Routine blood work and imaging are not needed to diagnose HSP but may be obtained in atypical presentations in order to rule out other diagnoses. Initial investigations may include a CBC, urea, creatinine, coagulation studies, and urinalysis (UA). If the UA is positive for hematuria or proteinuria, it should be followed up with a protein/creatinine ratio and further laboratory tests to rule out other causes of glomerulonephritis. Moderate leukocytosis with a mild increase in acute phase reactants is commonly seen. Even though HSP by definition requires a normal platelet count, mild thrombocytosis may be seen in children with GI involvement. Elevated IgA levels are seen in 50% of the patients with HSP, however, it is very nonspecific and there is also no co-relation of the IgA levels with the disease severity. Complement levels are normal.
Biopsy remains the most specific diagnostic tool but is rarely indicated. It is only considered if the diagnosis is uncertain or if the patient has progressive renal disease or nephrotic range proteinuria. Leukoclastic vasculitis affecting the small blood vessels along with perivascular infiltration of neutrophils is typical.
Immunofluorescence shows with IgA deposits along with possible C3 deposits seen in the mesangial wall.
The management of HSP is primarily supportive and involves hydration and pain control with NSAIDs, and rarely with opioids. The treatment with NSAIDs has not shown to increase the risk of GI bleeding. However, in patients with renal involvement and on NSAIDs, close monitoring of renal function and blood pressure is of utmost importance.
The use of steroids in patients with HSP is controversial. Whereas some studies have shown that early use of steroids decreases the duration of abdominal pain and prevents GI and renal complications, other studies have shown no difference.11,12 Immunosuppressive treatment and renal biopsy are reserved for patients with nephrotic range proteinuria and progressive renal impairment. An essential part of following patients with HSP is monitoring them for complications. An abdominal ultrasound is required for patients with new onset severe abdominal pain. Any change in mental status should prompt an evaluation for intracranial hemorrhage.
The prognosis is generally excellent with the majority of the children experiencing complete resolution of the symptoms within 2 to 3 weeks. One-third of the patients experience a recurrence, usually within the first 6 months of diagnosis.13 A recurrence is defined as reappearance of the symptoms after resolution of the disease for at least 2 weeks. It is more likely to happen in children with nephritis. The age of the patient has not been shown to have any association with recurrence risk.
MONITORING AND FOLLOW-UP
Close monitoring for children diagnosed with HSP is essential. Serial urinalysis and blood pressure checks should be performed weekly for the first month, every 2 weeks until 3 months, and monthly until 6 months.10 Renal involvement suggested by hypertension or macroscopic/microscopic hematuria or proteinuria should prompt a nephrology referral. The risk of renal involvement decreases significantly after 6 months.13
The patient in this case is being followed in clinic weekly for urinalysis and blood pressures, which have thus far been negative for hematuria or proteinuria. Her purpuric rash had resolved at about 2 weeks and she now has petechial rashes over the lower extremities. At 3 weeks postdiagnosis, she started complaining of intermittent abdominal pain after meals, and an ultrasound was done that was negative for intussusception. The patient will continue to be monitored as per protocol.
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