ESPN 50th Annual Meeting

ESPN 2017


 
Recurrent Henoch-Schoenlein Purpura with severe complications: a case report
HAGEN STAUDE 1 ULRIKE JACOBY 1 CHRISTINA HAUENSTEIN 2 FRIEDRICH PRALL 3 MICHAEL RADKE 1

1- DEPARTMENT OF PEDIATRICS, UNIVERSITY HOSPITAL OF ROSTOCK
2- DEPARTMENT OF RADIOLOGY, UNIVERSITY OF ROSTOCK
3- DEPARTMENT OF PATHOLOGY, UNIVERSITY OF ROSTOCK
 
Introduction:

Henoch-Schoenlein purpura (HSP) is the most common vasculitis in childhood. The main symptom is a palpable purpura, but inflammation can also affect joints and multiple organs, especially the gastrointestinal tract and the kidneys. Mostly the disease is self-limiting and has a good prognosis. In complicated cases, treatment is often guided by the degree of renal involvement.

Material and methods:

We report on a 3-year-old girl who presented with palpable purpura and abdominal colicky pain. Due to severe enterocolitis, therapy with prednisolone was started. But despite treatment recurrent episodes with acute abdomen including intraperitoneal bleeding and ileus occurred. She developed a nephritic-nephrotic syndrome (macrohematuria, arterial hypertension, protein/creatinine up to 24 g/g and azotemia with blood urea up to 30 mmol/l) which was treated with mycophenolate initially and due to inadequate response, cyclosporine was added. Four weeks later, complex focal seizures occurred and EEG and cMRT showed severe pathological findings. After a skin biopsy proved IgA-vasculitis, therapy with methylprednisolone-pulse combined with rituximab treatment achieved a remission.

In the next 4 months, she claimed about abdominal distension, intermittent abdominal pain and petechial lesions recurred. A further progress could be stopped by increasing the dose of prednisolone and continuing combined immunosuppressive therapy with cyclosporine and mycophenolate. However, 6 weeks later she developed a severe relapse with acute abdominal pain and nephrotic-nephritic syndrome. Despite monitoring showed complete CD20 depletion in peripheral blood, rituximab treatment in combination with methylprednisolone pulses were again successful. Immunosuppression was continued with daily prednisolone, cyclosporine, mycophenolate and low dose methylprednisolone 200 mg/m2 every 2nd-4th week.

After 6 months during an upper airway infection, there was another episode with acute abdominal pain and nephritic syndrome but without purpura. We decided to stop the regular methylprednisolone infusions and continue rituximab treatment every 3-4 months despite no detectable CD20 in peripheral blood.

Tragically, her father suffered a fulminant type of IgA nephritis with rapid progression to end-stage renal disease 3 years ago.

Results:

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Conclusions:

HSP is mainly a self-limiting disease. Genetic predispositions can increase the likelihood of developing severe complications and aggressive immunosuppressive therapy is required irrespective by the severity of renal involvement.