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Kawasaki disease preceded by acute appendicitis treated with intravenous immunoglobulin without surgery

Year: 2024

Type: article

Abstract: Kawasaki disease-related acute appendicitis may be treated with IVIG without surgery Dear Editor, A 9-year-old girl was admitted to our hospital with fever, abdominal pain, and vomiting. Abdominal computed tomography revealed appendiceal swelling. The patient was transferred to our hospital on the second day of the illness with a diagnosis of acute appendicitis. She had no relevant medical or family history, and neither she nor her family had been infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Physical examination revealed bilateral conjunctival hyperaemia, throat redness, abdominal distention, severe right lower abdominal pain, and rebound pain. Laboratory tests revealed the following: white blood cell count, 20 400/µL (neutrophils, 91.4%; lymphocytes, 4.3%); haemoglobin, 12.4 g/dl; platelet count, 367 000/µL; aspartate transaminase, 335 IU/l; alanine aminotransferase, 111 IU/l; lactate dehydrogenase, 409 IU/l; total bilirubin, 0.7 mg/dl; albumin, 3.0 g/dl; sodium, 132 mEq/l; and CRP, 12.18 mg/dl. Rapid faecal antigen test results were negative for rotavirus, norovirus, and adenovirus. SARS-CoV-2 PCR results were also negative. Blood, pharyngeal, and faecal cultures were negative for Yersinia pseudotuberculosis and Yersinia enterocolitica. The patient was initially diagnosed as having acute appendicitis. We regarded Adenovirus and group A Streptococcus infections as differential diseases based on the physical examination findings. Considering the risk of worsening the infection with general anaesthesia, we first administered antibiotics (tazobactam and piperacillin). However, the symptoms persisted, and a skin rash appeared on her trunk on the eighth day of the illness. It was small erythemas, and no other erythema was observed on her trunk. Based on her physical findings, the patient was diagnosed with incomplete Kawasaki disease (KD). IVIG (2 g/kg/day) and aspirin (30 mg/kg/day) were administered. Following the initiation of IVIG therapy, swelling of the entire fingers was observed, fulfilling the diagnostic criteria for KD. By the ninth day, the fever had subsided, and the abdominal pain gradually alleviated without surgical intervention. Transaminase levels progressively decreased and normalized. Antibiotics were discontinued on the 15th day, and the aspirin dose was reduced to 5 mg/kg/day on the 16th day. Ultrasonography revealed no coronary artery lesions, and the patient was discharged on the 17th day. Acute appendicitis associated with KD is relatively rare [1–12]. To date, 20 cases of KD-related acute appendicitis have been reported (Table 1). Thirteen patients were diagnosed with KD following an appendectomy. Pathological findings were detailed in five cases, with inflammation confined to the serosa or involving both the serosa and muscular layer in three cases. Since acute appendicitis is typically caused by bacterial infection of the mucous membrane and the spread of inflammation, these findings differ from those observed in acute appendicitis. In vasculitis-induced appendicitis, inflammation may occur in the serous membranes. The case reported by Fujita et al. [11] was the first in which acute appendicitis improved with IVIG therapy without the need for appendectomy. In contrast to previous reports, our patient presented with elevated transaminase levels at the time of acute appendicitis diagnosis. The normal values for transaminases are AST 18–32 IU/l and ALT 9–27 IU/l, and in this case, a clear elevation was observed. Elevated transaminase levels are seen in ∼30% of the patients during the early stages of KD but are not associated with acute appendicitis [2]. In such cases, IVIG treatment may be considered for KD without the need for appendectomy. Previous reports of Kawasaki disease preceded by appendicitis CAL: coronary artery lesion; KD: Kawasaki disease; ND: not disclosed. No funding was received to assist with the preparation of this manuscript. Disclosure statement: The authors have declared no conflicts of interest. Patient consent: Signed informed consent was obtained from the patient’s parents regarding the use of patient information for the purposes of writing a case report publication. We would like to thank Editage Co. Ltd (www.editage.com) for English language editing.

Source: Rheumatology Advances in Practice

DOI: 10.1093/rap/rkaf003