From the Research
Introduction to Henoch-Schönlein Purpura (HSP)
Henoch-Schönlein Purpura (HSP) is a systemic IgA vasculitis that affects small vessels, commonly presenting in children and rarely in adults 1. The condition is characterized by its impact on various body systems, including the skin, joints, gastrointestinal tract, and kidneys.
Major Manifestations of HSP
The major manifestations of HSP include:
- Cutaneous purpura, which is non-thrombocytopenic and typically found on the lower extremities and buttocks 2, 1
- Arthritis or arthralgia, indicating joint involvement 1, 3
- Gastrointestinal symptoms, such as abdominal pain and potentially gastrointestinal hemorrhage 1, 3
- Renal involvement, which may manifest as nephritis, hematuria, and/or proteinuria 1, 3
Less Common Manifestations
Less common manifestations of HSP can include:
- Urological involvement, affecting the kidney, ureter, bladder, prostate, scrotum, testicle, and penis 4
- Pulmonary, cardiac, genital, and neurological symptoms, although these are rare 1
Diagnosis and Treatment
Diagnosis of HSP is based on the presence of palpable purpura along with at least one of the following criteria: diffuse abdominal pain, arthritis or arthralgia, renal involvement, or a biopsy showing predominant IgA deposition 3. Treatment of HSP is generally supportive and may include:
- Nonsteroidal anti-inflammatory drugs for pain relief
- Oral corticosteroids for severe gastrointestinal pain and gastrointestinal hemorrhage
- Topical treatments for skin manifestations
- In severe cases, glucocorticoids, methylprednisolone, plasmapheresis, and peritoneal and hemodialysis may be considered 4
Treatment Considerations
Treatment considerations for HSP include:
- The use of systemic corticosteroids, which may be beneficial in reducing the extent of lesions and minimizing sequelae of disease, but its early use is controversial 2
- The potential need for angiotensin-converting enzyme inhibitors or angiotensin receptor blockers in addition to corticosteroids for patients with severe nephritis or renal involvement 3
- The importance of monitoring for long-term complications, such as persistent hypertension and end-stage kidney disease 3
Conclusion
Henoch-Schönlein Purpura is a complex condition with various manifestations. Understanding its clinical features, diagnosis, and treatment options is crucial for healthcare providers to manage the condition effectively and prevent long-term complications. Early recognition and appropriate management can significantly impact the prognosis of patients with HSP 5.