key: cord-0810448-fl0ouw9y
authors: Singh, Surjit; Gupta, Aman; Jindal, Ankur Kumar; Gupta, Anju; Suri, Deepti; Rawat, Amit; Vaidya, Pankaj C.; Singh, Meenu
title: Pulmonary presentation of Kawasaki disease—A diagnostic challenge
date: 2017-09-26
journal: Pediatr Pulmonol
DOI: 10.1002/ppul.23885
sha: 2641c873542ce7a1b220b4af567cb12a18e15901
doc_id: 810448
cord_uid: fl0ouw9y

OBJECTIVES: Kawasaki disease (KD) is a multisystemic vasculitis with predominant mucocutaneous manifestations. Pulmonary involvement in KD is distinctly uncommon and is not commonly recognized. We describe our experience of managing children with KD wherein the initial presentation was predominantly pulmonary. METHODS: Six hundred and two children have been diagnosed with KD during the period January 1993 to May 2017 in the Allergy Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh. Data were collected from inpatient records in Allergy Immunology Unit and follow‐up files in the Pediatric Rheumatology Clinic. RESULTS: Of 602 children, 11 (1.83%) had a predominant pulmonary presentation of KD. Mean age at diagnosis of KD was 2.5 years. Fever, cough and respiratory distress were the presenting complaints in all patients. First sign of KD was noted at a mean duration of 14.5 days from the onset of symptoms. Periungual desquamation was the most common clinical sign (72.7%). Persistent fever in spite of antimicrobials, thrombocytosis, and elevated erythrocyte sedimentation rate and C‐reactive protein levels pointed toward a diagnosis of KD in our patients. Parenchymal consolidation was evident on chest X‐ray in all patients, pleural effusion in six, empyema in three, and pneumothorax in two patients. Coronary artery abnormalities were evident in three patients. Intravenous immunoglobulin was given after a mean period of 22.4 days of onset of fever. CONCLUSIONS: The diagnosis of KD is often delayed in children who have a predominantly pulmonary presentation. This can have adverse clinical consequences.

there is no pathognomonic laboratory test for confirmation. The clinical course of KD can be divided into three phases 2 : (a) Acute febrile phase-lasts for initial 10-14 days; characterized by fever, mucocutaneous changes and other less commonly recognized findings such as hydrops of gall bladder, sterile pyuria and myocarditis; (b) Sub-acute phase-lasts from weeks 2-4 after symptom onset; characterized by periungual desquamation and coronary abnormalities along with resolution of principal clinical features; (c) Convalescent phasecharacterized by complete resolution of clinical signs and laboratory parameters. Exact etiology of KD remains unknown, though various hypotheses on the role of micro-organisms and superantigens have been postulated. 2 The principal features that constitute the diagnostic criteria are predominantly mucocutaneous. However, as KD is a vasculitis, it can involve several other organ systems including musculoskeletal, gastrointestinal, central nervous system, genitourinary, and pulmonary. 2, 3 A proportion of children with KD may occasionally present with unusual clinical features (atypical KD), or may have fever in the presence of less than four principal clinical features (incomplete KD). 4 The initial presentation may, at times, point toward a different disease but the child may develop KD during the course of illness. Pulmonary involvement in patients with KD is distinctly uncommon and is often not commonly recognized. Careful monitoring is essential because some features of KD may evolve over time and early recognition of this group of patients may prevent delays in diagnosis and allow institution of early appropriate therapy.

We describe our experience of managing children with KD wherein the initial presentation was predominantly pulmonary thereby resulting in considerable diagnostic difficulties and consequential delay in institution of appropriate therapy. Clinical findings, laboratory parameters and radiological data were recorded. Laboratory investigations included total leukocyte count (TLC), platelet count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and serum aminotransferases. Radiological examination included X-ray chest, ultrasonography (USG), computerized tomography (CT) of chest, wherever considered necessary and echocardiography to assess coronary arteries. All children were treated with intravenous antimicrobials for pneumonia and/or pleural effusion/ empyema. Following a diagnosis of KD, intravenous immunoglobulin (IVIG) was administered at 2 g/kg along with aspirin at 30-50 mg/kg/day which was continued until ≥48-72 h after fever cessation. The aspirin dose was thereafter reduced to 3-5 mg/kg/day and continued for the next 6-8 weeks. Aspirin was discontinued if the follow-up echocardiography did not show any coronary artery abnormality.

Of 602 children, 11 (1.83%) had a predominant pulmonary presentation of KD. Mean age at diagnosis of KD was 2.5 years (range 6 months-4 years). Clinical findings are summarized in Table 1 consolidation was evident on chest X-ray in all patients. This radiological picture was no different from that seen in other common forms of childhood pneumonia (eg, staphylococcal or pneumococcal pneumonia). All except two (81.8%) had complications evident on USG or X-ray chest in the form of pleural effusion in 6 (54.5%), empyema in 3 (27.3%), and pneumothorax in 2 (18.2%) ( Table 2 ). Four patients underwent computerized tomography of chest and the findings were largely compatible with those seen on X-ray chest.

Coronary artery abnormalities were evident on echocardiography in three patients. Dilatation of right coronary artery, ectasia of left main coronary artery, and bright coronaries were noted in one patient each.

Repeat echocardiography after 2 and 6 weeks was normal in two Examination findings suggestive of KD Pulmonary manifestations that have been described in patients with KD include bronchopneumonia, 5 hydropneumothorax, 6 and pleural effusion. 7 Cases of pulmonary presentation of KD described in the literature have been summarized in Table 4 . Lee et al reported chest X-ray abnormalities in 51.2% of patients with KD. 8 Abnormal chest X-ray findings were seen in all of our patients. Pleural effusion was seen in 54.5% patients followed by empyema in 27.3% and pneumothorax in 18.2%. Lung involvement in KD may occur due to inflammation of vessels resulting in increased vascular permeability. However, the entire spectrum of radiological pulmonary manifestations seen in children with KD cannot be solely explained by vessel inflammation. Intravenous immunoglobulin is the mainstay of treatment for KD and should preferably be given within 10 days of the onset of illness. 1, 15 "Incomplete" or "atypical" presentation of KD results not only in delayed diagnosis but also delays in initiation of appropriate therapy.

Pulmonary symptoms are usually initially treated with antimicrobials and highlight the importance of considering a diagnosis of KD in children where fever persists for 4 or more days despite antimicrobials.

The presence of relatively uncommon clinical features should not be a deterrent in considering underlying KD in such patients.

The strengths of this study are that this was a single centre study and all patients were diagnosed by the same set of clinicians (SS, DS) using standard guidelines. Management protocols were also uniform.

The limitation of this study is the small sample size-this is inevitable considering the fact that data were collated from only one center and pulmonary manifestations of KD are a distinctly unusual presentation of this disorder.

Various organ systems can be involved in KD and uncommon pulmonary manifestations can sometimes be seen in children.

Unresolving pneumonia in a child who continues to be febrile despite adequate antimicrobials can be a clue toward the diagnosis of KD. In view of late recognition of symptoms, IVIG in such patients is often delayed resulting in significant morbidities. Physicians managing children with "difficult-to-treat" pneumonia should be aware of the unusual pulmonary presentation of KD as early recognition can prevent delays in diagnosis and shorten the hospital stay.

ORCID Aman Gupta http://orcid.org/0000-0001-5897-7912

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