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Updated: Jul 24, 2025

Interventional Diagnostic Procedure: A Practical Guide for the Assessment of Coronary Vascular Function
Published on: March 15, 2022
Kawasaki disease or polyarteritis nodosa: coronary involvement, a diagnostic conundrum
Shivaprasad Pannasamudra Mohankumar1, Samannay Das1, P Likitha1
1Department of Paediatrics, AIIMS, New Delhi, India.
Insights
Polyarteritis nodosa (PAN), a medium-vessel vasculitis, can rarely affect coronary arteries, mimicking Kawasaki disease. Differentiating these conditions is crucial for appropriate treatment and improved outcomes in pediatric vasculitis.
Area of Science:
- Pediatric Rheumatology
- Vasculitis Research
- Cardiology
Background:
- Polyarteritis nodosa (PAN) is a medium-vessel vasculitis with multisystem involvement.
- Coronary artery aneurysms are characteristic of Kawasaki disease but rarely seen in PAN.
- Distinguishing between PAN and Kawasaki disease is vital due to differing treatment protocols and prognoses.
Purpose of the Study:
- To present two pediatric cases of PAN with coronary artery involvement that mimicked Kawasaki disease.
- To highlight the diagnostic challenges and key differentiating features between PAN and Kawasaki disease.
- To emphasize the importance of early and accurate diagnosis for optimal patient management.
Main Methods:
- Case report analysis of two pediatric patients.
- Review of clinical presentations, laboratory findings, and imaging studies (echocardiography, digital subtraction angiography).
- Comparison of diagnostic criteria and treatment approaches for PAN and Kawasaki disease.
Main Results:
- Two children presented with features suggestive of Kawasaki disease, including coronary aneurysms.
- Diagnostic workup revealed PAN involving coronary and other visceral arteries (celiac, renal).
- Digital subtraction angiography confirmed vasculitic changes consistent with PAN in both cases.
Conclusions:
- Coronary artery aneurysms, though rare, can be a presenting feature of childhood PAN.
- Accurate differentiation from Kawasaki disease is essential due to distinct therapeutic strategies and long-term outcomes.
- Clinical vigilance and appropriate imaging are crucial for diagnosing PAN presenting as Kawasaki disease.
Abstract:
Polyarteritis nodosa (PAN) is a medium-vessel vasculitis presenting with cutaneous and multisystem involvement with considerable morbidity. The necrotizing vasculitis in PAN typically involves renal, celiac, and mesenteric vascular beds. Coronary artery involvement is a characteristic feature of Kawasaki disease, another medium-vessel vasculitis; however, it has been rarely reported with PAN. Here, we present 2 cases with PAN involving coronaries mimicking Kawasaki disease. A 3.5-year-old boy with classical features of Kawasaki disease with giant coronary aneurysm refractory to IVIg, methylprednisolone, infliximab presented with persistent rise in inflammatory markers and gastrointestinal bleeding. Digital subtraction angiography (DSA) revealed celiac artery branches stenosis and beading suggestive of PAN. Another 2-year-old girl presented with persistent fever, abdominal pain, and distension. She had hypertension, hepatomegaly, and splenomegaly on examination. Echocardiography revealed multiple coronary aneurysms and DSA revealed numerous renal artery aneurysms. Coronary aneurysm although is a rare presentation of childhood PAN, and can mimic Kawasaki disease. Although both are medium-vessel vasculitis differentiation between these two entities is pivotal, as there are differences in treatment modalities, duration of immunomodulatory therapy, and the outcome. This manuscript describes the salient differences which can help differentiate PAN masquerading as Kawasaki disease at initial presentation.
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