Good Morning All!!Morning Report: Thursday, November 10th,

2011

Henoch-SchÖnleinPurpura

Epidemiology Most common systemic vasculitis of

childhood Incidence 10/100,000 children/yr

Average age of occurrence is 6 yo Majority of cases <10 yo

Affects boys more commonly (2:1) More cases in the winter and spring

Etiology Take a guess…UNKNOWN!

Many suspected “triggers”: Allergies Bacteria

Streptococcus pyogenes Viruses

50% of cases preceeded by a URI Drugs

PCN Cephalosporins Thiazide diuretics

Vaccines Insect bites

Pathophysiology Leukocytoclastic vasculitis

Aka: small vessel vasculitis

IgA accumulation in vessel walls and the renal mesangium Leads to inflammation and necrosis of

predominantly postcapillary venules, capillaries and arterioles

Clinical PresentationNonthrombocytopenic purpura

Arthritis

Abdominal pain/ GI bleeding

Renal disease

HSP

Nonthrombocytopenic Purpura

The Rash… Palpable purpuric lesions seen in 100% of

patients Presenting symptom in 50%

Usually found on the lower extremities in the dependent, pressure-bearing areas Buttocks especially prone Lesions can also occur on UE, face and trunk

Angioedema of the scalp/face,extremities, and GU region occurs in 20-46% of patients

The Rash…

Deep red macules palpable purpura or hemorrhagic bullae

Arthritis and/or Arthralgia

Joint Complaints… Occur in 50-80% of patients

Presenting symptom in 25% Periarthritis

Edema AROUND the joints and inflammation involving the tendon sheaths

Affected joints may be swollen, tender and painful on motion

Most commonly affects knees and ankles

GI Disease

GI Unpleasantness… GI disease noted in 67% of affected children

Due to submucosal and subserosal hemorrhage Most common manifestations:

Colicky abdominal pain +/- vomiting

Stools with gross or occult blood Can also see:

Intussusception 4-5% of patients Ileoileal in location rather than ileocolic

GI Unpleasentness… Can also see (con’t):

Bowel infarction/ perforation Pancreatitis Hydrops of the gallbladder

*Renal Disease

Renal Manifestations… Occur in up to 40-50% of patients with HSP Most present in the first month following rash

BUT can occur anytime within 3-4 months Histopathology very similar to Berger disease

IgA deposition *Wide range of clinical expression:

Microscopic hematuria (most common) Mild proteinuria Nephritic and/or nephrotic syndrome

Renal Manifestations Prognosis

Hematuria alone No long-term sequelae

Combination nephritic/nephrotic syndromes 50% of these patients develop ESRD within 10 years *Persistence of nephrotic-range proteinuria is the most

accurate predictor of eventual renal failure *Overall, progression to renal failure is seen in

only 1-5% of patients with HSP

*Less Common Features of HSP

Orchitis CNS symptoms

HA Seizures Coma

Guillain-Barre syndrome Parotitis Carditis Pulmonary hemorrhage

*Lab Findings Non-specific

CBC Nml or elevated WBC ct Eosinophilia Anemia (secondary to bleeding) Elevated plt ct

BMP Electrolyte disturbances due to GI disease Elevated BUN/Cr with renal disease

UA Microscopic hematuria (*most common lab finding*) Proteinuria

Elevated inflammatory markers Decreased Factor VIII levels

Normal PT/PTT

Treatment Supportive Care

NSAIDs for pain management Avoid with significant GI or renal disease

Maintain hydration, nutrition, electrolyte balance Glucocorticoids

Controversial!! Quickly improve abdominal pain and severe scrotal

swelling/edema No RCT have shown efficacy for HSP nephritis

?May decrease the chance of persistent renal disease ?May reduce risk of intussusception

Prognosis 67% of children with HSP run the course of the

disease within 4 weeks of onset Recurrence in 25% of patients

Usually manifests as rash and abdominal pain after a respiratory infection

Significant morbidity Acute GI bleeds Long-term renal involvement

Need to f/u with frequent UAs and BP measurements for the first 4 months after presentation Most children who develop abnormal urine findings do so

within 4 months of diagnosis

Thanks for your attention!Noon Conference: Dr. Mouallem, Fractures