1. Hypersensitivity Vasculitis & Henoch-Schonlein Purpura in Children
Iraj Salehi Abari MD.,
Internist
Rheumatologist

2. Hypersensitivity Vasculitis
RRC-Amiralam
Hypersensitivity Vasculitis
Definition:
Vasculitis of small blood vessels of the skin
Due to an immune response or hypersensitivity reaction to an antigen
Other names:
Predominantly Cutaneous Vasculitis
Cutaneous Leukocytoclastic Vasculitis
I. Salehi A.,MD.

3. Pathology: Small blood vessels PMN nuclear debris; Leukocytoclasis
RRC-Amiralam
Pathology:
Small blood vessels
PMN nuclear debris; Leukocytoclasis
Four histologic patterns:
PMN or Mononuclear cell predominant
Necrotizing or Non-necrotizing
I. Salehi A.,MD.

4. Pathogenesis: Immune Complex process CIC, IC deposition RRC-Amiralam
I. Salehi A.,MD.

5. Etiology: Antisera, serum sickness Idiopathic, most common Antigens:
RRC-Amiralam
Etiology:
Antisera, serum sickness
Idiopathic, most common
Antigens:
Drugs: Antibiotics, NSAIDs, Diuretic, Anticonvulsants
Food: Cow’s milk, Chocolate, Cocoa
Infection: HB or HC, Strep., M. TB., HIV
Malignancy( Tumoral Ag ): Rare
I. Salehi A.,MD.

6. Clinical manifestations:
RRC-Amiralam
Clinical manifestations:
Palpable petechiae &/or purpura:
Hallmark
Lower extremities or dependent areas
Other skin manifestations:
Macules, Papules
Vesicles, Bullae, Urticaria
Subcutaneous nodules
I. Salehi A.,MD.

7. Clinical manifestations:
RRC-Amiralam
Clinical manifestations:
Other clinical findings:
Fever, malaise, myalgia
Arthralgia
Lymphadenopathy
Renal: uncommon, mild proliferative GN
Liver, Lung, Heart, CNS: rare
I. Salehi A.,MD.

8. Laboratory Data : No specific laboratory test Mild leukocytosis
RRC-Amiralam
Laboratory Data :
No specific laboratory test
Mild leukocytosis
Low serum complement levels: C3, C4
Mild elevated ESR, CRP
I. Salehi A.,MD.

9. Diagnosis: Hx.: Drugs, infections Ph. Ex.: Skin; Palpable purpura
RRC-Amiralam
Diagnosis:
Hx.: Drugs, infections
Ph. Ex.: Skin; Palpable purpura
Pathology: small vessel vasculitis
R/O HSP:
No IgA deposition in skin or kidney
Low serum complement level
I. Salehi A.,MD.

10. Treatment: Discontinuation of the implicated drug
RRC-Amiralam
Treatment:
Discontinuation of the implicated drug
Treatment of the underlying infection
In severe or persistent disease:
Colchicine, NSAIDs, Dapsone
In fulminant or progressive disease or other organ involvement:
Corticosteroids + Cytotoxic agents
I. Salehi A.,MD.

11. Henoch-Schonlein Purpura( HSP )
RRC-Amiralam
Henoch-Schonlein Purpura( HSP )
Introduction:
The most common vasculitis in children
Ninety percent of cases occur in children
The tetrad of clinical manifestations:
Palpable purpura with Nl Plt. & Nl PT, PTT
Arthritis/ Arthralgia
Abdominal pain
Renal disease
I. Salehi A.,MD.

12. Epidemiology: Age: 3-15 y/o Incidence: 10-20 / 100,000
RRC-Amiralam
Epidemiology:
Age: 3-15 y/o
Incidence: / 100,000
Peak age of incidence:
In 4 to 6 years
70 / 100,000
M/F ratios: / 1
Black < White or asian
Occurs in: Fall, winter, spring
I. Salehi A.,MD.

13. Pathogenesis: Unknown Triggers: Leukocytoclastic + IgA1 ICs
RRC-Amiralam
Pathogenesis:
Unknown
Triggers:
URT with Strep: 50%
Vaccinations
Insect bites
Leukocytoclastic + IgA1 ICs
Increased serum levels of IgA & IgA ICs
I. Salehi A.,MD.

14. Clinical manifestation:
RRC-Amiralam
Clinical manifestation:
Initial presentation:
Lower limb palpable purpura: 75%
Arthralgia/ arthritis: 15%
The classic tetrad:
Palpable purpura: almost all
Arthralgia/Arthritis: 85%
Abdominal colicky pain: 50%
Renal disease: 20-50%
I. Salehi A.,MD.

15. Skin manifestations: Erythematous, macular, or urticarial wheals
RRC-Amiralam
Skin manifestations:
Erythematous, macular, or urticarial wheals
Ecchymoses, petechiae & palpable purpura
Rash; crops, symmetrical, gravity-dependent area
Lower extremities
Buttocks in toddlers
Face, trunks, upper limb in nonambulatory children
Edema in dependent, periorbital area in <3yr
I. Salehi A.,MD.

