Schonlein - Hennoch purpura

Июль 22, 2022

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Schonlein - Hennoch purpura

Содержание

2. Henoch-Schönlein Purpura (HSP) is a common vasculitis of small vessels with cutaneous and systemic complications. It
3. EPIDEMIOLOGY The etiology is unknown more frequent in children than adults, with most cases occurring between
4. PATHOGENESIS in specific populations, patients with HSP have a significantly higher frequency of HLA-DRB1*01 and decreased
5. CTLA-4 +49 A/G genotype and HLA-DRB1 polymorphisms in Turkish patients with Henoch-Schönlein purpura. presence of Cytotoxic
7. The immunobiology of Henoch-Schönlein purpura. group A beta-hemolytic streptococcus (GAS) has widely studied and found in
8. CLINICAL MANIFESTATIONS The disease onset may be acute, or insidious, with sequential occurrence of symptoms over
9. CLINICAL MANIFESTATIONS Rash (95-100%), especially involving the legs, may not be present on initial presentation Subcutaneous
10. Rash beginning as pinkish maculopapules that initially blanch on pressure and progress to petechiae or purpura,
11. Rash
12. Arthritis present in more than ⅔ of children with HSP, is usually localized to the knees
13. Gastrointestinal tract intermittent abdominal pain that is often colicky in nature. There may be peritoneal exudate,
14. Renal involvement occurs in 25-50% of children may manifest with: hematuria, proteinuria, or both; nephritis or
15. Increased serum levels of insulin-like growth factor (IGF)-1 and IGF-binding protein-3 in Henoch-Schonlein purpura. Serum IGF-1
16. What is the difference between IgA nephropathy and Henoch-Schönlein purpura nephritis
17. Relationship between initial clinical signs and risk of chronic renal failure in Henoch-Schönlein purpura nephritis
18. Complications of Henoch-Schönlein Purpura Hepatosplenomegaly Myocardial infarction Pulmonary hemorrhage Pleural effusion Unnecessary abdominal surgery Intussusception Hemorrhage
19. DIAGNOSIS Diagnostic uncertainty arises when the symptom complex of edema, rash, arthritis with abdominal complaints, and
20. DIAGNOSIS Affected children often have a moderate thrombocytosis and leukocytosis. The erythrocyte sedimentation rate (ESR) may
21. Definitive diagnosis confirmed by biopsy cutaneous site showing leukocytoclastic angiitis. Renal biopsy may show mesangial deposition
22. H & E stain of skin biopsy showing leukocytoclastic vasculitis with infiltration of neutrophils.
23. Henoch-Schönlein purpura. A: Cutaneous purpura; B: Urine sediment red blood cell cast; C: Acute glomerular inflammation
24. Immunofluorescence micrograph of a glomerulus from a patient with HSP nephropathy stained for the presence of
25. Differential Diagnosis of Henoch-Schönlein Purpura Acute abdomen Meningococcal meningitis or septicemia Rheumatoid arthritis Rheumatic fever Idiopathic
26. Acute hemorrhagic edema (AHE) is an acute cutaneous benign leukocytoclastic vasculitis seen in children ≤2 yr
27. Acute hemorrhagic edema (AHE)
28. TREATMENT Symptomatic treatment adequate hydration, bland diet, pain control with acetaminophen is provided for self-limited complaints
29. TREATMENT Therapy with oral or intravenous corticosteroids (1-2 mg/kg/day) is often associated with dramatic improvement of
30. TREATMENT is the same as for other forms of acute glomerulonephritis If anti-cardiolipin or antiphospholipid antibodies
31. Prognosis More than 80% of patients have a single isolated episode lasting a few weeks. Approximately
33. Скачать презентацию

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Henoch-Schönlein Purpura (HSP)
is a common vasculitis of small vessels with

cutaneous and systemic complications.
It is the most common cause of nonthrombocytopenic purpura in children.

Слайд 3

EPIDEMIOLOGY
The etiology is unknown
more frequent in children than adults, with most

cases occurring between 2 and 8 yr of age,
most frequently in the winter months.
The overall incidence is estimated to be 9/100,000 population.
Males are affected twice as frequently as females.

Слайд 4

PATHOGENESIS
in specific populations, patients with HSP have a significantly higher

frequency of HLA-DRB1*01 and decreased frequency of the *07 haplotype than controls.
increased serum concentrations of the cytokines tumor necrosis factor-α (TNF-α) and interleukin (IL)-6.
In one study, almost half of the patients had elevated antistreptolysin O (ASO) anti-bodies, implicating group A streptococcus.
HSP is an IgA-mediated vasculitis of small vessels.
Immunofluorescence techniques show deposition of IgA and C3 in the
small vessels of the skin and the renal glomeruli;

Слайд 5

CTLA-4 +49 A/G genotype and HLA-DRB1 polymorphisms in Turkish patients with

Henoch-Schönlein purpura.

presence of Cytotoxic T lymphocyte-associated protein 4( CTLA-4) AG genotype and HLA-DRB1*13 could be a risk factor for developing nephrotic-range proteinuria in these patients.
.

