Outlines: General introduc on Clinical manifesta on Diagnosis Classifica on criteria Lab finding Treatment Complica on& prognosis General introduction: Is the most common vasculi s of childhood. Characterized by leukocytoclas c vasculi s and immunoglobulin A deposi on in the small vessels in the skin, joints, gastrointes nal tract, and kidney. Leukocytoclas c refers to the debris of neutrophils within the blood vessel walls. 90% of HSP cases occur in children, usually between ages 3 and 10 yr. Is more common in the winter. Many cases of HSP follow a documented upper respiratory infec on. The incidence of HSP is es mated at 14-20 per 100,000 children per year and affects males more than females, with a 1.2-1.8 : 1. Skin 100 %, GI 2/3, Joints 75- 85%, 3-4% intussuscep on. 15-35 % of GI manifesta ons precede skin rash. Renal manifesta ons 20-55 %. Immunofluorescence iden fies IgA deposi on in walls of small vessels accompanied to a lesser extent by deposi on of C3, fibrin, and IgM. HSP Done By Dr. Farah Khaled
Clinical manifestations:
The hallmark of HSP is its rash palpable purpura star ng as pink macules or wheals and developing into petechiae, raised purpura, or larger ecchymoses. 100%.
Musculoskeletal involvement, including arthri s and arthralgias, occurs in up to 75% of children
with HSP. Arthri s usually resolves within 2 wk but can recur. Gastrointes nal manifesta ons occur in up to 80% of children with HSP and include abdominal pain, vomi ng, diarrhea, paraly c ileus, and melena. Intussuscep on, mesenteric ischemia, and intes nal perfora on are rare but serious complica ons. Renal involvement occurs in up to 30% of children with HSP, manifes ng as microscopic hematuria, proteinuria, hypertension, frank nephri s, nephro c syndrome, and acute or chronic renal failure.
Progression to end-stage renal disease (ESRD) is uncommon in children (1–2%).
Neurologic manifesta ons of HSP, caused by hypertension (posterior reversible encephalopathy
syndrome) or central nervous system (CNS) vasculi s, may also occur, including intracerebral hemorrhage, seizures, headaches, depressed level of consciousness, cranial or peripheral neuropathies, and behavior changes. HSP Done By Dr. Farah Khaled
Diagnosis:
The diagnosis of HSP is clinical and o en straigh orward when the typical rash is present. Most pa ents are afebrile. Around 25% of cases, the rash appears a er other manifesta ons, making early diagnosis challenging.
Classification Criteria for Henoch-Schoenlein Purpura:
Palpable purpura (in the absence of coagulopathy or thrombocytopenia) and 1 or more of the following criteria must be present:
Laboratory Findings:
No laboratory finding is diagnos c of HSP.
Common nonspecific findings Include: leukocytosis, thrombocytosis, mild anemia, and eleva ons of erythrocyte sedimenta on rate (ESR) and C-reac ve protein (CRP). The platelet count is normal in HSP. Occult blood is frequently found in stool specimens. Serum albumin levels may be low because of renal or intes nal protein loss. HSP Done By Dr. Farah Khaled
Work Up:
Treatment:
Treatment for mild and self-limited HSP is suppor ve (NSAID).
Cor costeroids are most o en used to treat significant GI involvement or other life-threatening manifesta ons. Glucocor coids such as oral prednisone (1-2 mg/kg/day), or in severe cases, intravenous (IV) methylprednisolone for 1-2 weeks, followed by taper. It reduces abdominal and joint pain but does not alter the overall prognosis. Intravenous Immune globulin (IVIG) and plasma exchange are some mes used for severe disease. ESRD develops in <5% of children with HSP nephri s. HSP Done By Dr. Farah Khaled
Management/General Guidance:
Treatment Controversy: Current evidence does not support universal treatment of HSP with cor costeroids, as they do not appear to prevent the onset of renal disease or abdominal complica ons, nor do they alter recurrence rates. However, cor costeroids do seem to have a role in the symptoma c management of HSP, specifically in trea ng abdominal pain, arthralgia, and purpura. Complications: Acutely, serious GI involvement, including intussuscep on and intes nal perfora on, affect significant morbidity and mortality. Renal disease is the major long-term complica on, occurring in 1–2% of children with HSP. Prognosis: Overall, the prognosis for childhood HSP is excellent, and most children experience an acute, self- limited course las ng on average 4 wk. However, 15– 60% of children with HSP experience 1 or more recurrences, typically within 4-6 mo of diagnosis. With each relapse, symptoms are usually milder than at presenta on. HSP Done By Dr. Farah Khaled
Summary:
Definition: small vessel vasculitis “igA mediated”
Clinical features “tetrad” Skin: non-thrombocytopenia palpable purpura. GI: Colicky abdominal pain Renal: Hematuria “glomerular nephri s”. Joints: joint pain. Diagnosis: - It is mainly clinical when a typical rash is present. -elevated ESR& CRP - Mild anemia……………low Hb. -Normal platelets. - Thrombocytosis, leukocytosis. -occult blood in stool specimen. - Serum albumin level is low. -elevated ig A leve. Treatment: - Mild+ self-limited………………...suppor ve NSAIDs/acetaminophen. - Significant GI involvement or life-threatening complica ons…………. Glucocor coids. - Severe disease……………………. IVIG Complications: - Intes nal perfora on. - Renal disease
