Henoch-Schonlein Purpura. Dr. Sergio Manzano
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- Aubrey Violet Kennedy
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1 Dr. Sergio Manzano 1
2 Introduction Epidemiology Aetiology Clinical diagnosis Differential diagnosis Complementary examinations Case definition Complications Treatment Follow-up 2
3 Introduction: Henoch-Schonlein Purpura Johann Schönlein: Association purpura & joint pain (1837) Eduard Henoch: Gastrointestinal & renal involvment (1868) 3
4 Epidemiology: Henoch-Schonlein Purpura o The most common vasculitis in childhood o All ages (2-6 years) o / children per year o Ethnicity: Asians > Caucasians > Africans o Male > Female (1.2:1) 4
5 Aetiology : o Immune complex-mediated disease o Genetic predisposition o Trigger: o URT Infections: o Gr. A β-hemolytic Streptococcus o Viruses (adenovirus, pb19, herpes ) o Mycoplasma o Vaccines: o MMR, pneumococcus, influenza, meningococcus & hepatitis B o Drugs (?) 5
6 Aetiology : IgA immune complexes deposits Skin vessels Kidney Joints Digestive tract 6
7 Aetiology : IgA immune complexes deposits Skin vessels
8 Aetiology : IgA immune complexes deposits Leucocytoclasticvasculitiswith perinuclear infiltration of polymorphs and mononuclear cells Skin vessels Purpuric lesions and petechiae 8
9 Aetiology : IgA immune complexes deposits Skin vessels Kidney Joints Digestive tract 9
10 Aetiology : IgA immune complexes deposits Kidney 10
11 Aetiology : IgA immune complexes deposits focal and segmental proliferative glomerulonephritis Kidney Indistinguishable from that of IgA nephropathy 11
12 Aetiology : IgA immune complexes deposits Skin vessels Kidney Joints Digestive tract 12
13 Aetiology : IgA immune complexes deposits Joints 13
14 Aetiology : IgA immune complexes deposits IgA immune complexes deposits in the synovial tissue Arthralgia or arthritis Joints 14
15 Aetiology : IgA immune complexes deposits Skin vessels Kidney Joints Digestive tract 15
16 Aetiology : IgA immune complexes deposits Digestive tract 16
17 Aetiology : IgA immune complexes deposits Bowel wall vasculitis Oedema and haemorrhage Abdominal pain, vomiting and bloody stools Intussusception Digestive tract 17
18 Clinical Diagnosis: Henoch-Schonlein Purpura 18
19 Clinical Diagnosis: Henoch-Schonlein Purpura o Good general appearance o Little or no fever 19
20 Clinical Diagnosis: o Petechiae or purpura Henoch-Schonlein Purpura Mainly on the feet, legs and buttocks 20
21 Clinical Diagnosis: o Arthralgia or arthritis Mainly in lower limbs Henoch-Schonlein Purpura 21
22 Clinical Diagnosis: Henoch-Schonlein Purpura o Cramping abdominal pain 22
23 Clinical Diagnosis: Henoch-Schonlein Purpura o Gastrointestinal bleeding 23
24 Clinical Diagnosis: o Hematuria Henoch-Schonlein Purpura 24
25 Clinical Diagnosis: o High blood pressure Henoch-Schonlein Purpura 25
26 Clinical Diagnosis: Henoch-Schonlein Purpura o Scrotal pain and swelling 26
27 Clinical Diagnosis: Henoch-Schonlein Purpura o Good general appearance, little or no fever o Petechiae or purpura o Arthralgia or arthritis o Cramping abdominal pain o Gastrointestinal bleeding o Hematuria, high blood pressure o Scrotal pain and swelling 27
28 Differential diagnosis: Henoch-Schonlein Purpura 28
29 Differential diagnosis: Henoch-Schonlein Purpura o Viral infection (enterovirus) Generalized rash abdominal pain No arthralgia 29
30 Differential diagnosis: Henoch-Schonlein Purpura o Septicemia (meningococcemia) Altered general appearance Signs of poor perfusion and shock Fever 30
