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A Rare Case of Granulomatous Polyangiitis With Lim ...
A Rare Case of Granulomatous Polyangiitis With Limited Renal Involvement
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This case report describes a 56-year-old man diagnosed with granulomatosis with polyangiitis (GPA), a rare necrotizing granulomatous vasculitis of small to medium vessels that most often affects the upper airways, lungs, and kidneys. The patient presented with worsening headaches, fevers, chills, malaise, and major weight loss after a recently treated dental abscess. He also had lymphadenopathy and elevated inflammatory markers, but initial basic labs and urinalysis were largely normal.<br /><br />Further evaluation showed positive ANCA with c-ANCA titer 1:40 and elevated proteinase 3 antibodies, supporting GPA. Infectious workup, including blood cultures and testing for fungal and other infections, was negative. Brain MRI showed acute sinusitis, chest X-ray showed bilateral consolidative opacities, and chest CT revealed multiple cavitary pulmonary nodules. Biopsies and bronchoscopy found chronic granulomatous inflammation and necrotizing inflammatory changes, with no evidence of malignancy, tuberculosis, or fungal disease.<br /><br />The patient was diagnosed with GPA and started on ampicillin/sulbactam, prednisone, methotrexate, and folic acid, with clinical improvement.<br /><br />The report emphasizes that this was a case of “limited GPA,” meaning disease predominantly involved the respiratory tract with only minimal renal involvement. Although the patient had microscopic hematuria, he had no proteinuria, red blood cell casts, or impaired kidney function. The authors highlight that limited GPA may be misdiagnosed because it does not always present with the classic triad of upper airway, lung, and kidney disease. Early recognition is important because prompt immunosuppressive treatment improves outcomes.
Asset Subtitle
Sara Hassan
Meta Tag
Author List
Keir F. MacKay, Margaret Beliveau, Nosher Ali, Sara Hassan, Waseem Ahmad
Category
Clinical Vignettes
Concept
Granulomatosis with Polyangiitis
Concept
Renal Disease
Concept
Antineutrophil cytoplasmic antibody
Concept
Biopsy
Concept
Upper Respiratory Tract
Distinguished
Non-Finalist
Presenter Organization
Ocean University Medical Center
Presenting Author
Sara Hassan
Track
Adult
Keywords
granulomatosis with polyangiitis
GPA
c-ANCA
proteinase 3 antibodies
necrotizing granulomatous vasculitis
cavitary pulmonary nodules
sinusitis
limited GPA
methotrexate
prednisone
Granulomatosis with Polyangiitis
Renal Disease
Antineutrophil cytoplasmic antibody
Biopsy
Upper Respiratory Tract
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