16. RRC-Amiralam
I. Salehi A.,MD.

17. RRC-Amiralam
I. Salehi A.,MD.

18. Joints: Arthritis / Arthralgia Oligoarticular Transient or migratory
RRC-Amiralam
Joints:
Arthritis / Arthralgia
Oligoarticular
Transient or migratory
Nondeforming
Lower limb, Large joints
1. Rash 2. Arthritis: common
Arthritis without Rash: uncommon
I. Salehi A.,MD.

19. GI. Manifestations: Occur in ½ of children
RRC-Amiralam
GI. Manifestations:
Occur in ½ of children
Mild: nausea, vomiting, abdominal pain, ileus
Severe: GI hemorrhage, Ischemia & Necrosis, Perforation, Intussusception
Guaiac( + )stool: 56%
Massive GI hemorrhage: rare
I. Salehi A.,MD.

20. GI manifestation: Intussusception: Rare GI manifest.:
RRC-Amiralam
GI manifestation:
Intussusception:
The most common GI manifest.
Ileoileal
Rare GI manifest.:
Acute pancreatitis
Gall bladder involvement
Bowel perforation
Protein-losing enteropathy
I. Salehi A.,MD.

21. GI manifestation: 1. Rash 2. GI: 65-85% 1. GI 2. Rash: 15-35%
RRC-Amiralam
GI manifestation:
1. Rash 2. GI: 65-85%
1. GI 2. Rash: 15-35%
GI without Rash: rare
I. Salehi A.,MD.

22. Renal manifestation: Within initial 4 weeks; 20-54%
RRC-Amiralam
Renal manifestation:
Within initial 4 weeks; 20-54%
Hematuria &/or proteinuria
Acute renal failure
Nephrotic syndrome
I. Salehi A.,MD.

23. Other organ involvement:
RRC-Amiralam
Other organ involvement:
Scrotum: Epididymitis, Orchitis
CNS & PNS
Eyes: Keratitis & Uveitis: rare
Respiratory tract:
Impaired lung diffusion capacity: 97%
Interstitial pneumonitis: 69%
Pulmonary hemorrhage: rare
I. Salehi A.,MD.

24. Recurrence: In 1/3 of cases Within 4 months of the initial episode
RRC-Amiralam
Recurrence:
In 1/3 of cases
Within 4 months of the initial episode
Occur more commonly in:
HSP with nephritis
HSP with elevated ESR
HSP need treatment with corticosteroid
I. Salehi A.,MD.

25. Laboratory Data: Serum IgA level is elevated: 50-70%
RRC-Amiralam
Laboratory Data:
Serum IgA level is elevated: 50-70%
Leukocytosis + elevated ESR
Nl Platelet count & Nl coagulation test
U/A
Serum Cr is checking if abnormal U/A
Pathology:
Skin: Leukocytoclastic V. + IgA
Kidney: Mesangial IgA GN
I. Salehi A.,MD.

26. Imaging studies: Plain abdominal X-Ray Abdominal Ultrasonography
RRC-Amiralam
Imaging studies:
Plain abdominal X-Ray
Abdominal Ultrasonography
Barium enemas in intussusception rather than ileoileal
Doppler US in scrotal involvement
I. Salehi A.,MD.

27. Diagnosis: Clinical: Classic Biopsy: Incomplete or unusual
RRC-Amiralam
Diagnosis:
Clinical: Classic
Biopsy: Incomplete or unusual
Pathology: Leukocytoclastic V. + IgA
Renal biopsy:
Dx. Is uncertain
Severe renal involvement
I. Salehi A.,MD.

28. Classification criteria:
RRC-Amiralam
Classification criteria:
ACR criteria:
Palpable purpura
Age at onset < 20 years
Acute abdominal pain
Biopsy showing PMN in the walls of small arterioles &/or venules
> 2 of 4  HSP; sen. & spec. # 90%
I. Salehi A.,MD.

29. Classification criteria:
RRC-Amiralam
Classification criteria:
EULAR & PReS consensus criteria( 2005 ):
Palpable purpura + Nl Plt. + Nl coagulation test plus one or more of the following
Diffuse abdominal pain
Arthritis or arthralgia
Any biopsy with predominant IgA deposition
I. Salehi A.,MD.

30. Treatment: Outpatient: in the most cases NSAIDs: Naproxen
RRC-Amiralam
Treatment:
Outpatient: in the most cases
NSAIDs: Naproxen
Corticosteroid: Prednisolone, Methylpred.
Oral hydration
Bed rest
Symptomatic relief of joint & abdominal pain
I. Salehi A.,MD.

31. Treatment: Hospitalization: Severe Renal involvement:
RRC-Amiralam
Treatment:
Hospitalization:
Severe Renal involvement:
Acute Renal Failure( nephritic )
Significant proteinuria( nephrotic )
Renal Biopsy
HDS + CTX
Severe GI involvement:
Intussusception
GI bleeding
HDS + Surgery
I. Salehi A.,MD.

32. Prognosis: Excellent in children
RRC-Amiralam
Prognosis:
Excellent in children
In follow-up: screening for renal disease
U/A BP
A small minority develop:
Renal complication
GI complication
I. Salehi A.,MD.

33. RRC-Amiralam
I. Salehi A.,MD.