Schönlein-Henoch Purpura Syndrome

At a glance

Most common inflammatory vascular disease in children that affects small blood vessels with IgA1-dominant immune deposits. Characterized by classical triad of purpuric rash, painful swollen joints, and abdominal pain with vomiting. Frequent nephropathy.

Synonyms

Anaphylactoid Purpura; Allergic Nonthrombocytopenic Purpura; Allergic Purpura-Arthralgia-Gastrointestinal Symptoms; Peliosis Rheumatica; Purpura Abdominalis; Purpura Infectiosa Acuta; Henoch-Schönlein Purpura; Immunoglobulin-A Vasculitis; IgA Vasculitis.

History

Named after Eduard H. Henoch and Johan L. Schönlein, German pediatricians in 1860.

Incidence

The most common vasculitis in children with an incidence established around 10:100,000 per annum. The onset usually occurs beyond age 2 years until adolescence. This medical condition is idiopathic with a sex ration male/female of 3:2.

Pathophysiology

Leukoclastic vasculitis of small vessels, with the potential for necrotizing vasculitis of the kidney and gastrointestinal tract. IgA and C³ deposition is found in the small vessels of the skin and the glomeruli. Etiology is unknown, although it occurs following exposure to infection and drugs.

Diagnosis

Based on clinical findings of rash over the lower extremities and buttocks, arthritis, and colicky abdominal pain. Fifty percent of patients have fever and palpable purpura. IgA deposition may also be found in the skin, and serum titers may also be elevated. It usually presents 1 to 3 weeks after an upper respiratory tract infection. Onset in preschool age with peak in spring and fall, occurring more often in males than females.

Clinical aspects

Rash over lower extremities and buttocks, arthritis, abdominal pain that may mimic an acute abdomen, intussusception, renal disease (10-40%), although usually resolves over the long term. Less frequent clinical features include: pulmonary hemorrhage, convulsions, hemiparesis, mononeuropathies, scrotal vasculitis, and pancreatitis. No specific treatment, although acute attacks may be treated with hydration, nonsteroidal antiinflammatory drugs (NSAIDs), steroids, and immunosuppression. Prognosis favors recovery in weeks to months.

Precautions before anesthesia

Check a full blood count (FBC) and electrolytes. Check potential pulmonary involvement.

Anesthetic considerations

Consider renal dysfunction when choosing drugs. Regional anesthesia is not contraindicated with normal platelet counts or lack of bleeding diathesis. Intravenous access may be difficult if severe tissue edema.

Pharmacological implications

The administration of anesthetic agents or other medications dependent on renal elimination must be avoided. Any medication with potential renal toxic by-products must be avoided in the presence of renal dysfunction.

References