Topic Presentation Henoch-Schonlein Purpura (HSP) : Name: Sweta Jaanvhi Raj S160258 DATE: 16/09/2020

TOPIC PRESENTATION
HENOCH- SCHONLEIN
PURPURA (HSP)
NAME: SWETA JAANVHI RAJ

S160258
DATE: 16/09/2020
BACKGROUND
- HSP is an acute IgA mediated autoimmune vasculitis illness

- First described by Heberdon in 1801; with the cases seen in a 5 year old
male child presenting with abdominal pain, purpuric rash, arthralgia and
with blood tinged urine.
- Most common form of systemic vasculitis in children
- Most commonly affected age group: 3-10 years
- Most cases has a tetrad of clinical manifestation and is a self- limiting
condition
- Etiology: multifactorial- with genetic, environmental, and antigenic
components. URTI, pharyngeal infection- streptococcus, or GI
infection. Multiple bacterial and viral infectious agents have been
associated with the development of HSP
• Genetic predisposition- Human leukocyte antigen +/- other immune
related genes polymorphism
• Other triggers: Vaccination- MMR, insect bites, drugs- beta lactams,
erythromycin, vancomycin, sulfonamides, beta blockers; neoplasia,
Systemic diseases.
EPIDEMIOLOGY
- Primarily a childhood illness, its incidence decreases with age. Less

common in adults, however; when present is more severe then in a child.
- In a population-based study from the United Kingdom, the annual
incidence was approximately 20 per 100,000 in children <17 years of age,
with a peak incidence of 70 per 100,000 in children between the ages of
four and six years
- Most studies show a male predominance, with reported male-to-female
ratios of 1.2:1 to 1.8:1
- More common in the White or Asian; compared to other races.
PATHOPHYSIOLOGY
• The exact cause and mechanism of HSP is unknown; although;

Immunologic, genetic, and environmental factors all seem to
play a role
• IgA complexes with complement gets deposited in target organs,
resulting in proliferation of inflammatory mediators, including
vascular prostaglandins such as prostacyclin, may play a central
role in the pathogenesis of HSP vasculitis
• The characteristic finding of IgAV (HSP) is leukocytoclastic vasculitis
accompanied by IgA immune complexes within affected organs
• Immunofluorescence studies show IgA, complement component 3 (C3),
and fibrin deposition within the walls of involved vessels. IgA, C3, fibrin,
immunoglobulin G (IgG), and, less commonly, immunoglobulin M (IgM)
also are deposited within the endothelial and mesangial cells of the kidney
• Can affect the small vessels- capillaries of any organ/ system- most
commonly affects the skin, joints, intestines and kidney.
CLASSIFICATION CRITERIA
• In 1990, a committee of the American College of Rheumatology (ACR) established criteria to
classify seven types of vasculitis including IgAV (HSP). The ACR criteria for the diagnosis of IgAV
(HSP) are as follows:
- Palpable purpura
- Age at onset ≤20 years
- Acute abdominal pain
- Biopsy showing granulocytes in the walls of small arterioles and/or venules
[Two or more of the criteria had a sensitivity and specificity of approximately 90 percent in separating
adult patients with IgAV (HSP) from those with other causes of vasculitis]
• 2005- pediatric consensus criteria developed by the European League
Against Rheumatism (EULAR) and the Paediatric Rheumatology
European Society (PRES) and were subsequently validated in conjunction
with the Paediatric Rheumatology International Trials Organisation
(PRINTO)
• The mandatory criterion:
- purpura (usually palpable and in clusters) or petechiae, with lower
limb predominance + without thrombocytopenia or coagulopathy.
• Patients also must have one or more of the following:
- Abdominal pain (usually diffuse, with acute onset)
- Arthritis or arthralgia (acute onset)
- Renal involvement (proteinuria, hematuria)
- Leukocytoclastic vasculitis or proliferative glomerulonephritis,
with predominant IgA deposition
CLINICAL MANIFESTATIONS
•Prodromal symptoms:
- Fever, headache, anorexia
• Rash
– Purpura is the presenting symptom in approximately 75% cases
- Often begins with erythematous, macular, or urticarial wheals
- The initial rash may coalesce and evolve into the typical ecchymoses,
petechiae, and palpable purpura
- symmetrically distributed, and located primarily in gravity/pressure-
dependent areas
• Arthralgia/arthritis manifestation (50-75%) – joint symptoms are the
second most common
- usually transient or migratory
- typically oligoarticular (one to four joints), and nondeforming.
- It usually affects the lower-extremity large joints (hips, knees, and ankles)
or less commonly the upper extremities (elbows, wrists, and hands
- There is often prominent periarticular swelling and tenderness but usually
without joint effusion, erythema, or warmth. Patients may have
considerable pain and limitation of motion.
• Gastrointestinal symptoms — Gastrointestinal symptoms occur in
approximately 50% of children with IgAV (HSP)
- typically develop within 8 days of the appearance of the rash
- nausea, vomiting, abdominal pain, and transient paralytic ileus
- More significant findings (complications)- Rarely; gastrointestinal
hemorrhage, bowel ischemia and necrosis, intussusception, and bowel
perforation
• Renal disease – (frequency of renal involvement ranges from 20-54%).
- It is more prevalent in older children and adults.
- The most common presentation is hematuria with or without red blood cell
casts and mild or no proteinuria.
- Nephrotic-range proteinuria, an elevated serum creatinine, and/or
hypertension are present in a minority of patients. These findings, as well
as the coexistence of hematuria and proteinuria, are associated with an
increased risk of progressive disease.
Other organ involvement
- Scrotum: Clinical findings include pain, tenderness, and swelling of the involved
testicle and/or scrotum
- Central and peripheral nervous system: headaches, seizures, encephalopathy, focal
neurologic deficits, ataxia, intracerebral hemorrhage, and central and peripheral
neuropathy
Most of the CNS findings are transient except for occasional permanent sequelae
associated with hemorrhagic stroke.
- Respiratory tract
- Eyes: Keratitis and uveitis
DIFFERENTIAL DIAGNOSIS
• Purpura
- Petechiae and purpuric rashes may be associated with septicemia,
Dengue hemorrhagic fever, immune thrombocytopenia (ITP),
hemolytic uremic syndrome, leukemia, and coagulopathies (eg,
hemophilia), meningitis.
- Normal platelet count and coagulation studies differentiate IgAV
(HSP) from these entities
• Purpura with normal platelet count and coagulation studies.
- Acute hemorrhagic edema of infancy (AHEI) – Also known as Finkelstein
or Seidlmayer disease
- Hypersensitivity vasculitis
- Other small vessel vasculitides- however uncommon in children
• Arthritis and arthralgia
- Autoimmune diseases, such as SLE, juvenile idiopathic arthritis (JIA), and
rheumatic fever, may present with joint symptoms similar to IgAV (HSP)
- Septic arthritis and toxic synovitis also may present with joint symptoms
similar to those seen in patients with IgAV (HSP)
• Abdomen pain- abdominal emergencies requiring surgical intervention
such as intussusception, appendicitis, bowel infarction, or perforation can
be a complication of HSP, however when it presents alone- difficult to
distinguish if it is caused by HSP.
• Renal disease
- IgA nephropathy
INVESTIGATION
• Routine blood tests- usually have nonspecific findings

