Henoch Schonlein Purpura
-
Upload
yellowsunfire102 -
Category
Health & Medicine
-
view
4.431 -
download
5
description
Transcript of Henoch Schonlein Purpura
![Page 1: Henoch Schonlein Purpura](https://reader036.fdocuments.in/reader036/viewer/2022082702/55506eefb4c90524138b4a01/html5/thumbnails/1.jpg)
Henoch Schonlein Purpura
Ahmad Abid Abas 2
![Page 2: Henoch Schonlein Purpura](https://reader036.fdocuments.in/reader036/viewer/2022082702/55506eefb4c90524138b4a01/html5/thumbnails/2.jpg)
What is HSP?
• Anaphylactoid purpura,Purpura rheumatica, Leukocytoclastic vasculitis.
• It is an inflammatory disorder characterized by a generalized vasculitis involving the small vessels of the skin, GI tract, kidneys, joints, and, rarely, the lungs and CNS.
• Mainly affects children 3-15 yr age.Rarely below 2 yr age.
![Page 3: Henoch Schonlein Purpura](https://reader036.fdocuments.in/reader036/viewer/2022082702/55506eefb4c90524138b4a01/html5/thumbnails/3.jpg)
Etiopathogenesis
• Unknown etiology. Multifactorial with genetic, environmental and antigenic components.
• More than 75% of patients report antecedent upper-respiratory, pharyngeal, or GI infections.
• An immunoglobulin A (IgA)–mediated autoimmune phenomenon. (clinical manifestation result fr this Ag-Ab complex)
![Page 4: Henoch Schonlein Purpura](https://reader036.fdocuments.in/reader036/viewer/2022082702/55506eefb4c90524138b4a01/html5/thumbnails/4.jpg)
How to Approach ?
• c/o – Usually the rash is the first to appear and the child is brought to medical attention by his/her parents.
• Hx – Prodromal symptoms such as headache,fever,anorexia.
• O/E – Rash --> Morphology (palpable) ?,(What type cutaneous lesions (petichea,ecchymosis)?,Site of distribution (thighs,buttocks) ?
![Page 5: Henoch Schonlein Purpura](https://reader036.fdocuments.in/reader036/viewer/2022082702/55506eefb4c90524138b4a01/html5/thumbnails/5.jpg)
How to Approach ?
• Other clinical features to be considered :1) Abdomen – crampy abdominal pain.2) Joints – migratory arthralgia,arthritis in ankles,knees (big joints) – expect periarticular swelling + significant pain.3) Renal – (HTN,edema,oliguria and hematuria +/- azotemia) similar CP of APSGN.
![Page 6: Henoch Schonlein Purpura](https://reader036.fdocuments.in/reader036/viewer/2022082702/55506eefb4c90524138b4a01/html5/thumbnails/6.jpg)
How to Approach ?Dx - Palpable purpura (mandatory) in the presence of at least one of the following four features:
• Abdominal pain• Arthritis (acute) or arthralgia• Renal involvement (any haematuria and/or
proteinuria)• Any biopsy showing predominant Iga deposition
![Page 7: Henoch Schonlein Purpura](https://reader036.fdocuments.in/reader036/viewer/2022082702/55506eefb4c90524138b4a01/html5/thumbnails/7.jpg)
What to be expected in Investigations?
• Electrolyte values Normal unless excess vomit.• BUN/creatinine(RFT) – May ↑ in presence renal
involvement.• CBC – (exclude thrombocytopenia) leukocytosis
possibly eosinophilia,Platelet counts N or ↑.Hb or Ht values may be N or ↓ 2ry to bleeding.
• Urinalysis – (hematuria, proteinuria, and occasional red cell casts.) (like APSGN)
![Page 8: Henoch Schonlein Purpura](https://reader036.fdocuments.in/reader036/viewer/2022082702/55506eefb4c90524138b4a01/html5/thumbnails/8.jpg)
How to Approach ? Criteria ITP Acute
LeukimiaAplastic Anemia
HSP
Hb/RBC count Normal ↓ ↓ Normal
WBC count Normal ↑↑↑ ↓ ↑ in some cases.
Platelet count ↓ ↓ ↓ Normal
![Page 9: Henoch Schonlein Purpura](https://reader036.fdocuments.in/reader036/viewer/2022082702/55506eefb4c90524138b4a01/html5/thumbnails/9.jpg)
How to narrow DDx ? • Tourniquet test [Hge tendency,capillary fragility]• Antistreptolysin-O (ASO) titer [recent Strept. infection]• Monospot test [rapid test for Mono. (EBV)]• Antinuclear antibody (ANA) test [exclude SLE]• Rheumatoid factor (RF) test [exclude RA]• Determination of C3/C4 levels (diff. fr APSGN)• Prothrombin time (PT) and activated partial
thromboplastin time (aPTT)• Blood cultures [potential infection,meningitis)• Abd U/S – [Evaluate Intussusception & exclude
appendicitis]
![Page 10: Henoch Schonlein Purpura](https://reader036.fdocuments.in/reader036/viewer/2022082702/55506eefb4c90524138b4a01/html5/thumbnails/10.jpg)
How to Manage ?• Rx is largely supportive.• Abdominal pain – Common and self-limited.• Joint pain – NSAID agents• Cutaneous manifest. – self-limited may relapse.• Ch. Cutaneous lesion –
colchicine,aspirin,systemic CST.• No optimal management for GIT and Renal
involvement.Should be follow up closely.
![Page 11: Henoch Schonlein Purpura](https://reader036.fdocuments.in/reader036/viewer/2022082702/55506eefb4c90524138b4a01/html5/thumbnails/11.jpg)
Conclusion (clinical point of view)
HSP is diagnosed clinically.GI involvement is early symptom and renal disease is late symptom.If occur in adults , it is more severe.Palpable purpura,abd pain and arthritis is clinical characteristic triad of HSP in pediatric.
![Page 12: Henoch Schonlein Purpura](https://reader036.fdocuments.in/reader036/viewer/2022082702/55506eefb4c90524138b4a01/html5/thumbnails/12.jpg)
References
• 1) The Indian Academy of Pediatrics,Bulletin of Rheumatology Chapter IAP September 2010.
• 2)http://emedicine.medscape.com/article/804681-clinical
• 3) http://www.orpha.net/data/patho/GB/uk-HSP1.pdf