Abstract
Granulomatosis with polyangiitis (GPA) is a vasculitis of small and medium-sized vessels and presents with varying signs and symptoms. It includes upper and lower airway manifestations and glomerulonephritis with a positive antineutrophil cytoplasmic antibody (ANCA) in serology in 90% of cases. However, about 10% of cases with GPA can have negative serology, often resulting in a diagnostic delay. Obtaining a tissue pathology is needed to confirm GPA. Here we present a 77-year-old male who presented with generalized weakness and loss of appetite and was found to have glomerulonephritis and bilateral opacities in the lungs with a negative ANCA. He was diagnosed with ANCA negative granulomatosis with polyangiitis after a renal biopsy revealed necrotizing inflammation with crescent formation. He was successfully treated with systemic glucocorticoids and rituximab. In conclusion, prompt diagnosis and treatment of ANCA negative vasculitis are required to decrease mortality.
Highlights
Granulomatosis with polyangiitis (GPA) is a subtype of antineutrophil cytoplasmic antibody (ANCA)associated vasculitis, which commonly affects small and medium-sized vessels
There is a higher incidence of ANCA negative vasculitis in the younger population with an average of 54 years as reported in a retrospective study [4, 5]
A cohort study done by Shah et al demonstrated that 30% of ANCA positive patients had a diagnosis of GPA before renal biopsy whereas no ANCA negative patients were assigned a diagnosis
Summary
Granulomatosis with polyangiitis (GPA) is a subtype of antineutrophil cytoplasmic antibody (ANCA)associated vasculitis, which commonly affects small and medium-sized vessels. We describe a 77-year-old male who presented with generalized weakness and was found to have glomerulonephritis and bilateral lung opacities and was diagnosed with ANCA negative GPA. Differentials included acute glomerulonephritis, acute tubular nephritis, infiltrative disease, or vasculitis On further review, he had multiple admissions in the past year for recurrent pneumonia requiring intravenous antibiotics every time. Following the completion of treatment with rituximab, renal function stabilized with an eGFR of 25 ml/min/1.73 sqm (chronic kidney disease 4) and proteinuria decreased significantly.
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