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The immunobiology of Henoch-Schönlein purpura.
group A beta-hemolytic streptococcus (GAS) has widely

studied and found in 20–50% of patients with acute HSP by serological tests or bacterial cultures,
Bartonella henselae (12 of 18 HSP patient sera were positive )
Parvovirus B19 (only one of 29 HSP patients )
Other HSP-associated pathogens have been reported
Staphylococcus aureus,
Helicobacter Pylori,
Hemophilus parainfluenza,
Coxsackie virus,
adenovirus,
hepatitis A virus,
hepatitis B virus

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CLINICAL MANIFESTATIONS
The disease onset may be acute, or insidious, with sequential

occurrence of symptoms over a period of weeks or months.
Low-grade fever and fatigue are present in more than half of affected children.
The typical rash and the clinical symptoms of HSP are a consequence of the usual location of the acute small vessel damage primarily in the skin, gastrointestinal tract, and kidneys.

Слайд 9

CLINICAL MANIFESTATIONS
Rash (95-100%), especially involving the legs, may not be present

on initial presentation
Subcutaneous edema (20-50%)
Abdominal pain and vomiting (85%)
Joint pain (60-80%), especially involving the knees and ankles
Scrotal edema (2-35%)
Bloody stools

Слайд 10

Rash
beginning as pinkish maculopapules that initially blanch on pressure and

progress to petechiae or purpura,
characterized clinically as palpable purpura that evolve from red to purple to rusty brown before they eventually fade
last from 3-10 days, and may appear at intervals that vary from a few days to as long as 3-4 mo.
In <10% of children, recurrences of the rash may not end until as late as a yr,
Damage to cutaneous vessels also results in local angioedema, which may precede the palpable purpura.
Edema independent of purpura occurs primarily in dependent areas such as below the waist, over the buttocks (or on the back and posterior scalp in the infant), or in areas of greater tissue distensibility such as the eyelids, lips, scrotum, or dorsum of the hands and feet.

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Rash

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Arthritis
present in more than ⅔ of children with HSP,
is usually

localized to the knees and ankles and appears to be concomitant with edema.
The effusions are serous, not hemorrhagic,
resolve after a few days without residual deformity or articular damage.
They may recur during a subsequent reactive phase of the disease.

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Gastrointestinal tract
intermittent abdominal pain that is often colicky in nature.
There

may be peritoneal exudate, enlarged mesenteric lymph nodes, segmental edema, and hemorrhage into the bowel.
More than half of patients have occult heme-positive stools,
diarrhea (with or without visible blood), or hematemesis.
Intussusception may occur, which may rarely be followed by complete obstruction or infarction with bowel perforation.
If not resolved by hydrostatic reduction during a contrast study, surgical intervention is necessary.

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Renal involvement
occurs in 25-50% of children
may manifest with:
hematuria,
proteinuria, or

both;
nephritis or nephrosis;
acute renal failure.
Renal involvement at presentation may lead to chronic hypertension or end-stage renal disease in the future

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Increased serum levels of insulin-like growth factor (IGF)-1 and IGF-binding protein-3

in Henoch-Schonlein purpura.
Serum IGF-1 levels were significantly higher in HSP with proteinuria than those without proteinuria and controls (p = 0.001 and p = 0.001, respectively).
Also, IGFBP-3 levels were greater in HSP with proteinuria compared to those without proteinuria and controls (p = 0.005 and p = 0.0001).
Serum immunoglobulin-A/complement-C3 ratio was higher in HSP than in the controls (p = 0.0001) but this ratio did not change according to proteinuria, hematuria or positive SOB.
In conclusion, IGF-1 and IGFBP-3 levels could be new markers for determination of renal involvement in HSP.

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What is the difference between IgA nephropathy and Henoch-Schönlein purpura nephritis

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Relationship between initial clinical signs and risk of chronic renal failure

in Henoch-Schönlein purpura nephritis

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Complications of Henoch-Schönlein Purpura
Hepatosplenomegaly Myocardial infarction Pulmonary hemorrhage Pleural

effusion Unnecessary abdominal surgery Intussusception Hemorrhage Shock Gastrointestinal bleeding Bowel infarction Renal failure Hematuria Proteinuria Seizures Mononeuropathies
Testicular torsion

Слайд 19

DIAGNOSIS
Diagnostic uncertainty arises when the symptom complex of edema, rash, arthritis

with abdominal complaints, and renal findings occurs for a prolonged period.

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DIAGNOSIS
Affected children often have a moderate thrombocytosis and leukocytosis.
The erythrocyte

sedimentation rate (ESR) may be elevated.
Anemia may result from chronic or acute gastrointestinal blood loss.
Immune complexes are often present, and 50% of patients have elevated concentrations of IgA as well as IgM
usually negative for antinuclear antibodies (ANAs), antibodies to nuclear cytoplasmic antigens (ANCAs), and rheumatoid factor (even in the presence of rheumatoid nodules).
Anticardiolipin or antiphospholipid antibodies may be present and contribute to the intravascular coagulopathy.
Intussusception is usually ileoileal in location;
Renal involvement manifests in red blood cells, white blood cells, casts, or albumin in the urine and azotemia

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Definitive diagnosis confirmed by biopsy
cutaneous site showing leukocytoclastic angiitis.
Renal biopsy

may show mesangial deposition of IgA and occasionally IgM, C3, and fibrin.

