Controversies in diagnosis and management of Kawasaki disease

Pilania, Rakesh Kumar; Bhattarai, Dharmagat; Singh, Surjit

doi:10.5409/wjcp.v7.i1.27

Cited by 51 publications

(38 citation statements)

References 88 publications

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“…One of the biggest challenges remaining in the treatment of KD is the difficulty in diagnosis, especially in incomplete forms of KD, which may be associated with serious CAL; this is because diagnosis is based on a set of criteria that are entirely clinical, and laboratory tests rarely provide conclusive evidence [116]. Another challenge is determining whether KD vasculitis is self-limiting or persistent with or without exacerbations and remissions such as subacute/ chronic vasculitis of KD.…”

Section: Future Considerations and Conclusionmentioning

confidence: 99%

Autoimmune Aspects of Kawasaki Disease

Sakurai¹

2019

J Investig Allergol Clin Immunol

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Kawasaki disease (KD) is classified as a medium-sized vasculitis of systemic vasculitis syndrome characterized by hypercytokinemia. Although the etiology of KD remains unidentified, epidemiological features point to the role of infection and genetic predisposition. Recent studies revealed endothelial damage and resultant thrombin generation, as well as B-cell activation during the acute phase of KD. Several anti-endothelial cell autoantibodies (AECAs) have been identified in KD patients. Taken together with the recently developed concept of immunothrombosis, a potential pathogenic mechanism for KD emerges. First, some polyclonal antibodies generated against invading microorganisms would exhibit cross-reactivity toward endothelial cell components and become dominant during affinity maturation. AECA binding to endothelial cells would cause endothelial activation or damage, with proinflammatory cytokine release, fostering a hypercoagulable state by leukocyte activation by proinflammatory cytokines. This, in turn, would lead to coronary artery lesions. KD vasculitis might be initiated upon AECA binding to the vasa vasorum and progress to panvasculitis and a vulnerable vessel wall, resulting in an aneurysm. The aneurysm would cause flow recirculation and alteration of wall shear stress. Consequently, platelets activated by shear stress, along with ultra-large von Willebrand factor (VWF) released by endothelial cells, would cause platelet-driven arterial thrombosis. Autoimmunity-associated thrombosis initiated by AECA binding to endothelial cells might play a major role in the pathogenesis of certain subtypes of KD. The notion of KD consisting of subtypes, the major one of which is AECA-associated vasculitis, will help facilitate a better understanding of KD and further promote early and accurate diagnosis, which remains challenging.

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Section: Future Considerations and Conclusionmentioning

confidence: 99%

Autoimmune Aspects of Kawasaki Disease

Sakurai¹

2019

J Investig Allergol Clin Immunol

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“…The diagnosis of KD is challenging and requires experience. Clinical features of KD are nonspecific and overlap with various common childhood disorders especially infections [33]. Patients who are not fulfilling clinical criteria completely are labelled as incomplete KD.…”

Section: Incomplete Kdmentioning

confidence: 99%

Kawasaki Disease

Pilania¹,

Singh²

2019

Periodic and Non-Periodic Fevers

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Kawasaki disease (KD) is an acute and usually self-limiting medium vessel vasculitis of childhood that has a predilection to involve the coronary arteries. It is characterized by the sequential appearance of a constellation of clinical features [1]. However, none of these clinical findings is, in itself, pathognomonic of KD. Many of these clinical features can, in fact, be seen in other common febrile illnesses of children. It is for this reason that the diagnosis of KD is often considered to be a clinical challenge [2]. Approximately 15-25% of untreated patients may go on develop coronary artery abnormalities (CAAs) and this remains the major cause of morbidity, and occasional mortality, in KD [3].

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“…Corticosteroid treatment appears to be beneficial in patients with severe KD, showing resistance to intravenous immunoglobulins. Combination of corticosteroids and intravenous immunoglobulins is indicated in patients at high risk of unresponsiveness [ 3 , 16 , 17 ].…”

Section: Discussionmentioning

confidence: 99%

Acute cholestasis as uncommon onset of Kawasaki disease: a case report

Gallerani¹,

Pala

Fabbian

et al. 2020

BMC Gastroenterol

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Background Kawasaki disease (KD) or mucocutaneous lymph node syndrome is a vasculitis that mostly occurs in young children. Adult-onset KD (AKD) is rare and often misdiagnosed. Here we report a rare case of KD with cholestasis as principal symptom. Case presentation A 43-year-old caucasian man was admitted to our hospital for high fever, lack of appetite related to nausea and vomiting, headache and significant malaise. Physical examination highlighted fever, increasing jaundice, bilateral laterocervical lymph nodes, erythema of the palms, and strikingly red lips and conjunctiva. The clinical course was complicated by arterial hypotension, tachycardia, decreasing haemoglobin, increasing acute phase reactants tests, and multiorgan failure. Due to cardiovascular instability the patient was admitted to the local Intensive Care Unit. Chest X-ray, abdominal ultrasound, chest and abdominal CT and Colangio Magnetic Resonance were normal. Jaundice was investigated and infections, autoimmune diseases or drugs adverse reactions, were excluded. Also coronary artery computed tomography was carried out excluding coronary artery aneurysms. Broad-spectrum antibiotics were not effective. After exclusion other possible conditions, diagnosis of KD was set. He was treated with high doses of corticosteroids and acetylsalicylic acid and clinical conditions as well as laboratory exams improved. Conclusions This report dealing with an adult onset of atypical KD may be of benefit to physicians of various specialties, including primary care doctors, hospital internists, intensivists and gastroenterologists due to its peculiarities. It demonstrates that a case of prolonged fever unresponsive to antibiotics and related to cholestatic jaundice, oedema or erythema of the extremity associated with desquamation of feet and hands, and red eyes, may suggest atypical form of KD.

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Controversies in diagnosis and management of Kawasaki disease

Cited by 51 publications

References 88 publications

Autoimmune Aspects of Kawasaki Disease

Autoimmune Aspects of Kawasaki Disease

Kawasaki Disease

Acute cholestasis as uncommon onset of Kawasaki disease: a case report

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