31 Differential diagnosis: Henoch-Schonlein Purpura o Idiopathic thrombocytopenic purpura No abdominal pain No arthralgia Bruises, bleeding Low platelets 31
32 Differential diagnosis: o Leukemia Henoch-Schonlein Purpura Petechiae, arthralgia Enlarged spleen and liver Altered CBC 32
33 Differential diagnosis: o Coagulopathy Henoch-Schonlein Purpura Bruises purpura Hemarthrosis may mimic HSP arthralgia Signs of bleeding Abnormal coagulation tests 33
34 Differential diagnosis: Henoch-Schonlein Purpura o Other etiologies for arthritis: o Septic arthritis o Post-streptococcal o Viral o Reactive o Rheumathoid 34
35 Differential diagnosis: Henoch-Schonlein Purpura o Other etiologies for abdominal pain o Appendicitis o Intussusception ( complication of HSP) o 35
36 Differential diagnosis: Henoch-Schonlein Purpura o Other etiologies for renal impairment 36
37 Complementary examinations: 37
38 Complementary examinations: o Blood: o Complete blood count: Normal platelets o Coagulation test: Normal o Urea, Creatinin, Albumin: May be abnormal o ASLO: Negative o Blood culture: Negative 38
39 Complementary examinations: o Urine : o Urine test strip: Hematuria and proteinuria possible (if renal involvment) 39
40 Complementary examinations: o Stools: o Search for blood: May be positive 40
41 Complementary examinations: o CSF: o If suspicion of meningitis o Ultrasound: o Abdominal (if severe abdominal pain) o Scrotal (if edema or testicular pain) o Skin or renal biopsy (if diagnosis unsure) 41
42 Case definition: Henoch-Schonlein Purpura 42
43 Case definition: 43
44 Case definition: 44
45 Case definition: 45
46 Case definition: 46
47 Case definition: 47
48 Complications : Henoch-Schonlein Purpura o Gastrointestinal: massive bleeding, intussusception o Urogenital: Orchitis (30%), torsion o Neurological: Seizure, cerebral vasculitis (rare) o Renal: Nephritis, nephrotic syndrom 48
49 Treatment: o HSP spontaneousoly resolves in 94% of children o Mainly supportive: Paracetamol and NSAIDS o Severe abdominal pain: oral Prednisone 1-2mg/kg/day 49
50 Follow-up: o Long-term complication: nephritis o Appears during the first year (99%) o Blood pressure + urine test strip (Proteins, blood) (regularely during 1 year) Refer to nephrologist if HBP, proteinuria or macrohematuria 50
51 Conclusion JAMA. 2012;307(7):742 51
52 Thank you! 52
53 Question 1: o What are the 4 main organs involved in HSP: A. Liver, kidney, skin and synovial tissue B. Synovial tissue, digestive tract, kidney and skin C. Digestive tract, synovial tissue, spleen and kidney D. Kidney, lungs, synovial tissue and liver 53
54 Question 1: o What are the 4 main organs involved in HSP: A. Liver, kidney, skin and synovial tissue B. Synovial tissue, digestive tract, kidney and skin C. Digestive tract, synovial tissue, spleen and kidney D. Kidney, lungs, synovial tissue and liver 54
55 Question 2: o Which of the following is not a typical HSP complication: A. seizures B. Intussusception C. Pneumonia D. Orchitis 55
56 Question 2: o Which of the following is not a typical HSP complication: A. seizures B. Intussusception C. Pneumonia D. Orchitis 56
57 Question 3: o Which of the following sentences is wrong: A. HSP occurs more frequently in Africans than Caucasians B. HSP affects all age groups C. HSP is the most common vasculitis in childhood D. HSP is an IgA immune complex-mediated disease 57
58 Question 3: o Which of the following sentences is wrong: A. HSP occurs more frequently in Africans than Caucasians B. HSP affects all age groups C. HSP is the most common vasculitis in childhood D. HSP is an IgA immune complex-mediated disease 58
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