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Henoch-Schonlein purpura (HSP), the most common form of childhood vasculitis, usually runs a benign course, and “most patients are fine even without treatment,” says Norman Ilowite, MD, chief, Pediatric Rheumatology, The Children’s Hospital at Montefiore (CHAM). “But HSP is so common that every pediatrician will see a child who won’t do well either in the short term with GI problems or the long run with the kidneys.”

Four-year-old boy diagnosed with bilateral ankle arthritis, palpable purpura and peticia secondary to HSP

An Auto-Inflammatory Multisystem Disorder
Henoch-Schonlein purpura is a reactive disease that causes inflammation in the skin, joints and kidneys. It aff ects 15 in 100,000 children each year and occurs twice as often in boys as in girls. Incidence peaks in children ages 3 to 10 – 75 percent of all HSP patients are age 9 or younger – and older children may be at higher risk for more serious disease complications.

HSP With No "P"
As would be expected with this eponymous disease, “you can’t make the diagnosis until you see the rash,” says Dr. Ilowite. Sometimes, however, the disease presents without purpura. “It can be confusing,” says Dr. Ilowite, “because you can start with GI or joint disease and it can be difficult to know what’s causing those things until the rash develops.” On very rare occasions, HSP has been diagnosed – through IgA tissue deposition or GI endoscopy – with no purpura.

Gastrointestinal Involvement in Most HSP Patients
Thee most common complaint with HSP is abdominal pain, a presenting sign in 80 percent of all patients. When pain is acute and severe or the patient presents with bloody stools or vomiting, it is essential that “an obstructive series be done to rule out a catastrophic GI event,” says Barry Wershil, MD, chief, Pediatric Gastroenterology, CHAM.

Gastrointestinal complications associated with HSP can include bowel edema, intestinal bleeding and – in 1 to 13 percent of cases – intussusception, a condition that causes the bowel to “fold in on itself,” says Dr. Wershil. “Ordinarily the bowel would simply unfold,” he explains, “but HSP’s inflammation creates a lead point that prevents spontaneous reduction.” Because HSP-associated intussusception is typically small bowel to small bowel – rather than the more common ileo-colonic type – ultrasound of the abdomen rather than barium enema is the preferred imaging modality. Dr. Wershil believes that “a high index of suspicion must be maintained, and the patients must be carefully followed with serial examinations of the abdomen.”

An estimated 2 to 5 percent of HSP patients have serious GI or renal complications, and reportedly 5 to 15 percent of all end-stage renal disease in children is associated with HSP.

Steroid Therapy May Mask Potential Disaster
Experienced care is critical for HSP with abdominal complaints. Steroids are sometimes used to treat GI symptoms in HSP, but Dr. Wershil strongly urges that this therapeutic decision be made by an experienced gastroenterologist because steroidal immunosuppressants can “mask an evolving intra-abdominal disaster,” he says. An undiagnosed intussusception can lead to perforation and peritonitis.

Severe HSP: Lifelong Renal Problems?
The kidneys are HSP’s second most frequently targeted system: Half of all children with HSP will have renal involvement, and 10 percent will develop serious kidney complications. “All patients who present with purpura should have their urine checked,” says Frederick Kaskel, MD, chief, Pediatric Nephrology, CHAM. While mild proteinuria is common, “persistent proteinuria is a poor prognosticator,” says Dr. Kaskel. Patients with hematuria and persistent proteinuria reportedly have a 15 percent chance of developing renal failure.

Kidney complications don’t always show up right away. “You may not have renal involvement at the time of the rash,” says Dr. Kaskel. “It can occur later,” sometimes months or even years after other symptoms resolve. A recent study showed that 35 percent of pediatric patients with severe HSP and glomerulonephritis developed kidney problems as adults.

Highly Experienced Specialty Care Is Crucial
Though life-threatening HSP complications are rare, when emergencies arise, expert sub-specialty evaluation is vital. At CHAM, customized pediatric facilities and proximate care from the metro area’s most experienced pediatric rheumatologists, nephrologists and gastroenterologists give young HSP patients a distinct advantage. Because “for this particular disease, says Dr. Wershil, “what to see – and what to expect – is really based on prior experience.”

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