Treatment of a patient with Kawasaki disease associated with selective IgA deficiency by continuous infusion of cyclosporine A without intravenous immunoglobulin

Anzai, Tatsuya; Minami, Takaomi; Sato, Tomoyuki; Furui, Sadahiro; Yamagata, Takanori

doi:10.24953/turkjped.2016.06.015

Cited by 5 publications

(4 citation statements)

References 9 publications

(7 reference statements)

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“…In patients with KD and concurrent selective IgA deficiency, IgM plasma cells might replace the function of IgA plasma cells that otherwise infiltrate coronary arteries and other tissues in KD. IgA plasma cells play an essential role in the inflammatory process associated with KD [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

“…His symptoms resolved with the initiation of methylprednisolone [ 11 ]. Anzai et al successfully treated a 5-year-old male with complete KD with intravenous cyclosporine A instead of IVIG who was diagnosed with selective IgA deficiency at the time of work-up for KD [ 12 ]. Şanlıdağ et al presented a 4-year-old boy diagnosed with transient hypogammaglobulinemia of infancy at 3.5 years of age who developed incomplete KD [ 13 ].…”

Section: Kawasaki Disease In Inborn Errors Of Immunitymentioning

confidence: 99%

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Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications

et al. 2023

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The exact etiopathogenesis of Kawasaki disease (KD), the most common childhood vasculitis, remains unknown; however, an aberrant immune response, possibly triggered by an infectious or environmental agent in genetically predisposed children, is believed to be the underlying pathogenetic mechanism. Patients with inborn errors of immunity (IEI) are predisposed to infections that trigger immune dysregulation due to an imbalance in various arms of the immune system. KD may develop as a complication in both primary and secondary immunodeficiencies. KD may occur either at disease presentation or have a later onset in IEIs. These include X-linked agammaglobulinemia (XLA), selective IgA deficiency, transient hypogammaglobulinemia of infancy; Wiskott–Aldrich syndrome (WAS), hyper IgE syndrome (HIES); chronic granulomatous disease (CGD), innate and intrinsic immunity defects, and autoinflammatory diseases, including PFAPA. Hitherto, the association between KD and IEI is confined to specific case reports and case series and, thus, requires extensive research for a comprehensive understanding of the underlying pathophysiological mechanisms. IEIs may serve as excellent disease models that would open new insights into the disease pathogenesis of children affected with KD. The current review highlights this critical association between KD and IEI supported by published literature.

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Section: Discussionmentioning

confidence: 99%

Section: Kawasaki Disease In Inborn Errors Of Immunitymentioning

confidence: 99%

Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications

et al. 2023

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“…12 We previously reported a patient with KD with selective immunoglobulin A (IgA) deficiency treated with intravenous CsA without IVIG. 13 However, little is known about the effectiveness of intravenous CsA administration. This study therefore aimed to evaluate the efficacy of continuous intravenous cyclosporine A (CICsA) in patients with IVIG-resistant KD.…”

mentioning

confidence: 99%

“…described the clinical course and outcome of KD patients treated with intravenous CsA followed by oral administration 12 . We previously reported a patient with KD with selective immunoglobulin A (IgA) deficiency treated with intravenous CsA without IVIG 13 . However, little is known about the effectiveness of intravenous CsA administration.…”

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confidence: 99%

Continuous cyclosporine a infusion in patients with severe Kawasaki disease

Seki

Minami

Suzuki

et al. 2022

Pediatrics International

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Background The efficacy and safety of continuous intravenous infusion of cyclosporine A (CICsA) in patients with intravenous immunoglobulin‐resistant Kawasaki disease are unclear. Methods Between 2010 and 2020, 83 patients with Kawasaki disease that was not responsive to intravenous immunoglobulin (total dose ≥ 4 g/kg) were enrolled. All patients were started on CICsA (3 mg/kg/day) and switched to oral cyclosporine A (CsA) (4–6 mg/kg/day). Treatment efficacy, occurrence of coronary artery lesions (CALs), and laboratory parameters were evaluated. Patients were divided into two groups according to CICsA response: the responder group (afebrile ≤24 h after CICsA without additional treatment) and the weak responder group (afebrile >24 h after CICsA requiring additional treatment). Results Fifty‐five patients became afebrile within 24 and 74 h became afebrile in less than 72 h. Adverse events included hypertension in four and hyperkalemia in two patients. Thirty‐nine patients were defined as responders and 44 patients as weak responders. There were no significant differences in CAL between the two groups. In weak responders, white blood cells, neutrophils, and C‐reactive protein levels were higher, and albumin, immunoglobulin G, and CsA concentration were lower than in responders, indicating that weak responders had more severe inflammatory findings. However, there were no significant differences in CAL. Logistic regression analysis revealed that the response to treatment for CICsA was associated with immunoglobulin G levels at baseline and CsA concentrations the day after CICsA. Conclusion Although CICsA required additional treatments in about half of the cases, a favorable clinical course was observed by using this strategy, especially for reducing CAL.

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Kawasaki disease and immunodeficiencies in children: case reports and literature review

et al. 2019

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Treatment of a patient with Kawasaki disease associated with selective IgA deficiency by continuous infusion of cyclosporine A without intravenous immunoglobulin

Cited by 5 publications

References 9 publications

Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications

Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications

Continuous cyclosporine a infusion in patients with severe Kawasaki disease

Kawasaki disease and immunodeficiencies in children: case reports and literature review

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