intravenous immunoglobulins: clinical evidence
- Prof. Shaye Kivity
- Latest Developments in the Field
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23. Adjuvants and autoimmunity
- Dr. Eitan Israeli
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24. Antiphospholipid syndrome (APS): from pathogenesis to treatment
- Prof. Roger Levy
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25. Vitamin D and autoimmunity
- Dr. Shir Azrielant
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26. Pregnancy in rheumatic diseases
- Prof. Pier Luigi Meroni
- Archived Lectures *These may not cover the latest advances in the field
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27. Pathogenic mechanisms of autoantibodies
- Prof. Cees Kallenberg
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28. B lymphocytes on the frontline of autoimmunity
- Prof. Pierre Youinou
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29. Apoptotic cell clearance deficiency
- Prof. Martin Herrmann
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30. Auto-antibodies as predictors of autoimmune disease
- Dr. Nicola Bizzaro
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31. Innate immunity and natural autoantibodies
- Prof. Luc Mouthon
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32. Accelerated atherosclerosis in autoimmune rheumatic disease
- Dr. Andrea Doria
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33. Pregnancy in autoimmune rheumatic diseases
- Prof. Angela Tincani
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36. Immunologic mechanisms in systemic lupus erythematosus
- Prof. Kok-Yong Fong
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37. Osteoimmunology
- Prof. João Eurico Fonseca
- Ms. Joana Caetano-Lopes
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38. ANCA-associated systemic vasculitides
- Prof. Loic Guillevin
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39. The common origin for diverse autoimmune diseases
- Prof. Juan-Manuel Anaya
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40. Anti-cytokine therapy in rheumatoid arthritis
- Prof. Piercarlo Sarzi-Puttini
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41. Sjogren's syndrome: autoimmune epithelitis
- Prof. Haralampos Moutsopoulos
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42. Vitamin D and autoimmunity
- Dr. Howard Amital
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43. Intravenous immunoglobulins: myth and reality
- Prof. Zera Tellier
Printable Handouts
Navigable Slide Index
- Introduction
- Historical facts
- Thrombosis (XIX century)
- Original paper on false serological test for syphilis
- Lupus anticoagulant and its various associations
- A major advance in this field exploded in 1984
- The first anticardiolipin antibodies detection paper
- Aziz Gharavi
- Beta2-glycoprotein I (beta2GPI)
- Beta2-glycoprotein I (beta2GPI) - mode of action
- Beta2GPI anti-pospholipid-Ab directed domains
- Phospholipid riding on the back of beta2GPI
- 6-8% of normals have antiphospholipid antibodies
- Several families of antiphospholipid antibodies
- Induction of anti-phospholipid (aPL)
- APS was not recognized until 1984
- The antiphospholipid syndrome
- The antiphospholipid syndrome: major subsets
- The syndromic charateristics of APS
- Systemic APS - Hughes syndrome
- Diagnostic criteria for APS
- Revised classification criteria for the APS
- 'Primary'' and ''secondary'' syndromes
- APL associated thromboses
- Intermediate syndromes
- Differences between primary and secondary (1)
- Differences between primary and secondary (2)
- Progression from primary APS to SLE
- The relationship between APS and SLE
- Follow-up of primary APS patients is necessary
- Clinical manifestations due to thromboembolism
- Non-thrombotic manifestations of APS
- Thrombotic and non-thrombotic complications
- Secondary thrombotic complications
- Clinical manifestations of APS
- Livedo reticularis
- Livedo reticularis - endotheliopathy present
- Thrombocytopenia
- Libman-Sacks endocarditis
- Clinical manifestations: summary
- CAPS ("Ashersons" syndrome)
- CAPS as defined in 1992
- Relationship of APS to CAPS
- Definite CAPS
- Probable CAPS
- Clinical manifestations of CAPS
- Clinical manifestations of SIRS
- CAPS vs. APS (1)
- CAPS vs. APS (2)
- Unusual features of CAPS
- DIC is another unusual feature of CAPS
- Prevalence features within DIC subset of patients
- Bone marrow necrosis in CAPS patients
