A 14-year-old female with constitutional mismatch repair deficiency syndrome and recurrent glioblastoma multiforme was referred for respiratory evaluation due to chronic cough and abnormal chest imaging.
Six months earlier, she had developed an intermittent dry cough occurring during both the day and night, occasionally associated with rhinorrhoea and mildly increased work of breathing. There was no wheezing, chest pain, persistent fever or haemoptysis. Pulmonary function tests were normal.
She had been receiving immune checkpoint inhibition with nivolumab (anti-programmed death 1) for 2 years without significant side effects. Following the diagnosis of a new glioblastoma, dual checkpoint inhibition with nivolumab–relatlimab (anti-lymphocyte-activation gene 3) was initiated 18 months prior to the referral. No other immune checkpoint inhibitor-related organ toxicity had been identified before the respiratory presentation.
Initial CT scan of the chest demonstrated multifocal nodular opacities with ill-defined margins and mild surrounding ground-glass attenuation involving bronchovascular and peripheral regions (