TY - JOUR
TI - MPO–ANCA-Positive Granulomatosis with Polyangiitis with Rapidly Progressive Glomerulonephritis and Saddle-Nose Deformity: A Case Report
AU - Petrou, D.
AU - Karagiannis, M.
AU - Nikolopoulos, P.
AU - Liapis, G.
AU - Lionaki, S.
JO - Human Antibodies
PY - 2022
VL - 11
TODO - 2
SP - 33
PB - MDPI
SN - 1093-2607, 1875-869X
TODO - 10.3390/antib11020033
TODO - 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
TODO - 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.
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