1. Henoch-Schönlein Purpura

2. WHAT IS Henoch-Schönlein Purpura  Also called anaphylactoid purpura  Henoch-Schönlein purpura (HSP) is the most common form of vasculitis in children. This small-vessel vasculitis is mediated by immunoglobulin A (IgA)-containing immune complexes and is characterized by  nonthrombocytopenic, Palpable purpura  Arthralgias  GI involvement, abdominal pain  Renal involvement

3. Background  1 st described in 1801 by William Heberden, a physician in London, who wrote about a case of 5 years old boy with hematuria, abdominal pain, joint pains and skin rashes

4.  In 1837, Johann Schonlein and later in 1874, Edouard Henoch described multiple case reports of similar cases  They also showed an association of upper respiratory tract infection preceding development of symptoms

5. EPIDEMIOLOGY  Peak : in children 4 – 7 years of age  Male to female ratio: 1.2 : 1  Renal involvement is more severe in adults

6. Pathogenesis  Thought to be an immune-complex mediated disease with deposition of IgA in glomerular capillaries, dermal capillaries and GIT  Mesangial deposits of IgA are the same as those

7. Clinical manifestations  In 2005 the European League Against Rheumatism (EULAR) and the Pediatric Rheumatology European Society (PReS) listed classification criteria for Henoch Schönlein purpura (HSP)  Purpura or petechiae with lower limb predominance and at least one of the following:  Arthritis or arthralgias  Abdominal pain  Histopathology demonstrating IgA deposition  Renal involvement (hematuria or proteinuria)

8. skin  Palpable purpura  Commonly seen on lower limbs and buttocks,however can also been seen on the trunk and arms  Lesion initially erythematous macules and progress to non-blanching mom-pruritic purpuric lesion  Leukocytoclastic vasculitis with IgA deposition

10. Joints: Arthralgia/ arthritis  2 nd common presentation 84%  Usually transient or migratory  Oligoarticular  Non deforming  Lower extremity large joints  Arthritis affects three-quarters of children and the most commonly affected joints are the knees and ankles. The arthritis is usually oligoarticular, self-limited, and non-destructive. It is the presenting symptom in 15% of patients.

11. The gastrointestinal  affect 50 to 75% of children  Typically develop within 8 days of the appearance of rash  The gastrointestinal (GI) manifestations of HSP and may include bleeding, intussusception, and abdominal pain.  GI bleeding: Intestinal bleeding, manifested as gross or occult blood per rectum, occurs in approximately one- third of children  Intussusception is a common complication in children, it occurs in 1 to 5% of children and is mostly ileo-ileal in location

12. Renal  Ranges from 21 – 54%  Microscopic Hematuria with or without red cell cast  Proteinuria ranges from mild to nephrotic range  Elevated S. creatinine and/ or HTN  Renal disease rarely precedes the onset of rash. Children may present with nephritic or nephrotic syndrome, or rarely renal failure.  The majority of children who develop renal disease do so within the first 6 weeks and 97% within 6 months  risk of chronic renal impairment and end-stage renal disease is 2– 15% and less than 1%, respectively.

13. Other organs  CNS including intracerebral Hge, seizures, stroke, and mental status changes.  Pulmonary He  edema of scrotum, eyes, or hands  Keratitis and Uveitis

14. Diagnosis  LAB:  CBC normal plt count  serum IgA ( 50 – 70 %)  Abdominal U/S  Biopsy :Renal / skin

15.  Renal biopsy is reserved for patients in whom the diagnosis is uncertain or evidence of severe renal impairment  Skin biopsy including small blood vessels of superficial dermis

16. Differential Diagnosis  other causes of purpura in childhood including  acute hemorrhagic edema of infancy,  immune thrombocytopenic purpura,  acute post-streptococcal glomerulonephritis,  hemolytic-uremic syndrome,  disseminated intravascular coagulation  hypersensitivity vasculitis.

17.  Hypersensitivity vasculitis is also known as cutaneous leukocytoclastic vasculitis and microscopic polyarteritis. It typically affects the small vessels and is idiopathic or triggered by infection or drug exposure. Clinical manifestations include urticaria, purpura, or a maculopapular rash, arthralgias, hypocomplementemia, and elevated inflammatory markers

18. Management  Supportive Care : include adequate Hydration, rest and pain relief

19. Management  Admission for indicated cases :  Severe abdominal pain  GI bleeding  Elevated creatinine, HTN and /or nephrotic  Severe Joint involvement  Changes in Mental status

20. Management  Symptomatic treatment  NSAID: Ibuprofen

21. Management  Steroid  Their use in PT with HSP is controversial  New literature supports that in the hospital setting, early use of corticosteroids for HSP is associated with improved outcomes, particularly GI co-morbidities

22. Management  Disease modifying agents  In life-threatening cases or acute renal failure, plasmapheresis followed by a more potent immunosuppressive agent such as cyclophosphamide, azathioprine, or cyclosporine, should be considered.

23. Prognosis  Generally in children : a milder course with shorter duration  Spontanous resolution occurs in up to 94 % within 4 weeks  Renal manifestation can present up to 6 months following onset of skin lesion  Protienuria > 1gm /day with worse prognosis if develop nephrotic syndrome  Risk of chronic renal impairment and end-stage renal disease is 2– 15% and less than 1%, respectively  Recurrence in up to 40%