Pulmonary and Pancreatic Aspergillosis Infection in the Setting of Immunosuppression from Granulomatosis with Polyangiitis Treatment and Concomitant SARS-CoV-2 Infection

Abstract

Introduction Typical orofacial manifestations of granulomatosis with polyangiitis (GPA) include rhinitis and sinusitis, but salivary gland involvement is rare. Treatment of GPA increases risk of opportunistic infections. In light of the recent SARS-CoV-2 pandemic, this places this population in a particularly vulnerable position. Here we describe a case of suppurative parotitis as the presenting sign of GPA, treated with prednisone and cyclophosphamide, subsequently complicated by SARS-CoV-2 infection, disseminated MRSA infection, and invasive pulmonary aspergillosis (IPA) with aspergillosis of the pancreas. Presentation A 71-year-old male with COPD was admitted to hospital for progressive facial pain with left parotid gland swelling despite outpatient antibiotics. Basic laboratory workup was unremarkable. He developed hemoptysis, and CT chest revealed a new left upper lobe (LUL) cavitary lesion with bilateral nodules. Diagnostic bronchoscopy showed thickened, nodular mucosa in the LUL with luminal narrowing. Endobronchial biopsy showed inflammation with necrosis but no malignancy;culture showed no microorganisms. Parotid gland pus grew normal oral flora. CT-guided core biopsy of the LUL lesion showed fibrotic and necrotic tissue with inflammation and multi-nucleated giant cells, again without tumor cells. Initial autoimmune workup revealed ANA positivity, but he elected for discharge to outpatient autoimmune workup. Four weeks later he was admitted to another hospital with acute renal failure where testing revealed hypocomplementemia, elevated c-ANCA and anti-PR-3 antibodies. Renal biopsy demonstrated focal necrotizing and diffuse crescentic glomerulonephritis. A diagnosis of GPA was made and treatment with prednisone and cyclophosphamide was initiated. After three months of this regimen, he was re-admitted to our facility for SARSCoV- 2 infection. Hospital course was complicated by MRSA endocarditis and presumed fungal pneumonia. Despite aggressive treatment of both, he developed septic shock and ultimately expired. Autopsy revealed invasive aspergillus in the lungs and necrotizing pancreatitis from aspergillus. Discussion Salivary gland involvement is a rare manifestation of GPA and documented infrequently in case reports. The presence of parotitis in a patient with hemoptysis and negative malignant or infectious workup should prompt the consideration of GPA. Importantly, IPA associated with SARS-CoV-2 infection in immunocompetent patients has been frequently documented in the literature, and immunosuppressed individuals such as this patient are surely at increased risk. The rare, incidental finding of aspergillus invading the pancreas on autopsy was likely related to his immunocompromised state. Given the high mortality rate, there should be a low threshold to treat for presumed IPA in patients with SARS-CoV-2 infection for which secondary infection is suspected.