Pleural Effusion as an Atypical Presentation of Kawasaki Disease

A Case Report and Review of the Literature

Elif Arslanoglu Aydin; Selcan Demir; Orkun Aydin; Yelda Bilginer; Seza Ozen


J Med Case Reports. 2019;13(344) 

In This Article

Discussion and Conclusion

The most important complication of KD is CAI, which leads to enlargement, aneurysm, ischemic heart disease, and sudden death.[1] The clinical course of KD is highly variable. There are no pathognomonic clinical or laboratory findings to help diagnose KD. The diagnosis of KD in this case was made using the criteria of the American Heart Association.[1] In the presence of at least 5 days of fever, if there are at least four of the five principal criteria (cervical adenopathy, bilateral non-purulent conjunctivitis, oropharyngeal mucosal changes, polymorphous rash, erythema of the palms or soles, and edema of the hands or feet) the patient is diagnosed as having KD.[1]

KD may present with uncommon symptoms such as pneumonia, pleural effusion, diarrhea, vomiting, sterile pyuria, gallbladder hydrops, acute cholestatic hepatitis, arthritis, and aseptic meningitis.[2–7] Pulmonary system involvement of KD is very rare; KD can present as pneumonia, pulmonary nodules, bronchopneumonia, hydropneumothorax, and pleural effusion.[6,8,9] Singh et al. showed that 1.3% of patients had pulmonary involvement and pleural effusion was seen in 54.5% of these patients.[6] Ugi et al. reported the case of an adult patient who presented with pulmonary involvement, specifically bilateral massive pleural effusions.[10] Occasionally, pleural effusion may be associated with bacterial agents such as Mycoplasma pneumoniae and Streptococcus.[11,12] Pulmonary symptoms are mostly initially treated with antibiotics. However, if fever and accompanying signs ensue, the diagnosis of KD should be considered. Patients with pulmonary involvement may be more likely to have CAI due to delays in diagnosing KD and administration of IVIG.[12–17]

We performed a review of the literature using PubMed and the search terms: Kawasaki disease AND pulmonary involvement; OR Kawasaki disease AND pulmonary presentation; OR Kawasaki disease AND pleural effusion. The searches were limited to the English language and pediatric patients. Case series and single case reports involving pediatric patients with KD with pulmonary involvement were included. Inconsistencies were resolved through discussion with the author SO, who also reviewed the literature. The authors EAA and OA searched the literature and manually screened titles and abstracts for relevance. Inconsistencies were resolved through discussion with the author SO.

Figure 2 lists the schematic analyses of the systematic literature review. At first, 25 related articles were found, but nine articles were excluded because of duplication, non-English language, and adult age, which left 16 articles.[6,8,11–24] The characteristics of these patients are summarized in Table 1, Table 2 and Table 3. Finally, 20 patients with pleural effusions due to KD were identified.[6,11–18,20,24] Of the 20 reviewed patients, TTE results were available in nine patients and seven had CAI.[6,12–17,24] Eleven patients presented with respiratory symptoms such as cough, dyspnea, and tachypnea.[6,12–15,20] Only four patients[14,17,18,24] had complete KD, 10 patients[6,13,15,16,20] had incomplete KD, and six patients'[11,12] presentations were not available. Although a definite infectious agent could be shown for two patients,[18,24] all of the patients received antibiotics except one.[14] Two patients[6,17] received a second dose of IVIG, and five patients received a second dose of IVIG and corticosteroid treatment for KD.[13–16,18]

Figure 2.

Study flowchart

In this case, our patient initially had an exudative, noninfectious pleural effusion and no response to antibiotics. CAI was also noticed and IVIG was administered on the 15th day of fever. After IVIG treatment, our patient's clinical and laboratory findings improved dramatically, and the fever and acute-phase reactants returned to normal. It remains unclear as to whether the KD was triggered by the infection of the pleural space or if the pulmonary finding was a feature of the inflammation of KD.

KD can affect various systems as well as the coronary arteries, and may present with an unusual clinical picture. The diagnosis of KD with atypical presentations may be difficult for pediatricians. Early diagnosis and treatment can prevent complications.