Evidence Based Strategies: Small Vessel Vasculitis, Potential for Big Complications

Column Author: Gillian Long, DO | Pediatric Resident, PGY3

Column Editor: Angela Etzenhouser, MD | PHM Director of Safety, PHM Director of Safety; Associate Program Director, Pediatric Residency Program, Assistant Professor of Pediatrics, University of Missouri-Kansas City School of Medicine; Clinical Assistant Professor of Pediatrics, University of Kansas School of Medicine

IgA vasculitis (IgAV), formerly known as Henoch-Schonlein purpura, is the most common small vessel vasculitis in children. In fact, it is the single most common vasculitis that pediatricians feel comfortable diagnosing and managing. While no diagnostic criteria are used in the diagnosis of IgAV, most patients will have the classic purpuric rash, plus at least one other typical symptom such as arthralgia, abdominal pain, or renal involvement.¹ In the acute phase, the best treatment is supportive care. However, providers should be aware of both the possible acute and long-term complications.

RASH:

The typical IgAV rash is a nonthrombocytopenic, palpable, purpuric rash, starting on the lower extremities and buttocks. Atypical presentations of the rash include upper extremity involvement, progression to vesiculobullous lesions, and ulceration.² Presence of these atypical features is associated with more severe organ involvement and a more aggressive disease course.³ A skin biopsy is not indicated for the typical rash; however, it can be considered in atypical presentations to exclude alternative diagnoses. Biopsy showing leukocytoclastic vasculitis with predominant IgA deposits suggests IgAV, although absence of these findings does not rule out the diagnosis.

JOINT PAIN:

Approximately 75% of patients with IgAV will have associated arthralgias and swollen joints affecting the knees, ankles, feet and hands. Any arthritis is known to be transient and nondestructive. As usual in the treatment of inflammatory joint pain, nonsteroidal anti-inflammatory drugs (NSAIDs) are the typical first-line management. Though some providers may feel uneasy using NSAIDs due to the possibility of renal involvement, it is not contraindicated to give NSAIDs in patients with normal kidney function, even if there is evidence of microscopic hematuria.⁴ It is contraindicated to use NSAIDs in patients with glomerulonephritis or acute gastrointestinal (GI) bleed, although no studies have shown that using cyclooxygenase inhibitors increases the risk of GI hemorrhage in children with vasculitis involving the bowel.

ABDOMINAL PAIN:

Abdominal symptoms in IgAV can present in many different ways; however, the most classic presentation is that of diffuse abdominal pain. The most common surgical complication of IgAV is intussusception, which can lead to bowel necrosis and even intestinal perforation. In cases of severe abdominal pain, obtaining an abdominal ultrasound is recommended to rule out intussusception.⁴ We know that the onset of GI symptoms prior to the start of the rash is an independent risk factor for development of intussusception and that steroid therapy initiated within 72 hours of those GI symptoms lessens the risk of intussusception.⁵ Most guidelines suggest using steroids in the presence of severe abdominal pain and GI bleeding or for other less common complications including orchitis, cerebral vasculitis, pulmonary hemorrhage or other severe organ damage.

NEPHRITIS:

In the long term, any morbidity related to IgAV is due to development of chronic kidney disease from IgAV nephritis (IgAVN). IgAVN is characterized by proteinuria, hematuria, hypertension and impaired kidney function. There is no good way to predict which patients will be affected by kidney disease, and it is well known that steroid use does not prevent the development of IgAVN. Thus, we must stay vigilant in our surveillance of these patients for development of kidney disease. All patients with suspected IgAV should have a screening urinalysis at the time of onset, plus measurements of eGFR, albumin and blood pressure. Even patients with normal urinalysis at onset should have at least monthly urinalysis done for the next six months. In patients with kidney involvement, screening is prolonged but can be done by a primary care provider, with a recommended schedule of monthly for six months, every three months for six months, and every six months for up to five years.⁶ Any patients who demonstrate nephrotic range proteinuria or decreased eGFR, along with patients who demonstrate persistent mild-moderate proteinuria (more than two to four weeks), should be directed to a pediatric nephrologist for a kidney biopsy. Additionally, management of biopsy-proven IgAVN should be done in conjunction with a pediatric nephrologist.⁷

Ultimately, all pediatricians should feel comfortable managing IgAV. The most important complications to remember are intussusception and IgAVN, and appropriate screening should be done to monitor for the development of these conditions. Finally, we can reassure our patients that if recognized and treated properly, the prognosis is overall very good for this diagnosis.

References:

1. Smith G. Management of Henoch-Schonlein purpura. Paediatr Child Health. 2022;32(7):261-265.
2. Kaneta KA, Adler R. Purpuric vesiculobullous rash in IgA vasculitis (IgAV). J Pediatr. 2022;251:217-218.
3. Carmona-Cruz SA, Durán-McKinster LC, García-Romero MT. Atypical purpura and other features associated with unfavorable outcomes of IgA vasculitis (Henoch-Schonlein purpura) in children: a retrospective study. Pediatr Dermatol. 2022;39:369-371.
4. Ozen S, Marks SD, Brogan P, et al. European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative. Rheumatology (Oxford). 2019;58:1607-1616.
5. Mu J. Clinical characteristics and risk factors of IgA vasculitis with intussusception and intestinal perforation. J Paediatr Child Health. 2024;60(1):5-11.
6. Vivarelli M, Samuel S, Coppo R, et al. IPNA clinical practice recommendations for the diagnosis and management of children with IgA nephropathy and IgA vasculitis and nephritis. Pediatr Nephrol. 2025;40(2):533-569.
7. Oni L, Platt C, Marlais M, et al. National recommendations for the management of children and young people with IgA vasculitis: a best available evidence, group agreement-based approach. Arch Dis Child. 2025;110:67-76.