Henoch Schonlein Purpura Flashcards

1
Q

What is Henoch-Schonlein purpura (HSP) and how is it associated with IgA nephropathy?

A

Henoch-Schonlein purpura (HSP) is an IgA mediated small vessel vasculitis often seen in children after an infection. It shares similarities with IgA nephropathy (Berger’s disease).

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2
Q

What are the typical features of Henoch-Schonlein purpura?

A

Features of HSP include a palpable purpuric rash over buttocks and extensor surfaces of arms and legs, abdominal pain, polyarthritis, and sometimes signs of IgA nephropathy such as haematuria and renal failure.

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3
Q

What is the typical treatment approach for Henoch-Schonlein purpura?

A

Treatment for HSP is largely supportive, focusing on analgesia for arthralgia. The use of steroids and immunosuppressants is inconsistent and typically reserved for managing nephropathy if it develops.

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4
Q

What is the prognosis for patients with Henoch-Schonlein purpura?

A

The prognosis for HSP is generally excellent, as it is usually a self-limiting condition in children, particularly those without renal involvement. Monitoring of blood pressure and urinalysis is important to detect any progressive renal involvement. About one-third of patients may experience a relapse.

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5
Q

A 13-year-old girl develops purpura on her lower limbs and buttocks associated with microscopic haematuria. A diagnosis of Henoch-Schonlein purpura is made. Her urea and electrolytes show mild renal impairment that is still present 4 weeks later, although she does not require any specific therapy. What is the most likely renal outcome?

Hypertension within 20 years
Persistent proteinuria
End stage renal failure
Full renal recovery
Frequent relapses

A

Full renal recovery

The most likely renal outcome for the 13-year-old girl with Henoch-Schonlein purpura (HSP) and mild renal impairment is full renal recovery. HSP, also known as IgA vasculitis, is a small-vessel vasculitis that predominantly affects children. While it can cause kidney involvement in the form of IgA nephropathy, most cases of HSP with renal involvement have a good prognosis and recover fully without any long-term complications.

Hypertension within 20 years is an unlikely outcome in this case. Although hypertension can develop in some patients with HSP who have severe kidney involvement, it is not common in those with mild renal impairment like our patient.

Persistent proteinuria could occur if there was significant damage to the glomeruli; however, given the mild nature of the renal impairment in this patient, persistent proteinuria is less likely. Most patients with mild kidney involvement will recover fully without any residual proteinuria.

End stage renal failure is a rare outcome in HSP patients, occurring only in a small percentage of cases where there is severe kidney involvement. In this patient’s case, her mild renal impairment does not indicate that she would progress to end-stage renal failure.

Finally, frequent relapses are also uncommon in HSP patients. The majority of cases are self-limiting and do not recur after resolution. Moreover, even if relapses do occur, they are typically less severe than the initial presentation and often resolve spontaneously without causing further damage to the kidneys.

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