Medicines for Henoch-Schonlein purpura

What it is

Henoch-Schonlein purpura (HSP), also called IgA vasculitis, is a condition in which small blood vessels become inflamed (a type of vasculitis), causing them to leak, which produces a characteristic rash and can affect several parts of the body. It is most common in children (particularly young children), though it can occur in adults. The exact cause is not fully understood, but it often follows an infection (such as a common cold or throat infection), suggesting it may be triggered by the immune system’s response to an infection; it involves a particular antibody (IgA). HSP has several characteristic features, which can include: a distinctive rash — of raised red or purple spots (purpura), which are caused by bleeding under the skin from the inflamed vessels, and which typically appear on the legs and buttocks (and sometimes elsewhere); joint pain and swelling (often the knees and ankles); tummy (abdominal) pain, which can sometimes be significant, and other digestive symptoms; and, importantly, effects on the kidneys, as the inflammation can affect the small blood vessels in the kidneys, which is why monitoring the kidneys is a key part of care. Not everyone has all the features. HSP is usually a self-limiting condition — in most children, it settles on its own over a few weeks, and most children make a full recovery without any lasting problems. The rash and other symptoms typically resolve, though HSP can sometimes recur (relapse) in the weeks or months afterwards in some children. The main concern, and the reason for follow-up, is the possible effect on the kidneys: while most children’s kidneys are not significantly affected or recover fully, in a small proportion the kidneys can be affected, sometimes with longer-term effects, so monitoring the kidneys (with urine and blood pressure checks) during and after the illness is an important part of care. Treatment is often mainly supportive (managing symptoms), as the condition usually settles by itself, though treatment may be needed for particular problems or for kidney involvement in some cases. The key messages are that Henoch-Schonlein purpura is inflammation of small blood vessels (mainly in children) causing a characteristic rash, joint pain, and tummy pain, that it usually settles on its own, and that the kidneys are monitored as an important part of care.

How it is treated

Henoch-Schonlein purpura is usually managed with supportive care as it typically settles on its own, with monitoring of the kidneys (and blood pressure) being a key part of care, and treatment for particular problems or kidney involvement where needed. Diagnosis is usually based on the characteristic features — particularly the typical rash (raised red or purple spots on the legs and buttocks), often with joint pain, tummy pain, and the context (frequently following an infection, and mainly in children) — and sometimes supported by tests (such as urine tests, blood tests, and, occasionally, other tests) to assess for kidney involvement and to help confirm the diagnosis and exclude other causes. Because HSP is usually self-limiting, treatment is often mainly supportive: managing the symptoms — such as rest, pain relief for the joint and tummy pain, and general supportive care — while the condition settles, usually over a few weeks; most children recover fully. A key and important part of care is monitoring the kidneys, because HSP can affect the kidneys, sometimes with longer-term effects in a small proportion of cases: this involves checking the urine (for blood or protein, which can indicate kidney involvement) and blood pressure during the illness and for a period afterwards (as kidney involvement can develop or persist), so that any kidney problems are detected and managed; the exact schedule of follow-up is guided by the doctors. Where the kidneys are significantly affected, further assessment and treatment (which may involve specialists, and, in some cases, specific treatments) are provided. Treatment may also be needed for particular problems — for example, more significant tummy symptoms or complications, or severe joint symptoms, may need specific treatment, and, in certain situations, medicines such as steroids may be used for particular aspects (guided by the doctors). Because HSP can sometimes recur in the weeks or months afterwards, being aware of this, and continuing follow-up (particularly of the kidneys) as advised, are important. For adults with HSP, and for more severe or atypical cases, assessment and management may be more involved, and specialist input may be needed. It is important to seek assessment for the characteristic features, particularly the rash (which should be checked, as a purpuric rash can have other, sometimes serious, causes that need excluding), and to attend the kidney monitoring. The reassuring messages are that Henoch-Schonlein purpura is usually a self-limiting condition that settles on its own over a few weeks with supportive care, that most children make a full recovery, and that monitoring the kidneys (an important part of care) allows any kidney involvement to be detected and managed; so supportive care, kidney monitoring, and treatment for any particular problems or kidney involvement are the keys to managing HSP.