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
- Classify vasculitis Outline management of HSP including its complications Marks - (3 + 3 + 4)
- 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 - 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)
- Classical sign
- Purpura, classically palpable, no itchy Starts with legs and buttocks
- Occur in absence of thrombocytopenia!
- May Occur after a brief urticarial rash
Arthritis (75% of cases)
- Self-limiting and non-deforming
- But severely painful
- knees and ankles are Commonly involved
Abdominal Pain (65% of cases)
- Colicky pain, vomiting
- May mimic acute surgical abdomen
- Easily confused with intussusception
- Intussusception is also a complication (6%)
Nephritis (occurs in 40% of cases)
- Hematuria is the most common feature of HSP nephritis
- Many have gross hematuria
- 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
- Mainly supportive care
- Analgesia may be achieved by NSAIDs
Steroids - Indication of steroids in HSP
- Significant gastrointestinal involvement
- Renal involvement
How to give steroid in HSP?
- Prednisolone (1 mg/kg/day for 1 to 2 wk, followed by a taper)
- 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 -
- Azathioprine
- Cyclophosphamide
- Mycophenolate Mofetil
Complications of Henoch schonlein Perpura
1 marks x 4 = 4 marks
Gastrointestinal
- Intestinal perforation is an immediate complication
- Intussusception (ileoileal)
- Acute pancreatitis
- Bowel Infarct
Renal
- HSP nephritis - Major long-term complication ( Occurs in 1-2% of children with HSP)
- Proteinuria
- 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
- Seizures
- CNS bleeding
- 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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