Vasculitis in Children | Clinical features, and management of HSP

Classification of pediatric Vasculitis based on the size of involved vessels

• Takayasu arteritis

• Childhood polyarteritis nodosa
• Cutaneous polyarteritis nodosa
• Kawasaki disease

Small vessel vasculitis can be further classified as

A. Granulomatous:

• Wegener granulomatosis
• Churg-Strauss syndrome

B. Non-granulomatous:

• Microscopic polyangiitis
• Henoch-Schonlein purpura
• Isolated cutaneous leukocytoclastic vasculitis
• Hypocomplementemic urticarial vasculitis

• Behcet disease
• Vasculitis secondary to infection (including hepatitis B–associated polyarteritis nodosa), malignancies, and drugs, including hypersensitivity vasculitis
• Vasculitis associated with connective tissue disease
• Isolated vasculitis of the central nervous system
• Cogan syndrome
• Unclassified

Henoch-Schonlein purpura

HSP is the most common vasculitis affecting children

Mechanism

widespread leukocytoclastic vasculitis from IgA deposition in blood vessel walls leads to clinical features

1.Rash (100% of cases)

1. Classical sign
2. Purpura, classically palpable, no itchy Starts with legs and buttocks
3. Occur in absence of thrombocytopenia!
4. May Occur after a brief urticarial rash

Image.1 - HSP rash - Notice the distribution

2.Arthritis (75% of cases)

1. Self-limiting and non-deforming
2. But severely painful
3. knees and ankles are Commonly involved

3.Abdominal Pain (65% of cases)

1. Colicky pain, vomiting
2. May mimic acute surgical abdomen
3. Easily confused with intussusception
4. Intussusception is also a complication (6%)

4.Nephritis (occurs in 40% of cases)

1. Hematuria is the most common feature of HSP nephritis
2. Many have gross hematuria
3. Can lead to chronic renal issues

5.Edema

Edema of the scalp, eyelids, back, feet, and/or perineum

Management of HSP

½ marks x 6 = 3 marks

1. Mainly supportive care
2. Analgesia may be achieved by NSAIDs

1. Significant gastrointestinal involvement
2. Renal involvement

How to give steroid in HSP?

1. Prednisolone (1 mg/kg/day for 1 to 2 wk, followed by a taper)
2. Reduces abdominal and joint pain but does not alter overall prognosis nor prevent renal disease

Intravenous immune globulin (IVIG) and Plasmapheresis have been tried.

Chronic disease is managed with immunosuppressants like -

1. Azathioprine
2. Cyclophosphamide
3. Mycophenolate Mofetil

Complications of Henoch schonlein Perpura

1 marks x 4 = 4 marks

1. Intestinal perforation is an immediate complication
2. Intussusception (ileoileal)
3. Acute pancreatitis
4. Bowel Infarct

1. HSP nephritis - Major long-term complication ( Occurs in 1-2% of children with HSP)
2. Proteinuria
3. End-stage renal disease (1%)

Cardiovascular

Hypertension Children with HSP should undergo serial monitoring of blood pressure and urinalyses for 6 months after diagnosis, especially those who presented with hypertension or urinary abnormalities

1. Seizures
2. CNS bleeding
3. Mononeuropathies

More frequently asked questions

1. Pediatric Vasculitis. Pediatr Clin North Am. 2012 Apr; 59(2): 407–423. (full article)
2. HSP Guidelines -Royal children Hospital Melbourne (Good read for diagnostic approach)
3. Vasculitis in Children - Despina Eleftheriou, Ezgi Deniz Batu, Seza Ozen, Paul A. Brogan, Vasculitis in children, Nephrology Dialysis Transplantation, Volume 30, Issue suppl_1, April 2015, Pages i94–i103,

Image [Source 1] - Wikimedia commons

Author

Shailesh Gophane | DCH DNB Pediatrics

Shailesh completed his Pediatric residency from Port Trust Hospital Mumbai after completing DCH from J.J. Hospital, Mumbai