Dr.SreeLekshmy S, DrSuma Balan(Paed Rheumatology), Dr.Sathyajith, Dr.CJayakumar
Seven yr old female child presented with recurrent episodes of fever, abdominal pain and vomiting for the last 2 months. To begin with child had fever followed purpuric rash over the extremities and multiple episodes of vomiting, for which she was started on oral steroids with a diagnosis of IgA vasculitis . Subsequently as abdominal pain and rashes were persisting he came to AIMS
Differentials considered were :
IgA Vasculitis
ITP,
SLE,
Leukemia
CBC19250k/uL
CRP 1.15
Hb 14.9g%
Platelet 4.3L.
Urine routine normal.
Occult bloodpositive.
C3,C4 141.3/ 27.5 were normal.
USG abdomen normal.
CECT abdomen jejunal wall edema and hyperenhancing mucosa.
Child was started on IVF and given bowel rest.
IV Methylprednisolone (250mg) was started in view of GI vasculitis.
After 3 days vomiting subsided but still had mild abdominal pain.
Enteral fluids started as oral sips.
IV Methylprednisolone (250mg) was given for 3days then 125mg for 2days and then switched to oral prednisolone at a dosage of 2mg/kg.
Her diet was changed to soft diet, which she tolerated.
Plan is to taper and stop steroids over 2weeks and to keep the child on regular followup with urine analysis.
Discussion:
IgA Vasculitis
It is the most common vasculitis of childhood and is characterized leukocytoclastic vasculitis and immunoglobulin A deposition in the small vessels in the skin, joints, gastrointestinal tract, and kidney. Incidence :14-20 per 100,000 children per year.
Affects males more than females, with a 1.2-1.8 : 1 male/female ratio.
90% of HSP cases occur in children, usually between ages 3 and 10 yr.
Exact pathogenesis of HSP remains unknown.
Given the seasonality of HSP and the frequency of preceding upper respiratory infections, infectious triggers such as group A-hemolytic streptococcus, Staphylococcus aureus, mycoplasma, and adenovirus have been suspected.
The hallmark of HSP is its rash, palpable purpura starting as pink macules or wheals and developing into petechiae, raised purpura, or larger ecchymoses. Others include arthritis,arthralgia, abdominal pain, vomiting, diarrhea, paralytic ileus, and melena. Intussusception, mesenteric and intestinal ischemia,intestinal perforation are rare but serious complications. Microscopic hematuria, proteinuria, hypertension, frank nephritis, nephrotic syndrome, and acute or chronic renal failure. Progression to end-stage renal disease (ESRD) is uncommon in children (1-2%).
Neurologic manifestations caused hypertension (posterior reversible encephalopathy syndrome) or central nervous system (CNS) vasculitis may also occur, intracerebral hemorrhage, seizures, headaches,cranial or peripheral neuropathies, and behavior changes. The diagnosis of HSP is clinical.
Classification criteria for HSP
-Palpable purpura (in absence of coagulopathy or thrombocytopenia) and 1 or more of the following criteria must be present:
– Abdominal pain (acute, diffuse, colicky pain)
– Arthritis or arthralgia
– Biopsy of affected tissue demonstrating predominant IgA deposition
– Renal involvement (proteinuria >3 g/24 hr), hematuria or red cell casts.
No laboratory finding is diagnostic of HSP.
Nonspecific findings include leukocytosis, thrombocytosis, mild anemia, and elevated ESR and CRP.
The platelet count is normal in HSP.
Occult blood is frequently found in stool specimens.
Treatment of mild and self-limited HSP is supportive like adequate hydration, nutrition, and analgesia.
Corticosteroids are used in significant GI involvement or other life-threatening manifestations. Glucocorticoids such as oral prednisone (1-2 mg/kg/day), or in severe cases, intravenous (IV) methylprednisolone for 1-2 wk, followed taper, reduces abdominal and joint pain but do not alter overall prognosis. In severe disease, immune globulin (IVIG) and plasma exchange. In Chronic HSP renal disease, immunosuppressants including azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil are used.
Renal disease is the major long-term complication occurring in (1-2%) of children with HSP.
Renal disease can develop up to 6 months after diagnosis but rarely does so if the initial urinalysis findings are normal.
Overall prognosis for childhood HSP is excellent. The long-term prognosis usually depends on the severity and duration of GI or renal involvement.
Take home message
Most of the cases can be managed symptomatically but serious intestinal involvement need short course parental or oral steroids