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Kawasaki disease or polyarteritis nodosa: coronary involvement, a diagnostic conundrum

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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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Author notes

  1. Shivaprasad Pannasamudra Mohankumar and Samannay Das are joint first authors.

Authors and Affiliations

  1. Department of Paediatrics, AIIMS, New Delhi, India

    Shivaprasad Pannasamudra Mohankumar, Samannay Das & P. Likitha

  2. Department of Radiodiagnosis and Interventional Radiology, AIIMS, New Delhi, India

    Priyanka Naranje & Manisha Jana

  3. Department of Cardiology, AIIMS, New Delhi, India

    Saurabh Kumar Gupta

  4. Division of Pediatric Rheumatology and Immunology, Department of Paediatrics, AIIMS, New Delhi, India

    Narendra Kumar Bagri

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  1. Shivaprasad Pannasamudra Mohankumar
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Contributions

SPM, SD: literature review and wrote initial draft. LP, MJ, PN, SG: reviewed the draft. NB: concept, design, and review of the draft. All authors approved the final version of the draft. All authors take full responsibility for the integrity, accuracy, and all aspects of the work.

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Correspondence to Narendra Kumar Bagri.

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Mohankumar, S.P., Das, S., Likitha, P. et al. Kawasaki disease or polyarteritis nodosa: coronary involvement, a diagnostic conundrum. Rheumatol Int 43, 2327–2331 (2023). https://doi.org/10.1007/s00296-023-05388-1

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  • DOI: https://doi.org/10.1007/s00296-023-05388-1

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