Τίτλος:
MPO–ANCA-Positive Granulomatosis with Polyangiitis with Rapidly Progressive Glomerulonephritis and Saddle-Nose Deformity: A Case Report
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Early diagnosis and initiation of appropriate immunosuppressive treatment remain the cornerstone of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis at the cost of significant toxicity. In this report, we present a case of a 69-year-old female who presented with advanced renal insufficiency and evidence of pulmonary hemorrhage and was MPO–ANCA-positive with a clinical phenotype of granulomatosis with polyangiitis. Organ involvement included rapidly progressive glomerulonephritis (GN), along with extrarenal manifestations (skin, upper and lower respiratory system involvement, and onset of saddle-nose deformity). Kidney biopsy established the diagnosis of pauci-immune crescentic, sclerotic GN. She received therapy with glucocorticoids and cyclophosphamide, mainly due to life-threatening extra-renal manifestations, such as pulmonary hemorrhage. She avoided vasculitis-related death but she developed severe therapy-related toxicity, resulting in the discontinuation of immunosuppressive therapy. Continuous re-evaluation of patients with ANCA-associated vasculitis in terms of response to immunosuppressive therapy and treatment-related toxicity is crucial for their management. © 2022 by the authors. Licensee MDPI, Basel, Switzerland.
Συγγραφείς:
Petrou, D.
Karagiannis, M.
Nikolopoulos, P.
Liapis, G.
Lionaki, S.
Περιοδικό:
Human Antibodies
Λέξεις-κλειδιά:
aciclovir; allopurinol; amlodipine; atorvastatin; creatinine; cyclophosphamide; entecavir; fluconazole; folic acid; gliclazide; glucocorticoid; hemoglobin; hemosiderin; levothyroxine; metformin; methylprednisolone; metoprolol; myeloperoxidase; neutrophil cytoplasmic antibody; omeprazole; rituximab; rivaroxaban; urea, aged; anemia; aphthous stomatitis; Article; bronchoscopy; case report; clinical article; computer assisted tomography; drug dose reduction; drug withdrawal; edema; erythrocyte; estimated glomerular filtration rate; female; fibrosing alveolitis; follow up; glomerulosclerosis; hematuria; Herpes simplex virus; histopathology; human; immune complex deposition; immunofluorescence; immunosuppressive treatment; kidney biopsy; kidney failure; low back pain; lumbar spine fracture; lung hemorrhage; lung lavage; oral mucositis; phenotype; proteinuria; rapidly progressive glomerulonephritis; saddle nose; serology; skin necrosis; toxicity; Wegener granulomatosis
DOI:
10.3390/antib11020033
