IGAV Flashcards
What is IgA Vasculitis also known as?
Henoch-Schönlein Purpura (HSP)
IgA Vasculitis (IgAV) is a type of vasculitis characterized by the presence of IgA in immune complexes.
What is the etiology of IgA Vasculitis?
Unknown etiology characterized by inflammation of small blood vessels
It involves leukocytic infiltration of tissue, hemorrhage, and ischemia.
What type of immune complexes are associated with IgA Vasculitis?
Predominantly composed of IgA
This suggests a hypersensitivity process.
What is the incidence of IgA Vasculitis in children?
13 per 100,000 children
It is the most common systemic vasculitis in childhood.
What age group is primarily affected by IgA Vasculitis?
Children aged 3-15 years
Although it has been described in adults, it predominantly affects younger individuals.
What is the most common cause of nonthrombocytopenic purpura?
IgA Vasculitis (Henoch-Schönlein Purpura)
It is recognized as the leading cause of this condition in children.
What is the hallmark clinical manifestation of IgA Vasculitis (IgAV)?
Palpable purpura
Palpable purpura is caused by small vessel inflammation in the skin, leading to extravasation of blood.
In which areas of the body is the rash of IgA Vasculitis typically found?
Below the waist, on the buttocks, and on lower extremities
The rash can begin as small macules or urticarial lesions but progresses to purpura.
What percentage of patients with IgA Vasculitis experience arthritis?
80%
Arthritis is most common in the lower extremities, particularly the ankles and knees.
What are common gastrointestinal symptoms in children with IgA Vasculitis?
Mild to moderate crampy abdominal pain
Gastrointestinal involvement occurs in about one half of affected children.
What laboratory test is most important for differentiating IgA Vasculitis from other causes of purpura?
Platelet count
IgAV is characterized by nonthrombocytopenic purpura with a normal or high platelet count.
What are the four criteria for the diagnosis of Immunoglobulin A Vasculitis?
- Palpable purpura
- Bowel angina
- Diagnostic biopsy
- Pediatric age group
The diagnosis is based on the presence of two of these four criteria.
True or False: Renal involvement in IgA Vasculitis is rare.
False
One third of children with IgAV develop renal involvement, which can be acute or chronic.
What serious gastrointestinal complications can occur in IgA Vasculitis?
- Bloody diarrhea
- Intussusception
- Abdominal perforation
These complications may require emergent intervention.
What histological changes are seen in a biopsy for IgA Vasculitis?
Granulocytes in the walls of arterioles or venules; IgA deposits in vessel wall
This helps differentiate it from other forms of leukocytoclastic vasculitis.
Fill in the blank: IgA Vasculitis occurs more frequently in _______ than in the summer months.
winter
The condition is slightly more common in males than females.
What is the typical presentation of joint swelling in IgA Vasculitis?
Acute and very painful
Joint swelling can be confused with peripheral edema seen with the rash.
What is the classification of Kawasaki disease?
Medium-sized vessel vasculitis
Kawasaki disease is classified under medium-sized vessel vasculitis in pediatric conditions.
What are the two types of small vessel vasculitis?
Granulomatous and nongranulomatous
Granulomatous small vessel vasculitis includes conditions like eosinophilic granulomatosis with polyangiitis, while nongranulomatous includes immunoglobulin A vasculitis.
What is the prognosis of IgA vasculitis (IgAV)?
Excellent, with complete resolution in most cases
Most children with IgAV resolve without significant sequelae.
What are the risks for children with renal involvement in IgAV?
Hypertension and renal insufficiency
Children with renal disease in IgAV are at higher risk for long-term complications.
What is the long-term risk of progression to end-stage renal disease in IgAV patients?
Less than 1%
Rarely, IgAV can progress to end-stage renal disease, necessitating renal transplantation.
What is the typical treatment for acute arthritis or skin manifestations in IgAV?
Non-steroidal anti-inflammatory drugs
Supportive therapy is usually the first line of treatment.
What is the typical dosing regimen for corticosteroids in IgAV?
Prednisone 1-2 mg/kg/day, maximum 60 mg/day
A gradual taper plan over 4-6 weeks is recommended.