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H & E stain of skin biopsy showing leukocytoclastic vasculitis with

infiltration of neutrophils.

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Henoch-Schönlein purpura.
A: Cutaneous purpura;
B: Urine sediment red blood cell

cast;
C: Acute glomerular inflammation and crescent formation;
D: Details of basal membrane
mesangial proliferation
and IgA deposits

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Immunofluorescence micrograph of a glomerulus from a patient with HSP nephropathy

stained for the presence of IgA.

Слайд 25

Differential Diagnosis of Henoch-Schönlein Purpura
Acute abdomen
Meningococcal meningitis or septicemia
Rheumatoid

arthritis
Rheumatic fever
Idiopathic thrombocytopenic purpura
Systemic lupus erythematosus
poly-arteritis nodosa,
Child abuse
Drug reactions
Bacterial endocarditis
Rocky Mountain spotted fever
familial Mediterranean fever
inflammatory bowel disease.
Kawasaki disease.

Слайд 26

Acute hemorrhagic edema (AHE)
is an acute cutaneous benign leukocytoclastic vasculitis
seen

in children ≤2 yr of age
AHE presents with fever; tender edema of the face, scrotum, hands, and feet; and ecchymosis (usually larger than the purpura of HSP) on the face and extremities
petechiae may be seen in mucous membranes.
The patient usually appears well except for the rash.
The platelet count is normal or elevated;
the urinalysis is normal.
The younger age, nature of the lesions, absence of other organ involvement, and biopsy may help distinguish AHE from HSP.

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Acute hemorrhagic edema (AHE)

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TREATMENT
Symptomatic treatment
adequate hydration,
bland diet,
pain control with acetaminophen is

provided for self-limited complaints of arthritis, edema, fever, and malaise.
Avoidance of competitive activities and avoidance of maintaining the lower extremities in a dependent position may decrease local edema.
If edema involves the scrotum, elevation of the scrotum and local cooling, as tolerated, may decrease discomfort.

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TREATMENT
Therapy with oral or intravenous corticosteroids (1-2 mg/kg/day) is often associated

with dramatic improvement of both gastrointestinal and CNS complications.
the effects of corticosteroids on renal manifestations are not clear.
intussusception may be life-threatening and managed with cortico-steroids and, when necessary, hydrostatic reduction (by air or with contrast) or resection of the intussusception.

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TREATMENT
is the same as for other forms of acute glomerulonephritis
If

anti-cardiolipin or antiphospholipid antibodies are identified and thrombotic events have occurred, aspirin (81 mg) given once may decrease the risks associated with a hypercoagulable state.
Rheumatoid nodules may respond to alternate-day colchicine (0.6 mg every other day).

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Prognosis
More than 80% of patients have a single isolated episode lasting

a few weeks.
Approximately 10-20% of patients have recurrences.
Fewer than 5% of patients develop chronic HSP.
Abdominal pain resolves spontaneously within 72 hours in most patients

Schonlein - Hennoch purpura

Содержание

Henoch-Schönlein Purpura (HSP) is a common vasculitis of small vessels with

EPIDEMIOLOGYThe etiology is unknownmore frequent in children than adults, with most

PATHOGENESIS in specific populations, patients with HSP have a significantly higher

CTLA-4 +49 A/G genotype and HLA-DRB1 polymorphisms in Turkish patients with

The immunobiology of Henoch-Schönlein purpura. group A beta-hemolytic streptococcus (GAS) has widely

CLINICAL MANIFESTATIONSThe disease onset may be acute, or insidious, with sequential

CLINICAL MANIFESTATIONSRash (95-100%), especially involving the legs, may not be present

Rash beginning as pinkish maculopapules that initially blanch on pressure and

Rash

Arthritispresent in more than ⅔ of children with HSP, is usually

Gastrointestinal tractintermittent abdominal pain that is often colicky in nature. There

Renal involvementoccurs in 25-50% of childrenmay manifest with: hematuria, proteinuria, or

Increased serum levels of insulin-like growth factor (IGF)-1 and IGF-binding protein-3

What is the difference between IgA nephropathy and Henoch-Schönlein purpura nephritis

Relationship between initial clinical signs and risk of chronic renal failure

Complications of Henoch-Schönlein Purpura Hepatosplenomegaly Myocardial infarction Pulmonary hemorrhage Pleural

DIAGNOSISDiagnostic uncertainty arises when the symptom complex of edema, rash, arthritis

DIAGNOSISAffected children often have a moderate thrombocytosis and leukocytosis. The erythrocyte

Definitive diagnosis confirmed by biopsy cutaneous site showing leukocytoclastic angiitis. Renal biopsy