- Mononeuritis muliplex in CAPS pateints
- Relationship of CAPS to gender and age
- Associated autoimmune diseases
- Triggering factors
- Infectious triggers
- Trauma/surgical procedures
- Obstetric causes
- Malignancies and lymphoma in CAPS patients
- Lupus "flares" in CAPS patients
- Double/treble HIT hypothesis
- Strokes in one third of patients
- Pulmonary complications
- Scan of diffuse alveolar haemorrhage
- Histology of diffuse alveolar haemorrhage
- Acute respiratory distress syndrome (ARDS)
- Pulmonary microthrombosis
- Cardiac pathology
- Occurrence of heart valve lesions in APS
- Occurrence of gastrointestinal manifestations
- Occurrence of thrombotic lesions
- Bowel infarctions
- Multiple hepatic infarcts in CAPS
- Adrenal haemorrhage/infarction in CAPS
- Enlarged adrenals on CT scan
- Occurrence of dermatolofical manifestations
- Occurrence of renal thrombotic microangiopathy
- Occurrence of peripheral thrombosis
- Outcomes of CAPS (1)
- Outcomes of CAPS (2)
- Outcomes of CAPS: mortality (1)
- Outcomes of CAPS: mortality (2)
- Major clinical causes of death in CAPS patients
- Infections and pulmonary causes in CAPS deaths
- Abdominal causes in CAPS deaths
- Deaths due to multiorgan failure
- Poor prognosis (1)
- Poor prognosis (2)
- Therapy of CAPS
- Prophylactic therapy
- Specific therapies
- Second line therapies
- Secondary non-specific therapies
- Improvement of CAPS with leg amputations
- Microangiopathic APL syndromes (MAPS)
- MAPS comprises a combination of pathologies
- Thrombotic microangiopathy and aPL Ab
- The major microangiopathic APL syndromes
- Triggering factors for MAPS
- HELLP syndrome and anti-phospholipid antibodies
- History of HELLP syndrome
- Patients with both HELLP syndrome and CAPS
- Patients with TTP/aPL Ab overlap syndromes
- Disseminated intravascular coagulation
- Conclusions (1)
- Conclusions (2)
- The CAPS registry
- Literature on the subject
Topics Covered
- Description of the antiphospholipid syndrome (APS)
- Primary, secondary and intermediate syndromes
- Thrombotic and non-thrombotic manifestations of the APS
- Beta 2 glycoprotein 1
- Relationship of APS to CAPS
- Clinical manifestations of CAPS
- Triggering factors
- Pathogenesis
- Microangiopathic antiphospholipid syndromes
Links
Series:
Categories:
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Talk Citation
Asherson, R. (2017, May 17). The catastrophic antiphospholipid syndrome and the microangiopathic antiphospholipid syndrome [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved December 7, 2024, from https://doi.org/10.69645/MAUC2867.Export Citation (RIS)
Publication History
Financial Disclosures
- Prof. Ronald Asherson has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.
The catastrophic antiphospholipid syndrome and the microangiopathic antiphospholipid syndrome
A selection of talks on Clinical Practice
Transcript
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0:00
Today's talk concentrates on the Antiphospholipid Syndromes and Variants in 2006,
including the Catastrophic Antiphospholipid Syndrome, known as CAPS,
as well as the newly delineated Microangiopathic Antiphospholipid Syndrome, termed MAPS.
0:20
A few historical facts.
0:24
The great Virchow's postulates at the end of the 19th century,
to explain thrombosis consisted of stasis of the blood,
vessel wall abnormalities, and blood abnormalities.
And it is of interest that more than a hundred years later,
these postulates still hold true.
0:43
Wassermann in 1906, first documented a serological diagnostic test for Syphilis,
and this serodiagnostic reaction,
in fact forms the basis for the Phospholipid Syndrome more than 80 years later.
1:01
Many papers, in fact had been published from 1963 onwards,
on the Lupus anticoagulant and its associations with thrombosis,
recurrent fetal losses, and thrombocytopenia.
1:16
However, in 1983, there was a major advance in this field,
which was due to the discovery of the anticardiolipin antibodies in the laboratories of
Graham Hughes at the Hammersmith Hospital in London by Nigel Harris and Azzudin Gharavi.
From 1983 onwards, Hughes himself,
as well as Ronald Asherson, his close associate,
and the many fellows who trained in this unit from 1980 to 1990,
then proceeded to describe the major clinical associations of these antibodies.
Here follows a slide with a picture of Nigel Harris at that time,
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