• Clinical science

Henoch-Schonlein purpura (Anaphylactoid purpura…)

Abstract

Henoch-Schonlein purpura (HSP) is an acute immune complex-mediated small vessel vasculitis that most commonly occurs in children. It is often preceded by an upper respiratory tract infection and typically presents with a tetrad of symptoms: palpable purpura, arthritis/arthralgia, abdominal pain, and renal disease. However, in any individual patient, only some of the classic tetrad of symptoms may be present. HSP is a clinical diagnosis, but in particularly unclear or atypical cases a biopsy may be used to confirm the diagnosis. Because the disease course is usually self-limiting, treatment is generally supportive. Severe cases may require glucocorticoids, antihypertensive drugs, and possibly dialysis. HSP has an excellent prognosis, usually resolving within one month when not complicated by significant renal disease.

Epidemiology

  • Sex: >
  • More common in children
    • 90% of cases < 10 years old
    • Peak incidence: 6 years

References:[1][2]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

  • The exact pathogenesis is unknown and assumed to be multifactorial. Factors that likely play a role include:
    • Preceding infection
      • > 75% of cases preceded by viral or bacterial infection 1–3 weeks prior
      • Most commonly an upper respiratory tract infection caused by group A streptococcus
      • GI infections also possible; many other organisms have also been associated with HSP
    • Genetic predisposition
    • Drugs (e.g., some antibiotics and antiarrhythmics) and vaccinations (e.g., yellow fever, cholera)

References:[3]

Pathophysiology

  • Hypothesized pathophysiological mechanism: exposure to allergen/antigen (e.g., infection, drugs) → stimulation of IgA production → deposition of IgA immune complexes in vascular walls (e.g., in the skin, GI tract, joints, kidneys) → activation of complement → vascular inflammation and damage

References:[3]

Clinical features

History

  • Symptom onset often 1–3 weeks after an infection, typically affecting the upper respiratory tract

Manifestations

  • Skin (∼ 100% of cases)
    • Symmetrically distributed, raised, erythematous macules or urticarial lesions that coalesce into palpable purpura (non-blanching skin lesions)
    • Most common sites: the lower extremities, buttocks, and other areas of pressure or constraint (e.g., from socks or clothing)
  • Joints (∼ 75% of cases): arthritis/arthralgia, most common in the ankles and knees
  • Gastrointestinal tract (∼ 60% of cases)
  • Kidneys (∼ 50% of cases): HSP nephritis with signs and symptoms of nephritic syndrome
  • Other organs
    • Scrotum (e.g., scrotal swelling, pain, and tenderness)
    • Central and peripheral nervous system (e.g., headaches, seizures, focal neurologic deficits, ataxia, intracerebral hemorrhage, central and peripheral neuropathy)
    • Respiratory tract (e.g., mild interstitial changes, pulmonary hemorrhage)
    • In rare cases, the eyes (e.g., keratitis, uveitis)

HSP is characterized by a tetrad of clinical findings: palpable purpura, arthritis/arthralgia, GI symptoms, and renal disease
HSP is one of the important differential diagnoses to consider in cases of pediatric limp!

References:[1][2][4]

Diagnostics

HSP is a clinical diagnosis; , laboratory tests are not essential to HSP diagnosis. However, they may be useful for excluding differential diagnoses in patients exhibiting only one or two of the HSP tetrad of symptoms, as is often the case in the first few days. Laboratory tests are also useful for monitoring the extent of renal involvement, which helps determine the prognosis. Definitive diagnosis in uncertain cases is made via biopsy.

Laboratory tests

The platelet count in HSP is normal or elevated, as opposed to other causes of purpura!

Imaging

  • Indication: performed in patients with marked abdominal symptoms or suspected complications
  • Abdominal ultrasound/CT
  • Endoscopy to evaluate GI bleeding
  • Arteriography in cases of hemorrhage
  • Barium enema if intussusception is suspected
  • Head MRI in cases of CNS involvement

Biopsy

  • Indications: reserved for patients with unusual skin presentations or severe renal involvement (e.g., persistent nephrotic syndrome)
  • Skin: Leukocytoclastic vasculitis with IgA and C3 immune complex deposition (hallmark) in small vessels of the superficial dermis
  • Kidney
    • Renal involvement characterized by mesangial IgA deposition; C3 and fibrin are also frequently present.
    • Crescent formation in more severe cases

References:[1][2][3][5]

Differential diagnoses

HSP is a unique cause of purpura without thrombocytopenia!

Purpura

Arthritis/Arthralgia

Renal disease

References:[1][3]

The differential diagnoses listed here are not exhaustive.

Treatment

Most cases of HSP are self-limiting and only require supportive care; (e.g., pain management) with regular outpatient follow-up. Severe HSP requires hospitalization and intensive medical therapy.

Mild disease

  • Outpatient treatment
  • Usually no treatment necessary
  • NSAIDs for pain management, rest, and adequate hydration
  • Discontinuation of suspected precipitating drug, if applicable

Severe disease

References:[3][6]

Complications

References:[3][7]

We list the most important complications. The selection is not exhaustive.

Follow-up

  • All patients
    • Blood pressure monitoring and urinalysis
      • Following diagnosis → weekly or biweekly
      • Once symptoms subside → monthly for the first 6 months, then every other month for an additional 6 months
      • At subsequent well-child visits
  • In patients with abnormal blood pressure or urinalysis: measure serum creatinine with referral to a pediatric nephrologist if abnormal

Prognosis

  • HSP usually resolves with full recovery. However, relapse is likely in patients with previous renal involvement.
  • Renal involvement is prognostically significant in HSP. The prognosis is worse in patients with nephrotic range proteinuria. In rare cases, ESRD may occur (approx. 1% of cases) → see “Follow-up” above
last updated 09/07/2018
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