PSR Case Discussion

Henoch Schonlein Purpura (IgA vasculitis)

May is Vasculitis Awareness Month

Case Analysis
12-year-old girl with purpura rashes

Typical Atypical

• GI symptoms: severe • Markedly elevated

abdominal pain, GI WBC
bleeding • Atypical purpura?
• Purpura

No data
Renal manifestation
• Haematuria, foamy urine
• Blood pressure
• Urinalysis, kidney function?
IgA Vasculitis
Clinical manifestations
Symptoms Affected body area Average duration Estimated % of
patients affected
Palpable purpura or petechiae Skin (mainly lower extremities and lower 3–10 days 100
parts of the arm)
(Poly)arthralgia Joints (knees and ankles) 7–10 days >80
Gastrointestinal disturbances (incl. Lower digestive tract 4-8 days >50
haematochezia and colicky abdominal pain)
Glomerulonephritis Kidney 3–12 days 40-50

120

100

80

60

40

20

0
Purpura Athralgia, arthritis Abdominal pain Gastrointestinal bleeding Renal involvement Subcutaneous edema Orchitis
Emery, et al Rosenblum & Winter Bagga, et al Saulsbury, et al Nong, et al Average

May is Vasculitis Awareness Month

IgA Vasculitis
EULAR/PRINTO/PRES criteria for Henoch–Schönlein purpura
Sensitivity Specificity AUC
Criterion Glossary (%) (%) (%)

Purpura Purpura (commonly palpable and in crops) or petechiae, with lower limb
89 86 87.5
mandatory criterion predominance, *not related to thrombocytopenia.
Diffuse abdominal colicky pain with acute onset assessed by history and physical
1. Abdominal pain 61 64 62.2
examination. May include intussusception and gastrointestinal bleeding.
Typically, leukocytoclastic vasculitis with predominant IgA deposit or proliferative
2. Histopathology 93 89 91.1
glomerulonephritis with predominant IgA deposit
Arthritis of acute onset defined as joint swelling or joint pain with limitation on
3. Arthritis or motion
78 42 59.9
arthralgias Arthralgia of acute onset defined as joint pain without joint swelling or limitation
on motion
Proteinuria >0.3 g/24 h or >30 mmol/mg of urine albumin/creatinine ratio on a
spot morning sample
4. Renal involvement 33 70 51.4
Haematuria or red blood cell casts: >5 red blood cells/high power field or red
blood cells casts in the urinary sediment or ≥2+ on dipstick
HSP EULAR/PRINTO/PRES Ankara 2008 classification definition: κ 0.90 (95% CI 0.84 to 0.96) 100 87 93.5

Ozen S, et al. Ann Rheum Dis. 2010;69:798-806

IgA Vasculitis
SHARE Recommendations for the Diagnosis of IgAV
No Recommendation LoE SoR

Use of biopsy

1. A skin biopsy including specific immunofluorescence staining for IgA should be performed in case of atypical 4 D
rash and/or to exclude alternative diagnoses; skin biopsy is not needed in a patient with the typical purpuric
skin rash on lower limbs and buttocks
2. Absence of IgA immunofluorescence staining on biopsy does not exclude the diagnosis of IgAV 3 C

Renal work-up

3. Renal involvement should be investigated using eGFR and urinalysis (haematuria and UP:UC ratio or UA:UC 2B C
ratio)
4. A paediatric nephrologist should be consulted if an IgAV patient has moderate proteinuria and/or impaired GFR 4 D

5. A renal biopsy should be performed if an IgAV patient has severe proteinuria (>250mg/mmol for at least 4 2A
weeks; although shorter duration of severe proteinuria is also a relative indication for biopsy), persistent
moderate (100-250mg/mmol) proteinuria or impaired GFR
Imaging

6. In severe abdominal pain, an US should be performed by an ultrasonographer with paediatric expertise to 4 D

exclude intestinal intussusception

Ozen S et al. Rheumatology. 2019;58:1607–16.

IgA Vasculitis
SHARE Recommendations for the Treatment of IgAV
No Recommendation LoE SoR

Analgesia
1. Adequate analgesia should be prescribed for IgAV-associated arthropathy 4 D
2. NSAIDs are not contraindicated if renal function is normal in IgAV 4 D
3. Adequate analgesia should be prescribed for IgAV-associated abdominal pain 4 D
Use of corticosteroid
4. CS treatment is indicated in case of: 4 D
• Orchitis
• Cerebral vasculitis
• Pulmonary haemorrhage
• Other severe organ- or life-threatening vasculitis manifestations
5. In patients with severe abdominal pain and/or rectal bleeding (in whom intestinal intussusception has been 4 D
excluded), CS treatment could be considered
6. The dose of oral CS (prednisolone/prednisone) should be 1-2 mg/kg/day (max dose 60 mg/day) 4 D
7. If CS are indicated, pulsed i.v. methylprednisolone (e.g. 10-30 mg/kg with a maximum of 1 g/ day on three 4 D
consecutive days) may be considered for severe cases.
8. Prophylactic CS treatment to prevent the development of IgAV-associated nephritis is not indicated 1B A

Ozen S et al. Rheumatology. 2019;58:1607–16.

IgA Vasculitis
Monitoring

Renal involvement
Ninety percent of children who develop renal involvement do so within two months
of onset, and 97 percent within six months

Renal follow-up
All patients with IgAV (HSP) should be followed with urinalysis and blood pressure
monitoring weekly or biweekly for the first one to two months after presentation.

Additional follow-up for urine and blood pressure monitoring should be scheduled
monthly at first, then every other month until one year after the initial presentation

May is Vasculitis Awareness Month