IGAV

Question 1

What is IgA Vasculitis also known as?

Answer 1

Henoch-Schönlein Purpura (HSP)

IgA Vasculitis (IgAV) is a type of vasculitis characterized by the presence of IgA in immune complexes.

Question 2

What is the etiology of IgA Vasculitis?

Answer 2

Unknown etiology characterized by inflammation of small blood vessels

It involves leukocytic infiltration of tissue, hemorrhage, and ischemia.

Question 3

What type of immune complexes are associated with IgA Vasculitis?

Answer 3

Predominantly composed of IgA

This suggests a hypersensitivity process.

Question 4

What is the incidence of IgA Vasculitis in children?

Answer 4

13 per 100,000 children

It is the most common systemic vasculitis in childhood.

Question 5

What age group is primarily affected by IgA Vasculitis?

Answer 5

Children aged 3-15 years

Although it has been described in adults, it predominantly affects younger individuals.

Question 6

What is the most common cause of nonthrombocytopenic purpura?

Answer 6

IgA Vasculitis (Henoch-Schönlein Purpura)

It is recognized as the leading cause of this condition in children.

Question 7

What is the hallmark clinical manifestation of IgA Vasculitis (IgAV)?

Answer 7

Palpable purpura

Palpable purpura is caused by small vessel inflammation in the skin, leading to extravasation of blood.

Question 8

In which areas of the body is the rash of IgA Vasculitis typically found?

Answer 8

Below the waist, on the buttocks, and on lower extremities

The rash can begin as small macules or urticarial lesions but progresses to purpura.

Question 9

What percentage of patients with IgA Vasculitis experience arthritis?

Answer 9

80%

Arthritis is most common in the lower extremities, particularly the ankles and knees.

Question 10

What are common gastrointestinal symptoms in children with IgA Vasculitis?

Answer 10

Mild to moderate crampy abdominal pain

Gastrointestinal involvement occurs in about one half of affected children.

Question 11

What laboratory test is most important for differentiating IgA Vasculitis from other causes of purpura?

Answer 11

Platelet count

IgAV is characterized by nonthrombocytopenic purpura with a normal or high platelet count.

Question 12

What are the four criteria for the diagnosis of Immunoglobulin A Vasculitis?

Answer 12

• Palpable purpura
• Bowel angina
• Diagnostic biopsy
• Pediatric age group

The diagnosis is based on the presence of two of these four criteria.

Question 13

True or False: Renal involvement in IgA Vasculitis is rare.

Answer 13

False

One third of children with IgAV develop renal involvement, which can be acute or chronic.

Question 14

What serious gastrointestinal complications can occur in IgA Vasculitis?

Answer 14

• Bloody diarrhea
• Intussusception
• Abdominal perforation

These complications may require emergent intervention.

Question 15

What histological changes are seen in a biopsy for IgA Vasculitis?

Answer 15

Granulocytes in the walls of arterioles or venules; IgA deposits in vessel wall

This helps differentiate it from other forms of leukocytoclastic vasculitis.

Question 16

Fill in the blank: IgA Vasculitis occurs more frequently in _______ than in the summer months.

Answer 16

winter

The condition is slightly more common in males than females.

Question 17

What is the typical presentation of joint swelling in IgA Vasculitis?

Answer 17

Acute and very painful

Joint swelling can be confused with peripheral edema seen with the rash.

Question 18

What is the classification of Kawasaki disease?

Answer 18

Medium-sized vessel vasculitis

Kawasaki disease is classified under medium-sized vessel vasculitis in pediatric conditions.

Question 19

What are the two types of small vessel vasculitis?

Answer 19

Granulomatous and nongranulomatous

Granulomatous small vessel vasculitis includes conditions like eosinophilic granulomatosis with polyangiitis, while nongranulomatous includes immunoglobulin A vasculitis.

Question 20

What is the prognosis of IgA vasculitis (IgAV)?

Answer 20

Excellent, with complete resolution in most cases

Most children with IgAV resolve without significant sequelae.

Question 21

What are the risks for children with renal involvement in IgAV?

Answer 21

Hypertension and renal insufficiency

Children with renal disease in IgAV are at higher risk for long-term complications.

Question 22

What is the long-term risk of progression to end-stage renal disease in IgAV patients?

Answer 22

Less than 1%

Rarely, IgAV can progress to end-stage renal disease, necessitating renal transplantation.

Question 23

What is the typical treatment for acute arthritis or skin manifestations in IgAV?

Answer 23

Non-steroidal anti-inflammatory drugs

Supportive therapy is usually the first line of treatment.

Question 24

What is the typical dosing regimen for corticosteroids in IgAV?

Answer 24

Prednisone 1-2 mg/kg/day, maximum 60 mg/day

A gradual taper plan over 4-6 weeks is recommended.

Question 25

True or False: The arthritis associated with IgAV leaves permanent joint damage.

Answer 25

False

The arthritis in IgAV typically does not recur and does not cause permanent joint damage.

Question 26

What complications can arise from gastrointestinal involvement in IgAV?

Answer 26

Intussusception and possible obstruction or infarction

Gastrointestinal symptoms can lead to abnormal peristalsis, increasing the risk of serious complications.

Question 27

Fill in the blank: Most cases of IgAV are ________, lasting 3-4 weeks.

Answer 27

Monophasic

IgAV typically resolves completely after a monophasic course.

Question 28

What should parents be warned about regarding IgAV?

Answer 28

Possible recurrences of symptoms

Although most cases resolve, the rash can wax and wane for up to a year.