Vasculitis in Children - types, complications and management • DNB Pediatrics

Author - Shailesh Gophane is DCH from J.J. Hospital, Mumbai and DNB from Port Trust Hospital Mumbai.

“ This is a series of Notes for dnb pediatrics theory exams. Definitely they are not the alternative to reading Nelson's thoroughly, but these notes will prove helpful during final days of revision and may be helpful to overcome any loopholes if you are not having enough time to cover whole system. " December 2015 had 6 repeat questions from these notes.

FAQ: 3

Classify vasculitis. Outline management of HSP including its complications.

(3 + 3 + 4)

Classification of Vasculitis: 3 marks

I. PREDOMINANTLY LARGE VESSEL VASCULITIS

• Takayasu arteritis

II. PREDOMINANTLY MEDIUM VESSEL VASCULITIS

• Childhood polyarteritisnodosa

• Cutaneous polyarteritisnodosa

• 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 polyarteritisnodosa), malignancies, and drugs, including hypersensitivity vasculitis

• Vasculitis associated with connective tissue disease

• Isolated vasculitis of the central nervous system

• Cogan syndrome

• Unclasssified

Management of HSP: ½ marks x 6 = 3 marks

1. Mainly supportive

2. Analgesia may be achieved by NSAIDs

3. Steroids may be used if there is significant gastrointestinal involvement or renal involvement.

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

5. For severe disease Intravenous immune globulin (IVIG) and Plasma pheresis has been tried.

6. chronic HSP renal disease is managed with immunosuppressants like –

Azathioprine

Cyclophosphamide

Mycophenolate Mofetil.

Complications of HSP: 1 marks x 4 = 4 marks

Gastrointestinal - intestinal perforation – immediate complication.

Renal – HSP nephritis - Major long-term complication

Occurs in 1-2% of children with HSP.

Cardiovascular – Hypertension

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