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Granulomatosis with polyangiitis (GPA) is a rare small-vessel vasculitis associated with high mortality without appropriate treatment. Acute ST-elevation myocardial infarction (STEMI) has been reported as an atypical presentation of GPA. We report a case of STEMI, shortly followed by subacute in-stent thrombosis with extensive thrombus burden in a 53-year-old male patient with undiagnosed GPA. After aggressive treatment with triple therapy consisting of aspirin, clopidogrel and rivaroxaban, He started to have haemoptysis. Despite the discontinuation of aspirin, he ended up with massive haemoptysis and acute respiratory failure necessitating endotracheal intubation. CT of the chest revealed bilateral ground-glass opacities consistent with diffuse alveolar haemorrhage. Extensive workup revealed positive antiproteinase 3 antibodies; hence, a diagnosis of GPA was made. He was treated with induction therapy consisting of methylprednisolone, mycophenolate mofetil, cyclophosphamide and rituximab, leading to a gradual improvement in his clinical conditions and subsequent extubation., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)