Teenage heartache: A 14-year-old with pleuritic chest pain

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A 14-year-old transgender male presented with 1 month of worsening intermittent pleuritic chest pain, 2–3 weeks after an upper respiratory infection. A review of systems revealed mild fatigue, low appetite and mild frontal headache without red flags. Examination revealed left-sided ptosis, persistent tachycardia (max 125 bpm), pulsus paradoxus, distant heart sounds and decreased air entry at lung bases. No pericardial rub. C-reactive protein (CRP) 201.9 mg/L (high). Blood count showed microcytic anaemia (haemoglobin 77 g/L). Serum electrolytes, creatinine, urinalysis, liver enzymes and coagulation profile were normal. Troponin 4 ng/L (high). Echocardiogram showed significant pericardial effusion with right atrial collapse. Chest x-ray revealed small pleural effusions (Figure 1). Urgent pericardiocentesis was performed, and Naproxen was started for presumed viral pericarditis. ... Rheumatology was consulted after limited improvement on Naproxen and a negative infectious workup (including tuberculosis). Ophthalmic exam showed inflammatory dacryoadenitis. CT orbit revealed a left lacrimal mass (Figure 2). Biopsy showed scar tissue with few plasma cells, no granulomas or eosinophils, and negative IgG4 staining. Chest CT showed non-specific ground-glass opacities and mild diffuse interlobular septal thickening. Pulmonary function test (PFT) showed a mixed obstructive-restrictive pattern. Serum ANA and IgG4 normal. Myeloperoxidase (MPO)-ANCA was strongly positive >134 U/L, informing diagnosis and management.

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