Vasculitis in Children | Clinical features, and management of HSP

Table of contents

  • Classification of pediatric vasculitis according to vessel size
  • Clinical features of HSP - Frequency wise
  • Management
  • Complications of HSP
  • Points to remember
  • DP Video wrap

DNB Questions asked

  1. Classify vasculitis Outline management of HSP including its complications Marks - (3 + 3 + 4)
  2. Classify vasculitis based on the size of involved vessels and give examples of each category Describe etiology, clinical features, and management of Takayasu’s arteritis
    (09/2)5+5
  3. HSP- clinical features and diagnosis June 2018 Marks - 5

How to Classify Pediatric Vasculitis based on the size of involved vessels

I PREDOMINANTLY LARGE VESSEL VASCULITIS

  • • Takayasu arteritis

II PREDOMINANTLY MEDIUM VESSEL VASCULITIS

  • • Childhood polyarteritis nodosa
  • • Cutaneous polyarteritis nodosa
  • • Kawasaki disease

III PREDOMINANTLY SMALL VESSEL VASCULITIS

A Granulomatous:

  • • Wegener granulomatosis
  • • Churg-Strauss syndrome

B Nongranulomatous:

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

IV OTHER VASCULITIDES

  • • 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


What are symptopms of Henoch-Schonlein purpura (HSP)

Mechanism

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

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
HSP rash in children
Image.1 - HSP rash - Notice the distribution .

Arthritis (75% of cases)

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

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%)

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

Edema

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


Management of HSP

½ marks x 6 = 3 marks

General management

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

Steroids - Indication of steroids in HSP

  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

For severe disease

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

chronic HSP renal disease

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


Gastrointestinal

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

Renal

  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

CNS

  1. Seizures
  2. CNS bleeding
  3. Mononeuropathies

DP Pearls

Points to remember

  • HSP is the most common vasculitis affecting children
  • Rash is the most common feature
  • Hematuria most common feature of HSP nephritis

DP Video wraps

Best of video's on related topics with the best ingredients

One of the best videos on HSP


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auhtor

About Author

Shailesh Gophane | DCH DNB Pediatrics

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