Submandibular salivary gland involvement in granulomatosis with polyangiitis

Introduction Granulomatosis with polyangiitis (GPA) is one of the forms of small vessel vasculitis. It is a rare condition that needs a high degree of suspicion to reach the diagnosis. It is one of the causes of diffuse parenchymal lung disease, with a very wide differential diagnosis. It is commonly misdiagnosed with malignant, granulomatous, and infectious lung diseases. Case presentation We report a case of a 31-year-old male who presented with productive cough, shortness of breath, hemoptysis, nasal obstruction, and epistaxis together with submandibular salivary gland swelling. Diagnosis of GPA was based on characteristic cavitary lung lesions, nasal and salivary gland involvement, pathological samples that revealed necrotizing granulomatous inflammation, characteristic positive Cytoplasmic- ANCA (C-ANCA), together with exclusion of malignancy and tuberculosis. Conclusion GPA is a rare condition. Salivary gland involvement should raise suspicion about GPA, in addition to other systemic manifestations.