A case of Henoch-Schonlein Purpura with dilated coronary arteries

Abstract

BackgroundHenoch-Schonlein Purpura (HSP) is one of the most common vasculitides of childhood, with 10–20 cases per 100,000 children. It frequently occurs following an infectious trigger and involves IgA and C3 deposition in small vessel walls. HSP is characterized by palpable purpura plus IgA deposition on biopsy, arthritis/arthralgia, renal involvement (hematuria and/or proteinuria), and/or abdominal pain. It is not generally recognized as a cause of dilated coronary arteries.Case presentationWe describe the first reported case of HSP presenting with dilated coronary arteries. This patient is a nine-year-old previously healthy Caucasian male who presented with 1 week of petechiae on his lower legs, knee and ankle arthritis, and abdominal pain without fever, consistent with HSP. An echocardiogram revealed coronary dilation, including the left main (5.32 mm, Z score + 4.25) and left anterior descending (LAD) (3.51 mm, Z score + 2.64) coronary arteries. He received high dose aspirin, IVIG, and infliximab with normalization of the LAD. Skin biopsy revealed leukocytoclastic vasculitis with positive IgA staining. He was Rhinovirus/Enterovirus positive with Group A Streptococcus on throat culture.ConclusionCardiac findings, while rare, can exist in HSP. Coronary dilation appeared to respond to our hospital protocol’s Kawasaki Disease (KD) therapy, possibly indicating an overlap in HSP and KD pathophysiology. This case, along with prior reports of dilated coronaries in systemic juvenile idiopathic arthritis (SJIA), highlights the importance of considering other sources of systemic inflammation, in addition to KD, when coronary dilation is identified. The appropriate therapy, follow-up, and prognosis for our patient are not clear, as further studies are needed to determine the natural course of these findings.

Intestinal Permeability and IgA Provoke Immune Vasculitis Linked to Cardiovascular Inflammation

“…Furthermore, children with KD (Stagi et al, 2006) and those with IgAN (Coppo, 2018b) have an increased prevalence of celiac disease. A recent case report of a child with IgA vasculitis who developed dilated coronary arteries and responded to IVIG and infliximab therapy further supports an overlap in IgA vasculitis and KD pathogenesis (Bloom et al, 2018). Finally, similar to the important role that IL-1b plays in KD vasculitis (Burns et al, 2017), this master cytokine has been implicated in the pathogenesis and renal complications of both IgA vasculitis (Besbas et al, 1997;Boyer et al, 2011) and IgAN (Atkins, 1995;Chun et al, 2016).…”

Henoch-Schönlein Purpura /IgA Vasculitis Complicated by Coronary Artery Aneurysm: A Case Report and Literature Review

“…At present, the clinical treatment of HSP/IgAV combined with coronary artery dilatation is still derived from the empirical treatment of KD combined with coronary artery dilatation. Only a one case, a 9-year-old male child with HSP/IgAV with coronary artery dilatation who had left main coronary artery and anterior descending branch dilatation, can has been recorded in the PubMed database so far, reported by Bloom et al (22). After IVIG, infliximab, and aspirin treatment, this patient's coronary artery returned to normal after 3 months (22).…”

Myocarditis and intracardiac thrombus due to Henoch-Schönlein purpura: case report and literature review

“…The authors concluded that dilatation in the coronary arteries could be secondary to chronic vasculitis developed due to previous HSP. Blomm et al [21] reported a patient who had coronary artery enlargement with the presence of HSP clinic and who achieved complete recovery with treatment, such as Kawasaki disease. These results might support the hypothesis of the Kawasaki disease that occurred coincidentally during HSP.…”