Background Kawasaki disease (KD) is a medium vessel vasculitis which predominantly affects children less than 5 years of age. Though principal clinical features are mucocutaneous, KD in children may have multiple systemic manifestations, including pulmonary, which may create diagnostic difficulties for the treating physician.

Objectives We describe our experience of managing children with uncommon pulmonary presentation of KD.

Methods Five hundred and sixty five (565) children were diagnosed with KD during the period from January 1993 to December 2016 in Pediatric Rheumatology Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India. Nine children had pulmonary presentation of KD. A retrospective case review with respect to clinical presentation, radiological findings and treatment was done.

Results Pulmonary presentation of KD was seen in 1.6% patients. Mean age at diagnosis of KD was 2.9years (range 9months – 4 years). 77.8% patients had no features suggestive of KD either on history or at presentation. First sign of KD was noted at a mean duration of 17.6 days (range 6–28 days) from the onset of symptoms. Periungual desquamation was the most common clinical sign seen in 66.6% patients followed by erythematous rash and perianal desquamation in 33.3% patients each.

Persistent fever, thrombocytosis and elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were seen in all patients. Microbiological investigations showed evidence of infection in only 2 patients – methicillin sensitive staphylococcus aureus in pus and positive mycoplasma agglutinin titre in one patient each. Parenchymal consolidation was the most common radiological finding (100%) followed by pleural effusion (55.5%), empyema (33.3%) and pneumothorax (11.1%). Coronary artery abnormalities were evident on echocardiography in 22.2% patients with dilatation of right coronary artery and left main coronary artery in 1 patient each.

All patients received intravenous antimicrobials for pneumonia. Intravenous immunoglobulin (IVIG) at 2g/kg was given after a mean duration of 24 days of onset of fever. Fever, which was unresponsive to intravenous antimicrobials, responded to IVIG therapy in all except 2 patients which required a second dose of IVIG. 55.5% patients required intercostal drainage tube (ICDT) insertion, 2 patients required streptokinase and 1 patient each required video assisted thoracoscopic surgery (VATS) and decortication. Mean follow up period was 15.9 months (range 0–62 months).

Conclusions Pulmonary involvement in patients with KD is uncommon and is less commonly recognized. Unresolving pneumonia in a child who continues to be febrile despite adequate antimicrobials with elevated inflammatory markers can be a clue towards the diagnosis of KD. Early recognition can prevent delays in diagnosis and shorten the hospital stay.

Disclosure of Interest None declared