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Background <p>Kawasaki disease (KD) was first reported by Kawasaki in Japan in 1967. Although more than 50 years have passed since then, the aetiology and long-term prognosis of this condition remain not fully elucidated. Clinically, it frequently affects the cardiovascular system, leading to complications such as coronary aneurysms, macrophage activation syndrome, or KD shock syndrome. Selective IgA deficiency (SIgAD) is a common primary immunodeficiency disorder associated with other clinical conditions, such as autoimmune diseases, pulmonary diseases, gastrointestinal diseases, allergic diseases, neurological disorders, and malignancies. However, there is a conflict between the treatment regimens for the two conditions, and selecting the appropriate treatment strategy for patients with both conditions presents a challenge.</p> Methods <p>We report the case of a 5-year-old child who was admitted to hospital with fever and, during his hospitalisation, was diagnosed with KD complicated by SIgAD. During the course of clinical treatment, the patient’s anti-IgA antibody levels were not measured; following a comprehensive assessment, the patient was treated with intravenous immunoglobulin (IVIG)(an ultra-low, IgA-depleted formulation) and aspirin.J06BAJ215</p> Results <p>On the second day of hospitalisation, the child underwent an echocardiogram: the width of the left coronary artery orifice was 0.20&#xa0;cm, with a Z-score of − 1.789; the width of the right coronary artery orifice was 0.20&#xa0;cm, with a Z-score of − 0.472. Following administration of IVIG, the fever gradually subsided, and the rash and conjunctival hyperaemia improved compared with previously. On the fourth day of hospitalisation, the child’s temperature returned to normal. On the sixth day of admission, the child’s cervical lymph nodes began to shrink, and the indurations, swelling and erythema in the limbs had resolved. On the seventh day of admission, the child’s conjunctival injection and strawberry tongue had virtually disappeared, the rash had largely resolved, and no enlarged cervical lymph nodes were palpable. A follow-up echocardiogram showed that the width of the left coronary artery ostium was 0.18&#xa0;cm with a Z-score of − 2.406, whilst the width of the right coronary artery ostium was 0.20&#xa0;cm with a Z-score of − 0.472. The child was discharged. A follow-up echocardiogram performed 16 days after discharge showed: the left coronary artery orifice measured 0.28&#xa0;cm in width with a Z-score of 0.68; the right coronary artery orifice measured 0.27&#xa0;cm in width with a Z-score of 1.636. A follow-up echocardiogram performed 44 days after discharge showed: the main truncus of the left coronary artery was 0.16&#xa0;cm wide with a Z-score of -3.023, and the main truncus of the right coronary artery was 0.21&#xa0;cm wide with a Z-score of -0.171. The child reported no discomfort.</p> Conclusions <p>Although, in this case, no allergic reactions were observed in the patient with SIgAD following IVIG treatment, this does not imply that all patients with SIgAD are suitable candidates for IVIG therapy. Given that the patient was not tested for anti-IgA antibodies, the possibility cannot be ruled out that other patients with SIgAD may have sufficient levels of anti-IgA antibodies to trigger an allergic reaction. This case report merely provides a literature-based clinical management protocol for KD complicated by SIgAD. We do not advocate a blanket rejection of existing safety guidelines for the use of immunosuppressive agents in immunodeficient patients; rather, we support the adoption of a localised clinical management strategy: implementing strict, proactive and continuous monitoring of relevant cases, with follow-up assessments conducted within a controlled inpatient setting. This will help to control the progression of KD in a timely manner and improve patient prognosis.</p>

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Treatment of children with Kawasaki disease complicated by selective IgA deficiency using immunoglobulin preparations: a case report

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  • 发表日期：

    2026年8月3日

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  • Kun Liang Zhang,
  • Hui Chen

摘要

Background

Kawasaki disease (KD) was first reported by Kawasaki in Japan in 1967. Although more than 50 years have passed since then, the aetiology and long-term prognosis of this condition remain not fully elucidated. Clinically, it frequently affects the cardiovascular system, leading to complications such as coronary aneurysms, macrophage activation syndrome, or KD shock syndrome. Selective IgA deficiency (SIgAD) is a common primary immunodeficiency disorder associated with other clinical conditions, such as autoimmune diseases, pulmonary diseases, gastrointestinal diseases, allergic diseases, neurological disorders, and malignancies. However, there is a conflict between the treatment regimens for the two conditions, and selecting the appropriate treatment strategy for patients with both conditions presents a challenge.

Methods

We report the case of a 5-year-old child who was admitted to hospital with fever and, during his hospitalisation, was diagnosed with KD complicated by SIgAD. During the course of clinical treatment, the patient’s anti-IgA antibody levels were not measured; following a comprehensive assessment, the patient was treated with intravenous immunoglobulin (IVIG)(an ultra-low, IgA-depleted formulation) and aspirin.J06BAJ215

Results

On the second day of hospitalisation, the child underwent an echocardiogram: the width of the left coronary artery orifice was 0.20 cm, with a Z-score of − 1.789; the width of the right coronary artery orifice was 0.20 cm, with a Z-score of − 0.472. Following administration of IVIG, the fever gradually subsided, and the rash and conjunctival hyperaemia improved compared with previously. On the fourth day of hospitalisation, the child’s temperature returned to normal. On the sixth day of admission, the child’s cervical lymph nodes began to shrink, and the indurations, swelling and erythema in the limbs had resolved. On the seventh day of admission, the child’s conjunctival injection and strawberry tongue had virtually disappeared, the rash had largely resolved, and no enlarged cervical lymph nodes were palpable. A follow-up echocardiogram showed that the width of the left coronary artery ostium was 0.18 cm with a Z-score of − 2.406, whilst the width of the right coronary artery ostium was 0.20 cm with a Z-score of − 0.472. The child was discharged. A follow-up echocardiogram performed 16 days after discharge showed: the left coronary artery orifice measured 0.28 cm in width with a Z-score of 0.68; the right coronary artery orifice measured 0.27 cm in width with a Z-score of 1.636. A follow-up echocardiogram performed 44 days after discharge showed: the main truncus of the left coronary artery was 0.16 cm wide with a Z-score of -3.023, and the main truncus of the right coronary artery was 0.21 cm wide with a Z-score of -0.171. The child reported no discomfort.

Conclusions

Although, in this case, no allergic reactions were observed in the patient with SIgAD following IVIG treatment, this does not imply that all patients with SIgAD are suitable candidates for IVIG therapy. Given that the patient was not tested for anti-IgA antibodies, the possibility cannot be ruled out that other patients with SIgAD may have sufficient levels of anti-IgA antibodies to trigger an allergic reaction. This case report merely provides a literature-based clinical management protocol for KD complicated by SIgAD. We do not advocate a blanket rejection of existing safety guidelines for the use of immunosuppressive agents in immunodeficient patients; rather, we support the adoption of a localised clinical management strategy: implementing strict, proactive and continuous monitoring of relevant cases, with follow-up assessments conducted within a controlled inpatient setting. This will help to control the progression of KD in a timely manner and improve patient prognosis.