- FBC: normal findings or may indicate normochromic anemia, platelets usually
normal, +/- leukocytosis
- Coagulation profile- normal
- Blood urea nitrogen (BUN) and creatinine
• Serum IgA levels- elevated
• Urinalysis- presence of microscopic hematuria/ proteinuria
• Fecal occult blood test
• Skin biopsy or renal biopsy in severe cases
MANAGEMENT
• Self limiting condition; thus, herapy is primarily supportive and includes

adequate hydration, rest, and symptomatic relief.
• Pain relief- Acetaminophen
- NSAIDs- Naproxen 10-20mg/kg in 2 divided doses per day;
Ibuprofen
- Glucocorticoids- in severe cases, oral prednisone 1- 2mg/kg
per day or IV methylprednisolone 0.8-1.6mg/kg/day.
• Indication of hospitalization
- Inability to maintain adequate hydration with oral intake
- Severe abdominal pain
- Significant gastrointestinal bleeding
- Changes in mental status
- Severe joint involvement limiting ambulation and/or self-care
- Renal insufficiency (elevated creatinine), hypertension, and/or nephrotic
syndrome
• Renal disease: Specific treatment of IgAV (HSP) nephritis should be
considered only in patients with marked proteinuria (>1 g/day) and/or
impaired renal function during the acute episode.
• Limited renal involvement: monitor urine protein excretion and serum
creatinine once per week for one month, then every two weeks for two
months to assess for disease progression.
• Severe impairement: proteinuria to >1 g/day or an increase in serum
creatinine, we perform a renal biopsy to evaluate the need for more
aggressive therapy- IV methyprednisolone
PROGNOSIS
• Prognosis is usually excellent in the absence of significant renal disease,

the initial episode of IgAV (HSP) typically resolves within one month.
• Reoccurrence usually in almost one third of the children, typically presents
within 4 months of initial visit.
• Morbidity in the initial phase of IgAV (HSP) is primarily a result of
gastrointestinal complications, including intussusception and, less
commonly, bowel ischemia, bowel perforation, or pancreatitis.
• The long-term morbidity in patients with IgAV (HSP) is a result of renal
disease. 90% of children who develop renal involvement do so within 2
months of onset, and 97% within 6 months. Adults are at higher risk for
renal disease both early in the course of disease and longer term
- Thus monthly F/up visits with vitalo signs- blood pressure and urinalysis
for 1st few months- then every other month for the 1st year of presentation
TAKE HOME MESSAGE
• HSP is the commonest cause of systemic vasculitis in children

• - Self limiting condition, prognosis is excellent, however the
greater the renal involvement- more worse is the prognosis
• Etiology- multifactorial: genetic, immunological, environmental
factors.
• Identify classical tetrad of symptoms-also keeping in mind; it may
be absent
- treatment: supportive- hydration, symptomatic management and
rest.
REFERENCE
• https://
www.uptodate.com/contents/iga-vasculitis-henoch-schonlein-purp
ura-management?search=IgA%20vasculitis&source=search_result&s
electedTitle=2~150&usage_type=default&display_rank=2#H1
• https://
www.uptodate.com/contents/iga-vasculitis-henoch-schonlein-purp
ura-clinical-manifestations-and-diagnosis?search=IgA%20vasculitis&
source=search_result&selectedTitle=1~150&usage_type=default&d
isplay_rank=1
• https://emedicine.medscape.com/article/984105-differential#1
• Paediatrics at a Glance 2nd edition by Lawrence Miall, Mary Rudolf
and Malcolm Lavene

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TOPIC PRESENTATION

NAME: SWETA JAANVHI RAJ

- HSP is an acute IgA mediated autoimmune vasculitis illness

- Primarily a childhood illness, its incidence decreases with age. Less

• The exact cause and mechanism of HSP is unknown; although;

• Routine blood tests- usually have nonspecific findings

• Self limiting condition; thus, herapy is primarily supportive and includes

• Prognosis is usually excellent in the absence of significant renal disease,

• HSP is the commonest cause of systemic vasculitis in children

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