HSP treatment

Henoch-Schonlein purpura or HSP is a condition seen mainly in children, with 75% of cases occurring between the ages of 2 and 11, although cases in adults do occur. It is more common in boys with a boy/girl ration of 2:1. HSP is not a common disease, in the USA there are 14 cases in every 100,000 children of school age.

It is a vasculitis caused by the deposition of immunoglobin A (IgA), C3 and immune complexes in the small blood vessels (arterioles, venules and capillaries). This causes purpura and nephritis (kidney inflammation).



The main symptom of HSP is a red rash caused by point bleeding into the skin (purpura) this is particularly noticeable on the lower limbs. Other symptoms include a painful edema (in males this often involves the scrotum), joint pain (particularly the knees an ankles), abdominal pain and blood in the stools and urine. In less than 1% of cases, the patient will develop renal failure.

At present, the exact cause of HSP is unknown. Infections, insect bites, drugs and even certain foods may trigger the start of the condition. Clinical features, with a blood test to exclude idiopathic thrombocytopenic purpura (ITP) or thrombotic thrombocytopenic purpura (TTP), will diagnose HSP.

Treatment of HSP is a matter of treating the symptoms while monitoring the patient for complications such a renal failure or abdominal intussusceptions (abnormal folding of the bowel).

Most cases of HSP are treated with pain relieving non-steroid anti-inflammatory drugs (NSAIDS). It is important to monitor the patient's kidney function when using NSAIDS in case they develop renal insufficiency. The NSAID of choice is ibuprofen (trade names Ibuprin, Advil or Motrin). Other NSAIDS of use in treating HSP include flurbiprofen (Ansaid), ketoprofen (Oruvail, Orudis or Actron) and naproxen (Anaprox, Naprelan or Naprosyn).

Severe cases of HSP can be treated with either intravenous or oral corticosteroids such as prednisone (Deltasone, Sterapred or Orasone) or methyloprednisolone (Solu-Medrol or Depo-Medrol). Steroid treatment is often combined with other therapeutic agents or procedures such as fish oil, azathioprine (Imuran), cyclophosphamide (Cytoxan), cyclosporine, dipyridamole, plasmapheresis or high-dose intra-venous immunoglobulin G (IgG).

Cases of renal failure in HSP will require dialysis treatment.

The best option for treatment of HSP is in doubt. To date there are no large-scale studies of treatment options for HSP in published literature. The small number of cases and many of the being children make performing full scale clinical trials of HSP medication a difficult task.

Most cases of HSP will resolve without any complications. 10 to 20 percent of patients will experience a subsequent bout of HSP while fewer than 5% will develop long term or chronic HSP.