MPO–ANCA-Positive Granulomatosis with Polyangiitis with Rapidly Progressive Glomerulonephritis and Saddle-Nose Deformity: A Case Report
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 insuff...
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MDPI AG
2022-05-01
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| Series: | Antibodies |
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| Online Access: | https://www.mdpi.com/2073-4468/11/2/33 |
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| author | Dimitra Petrou Minas Karagiannis Petros Nikolopoulos George Liapis Sophia Lionaki |
| author_facet | Dimitra Petrou Minas Karagiannis Petros Nikolopoulos George Liapis Sophia Lionaki |
| author_sort | Dimitra Petrou |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-10T00:35:25Z |
| format | Article |
| id | doaj.art-727e852dd50745e5b03c5bb109739dd4 |
| institution | Directory Open Access Journal |
| issn | 2073-4468 |
| language | English |
| last_indexed | 2024-03-10T00:35:25Z |
| publishDate | 2022-05-01 |
| publisher | MDPI AG |
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| series | Antibodies |
| spelling | doaj.art-727e852dd50745e5b03c5bb109739dd42023-11-23T15:18:16ZengMDPI AGAntibodies2073-44682022-05-011123310.3390/antib11020033MPO–ANCA-Positive Granulomatosis with Polyangiitis with Rapidly Progressive Glomerulonephritis and Saddle-Nose Deformity: A Case ReportDimitra Petrou0Minas Karagiannis1Petros Nikolopoulos2George Liapis3Sophia Lionaki4Department of Nephrology, National and Kapodistrian University of Athens, Attikon Hospital, 12462 Athens, GreeceDepartment of Nephrology, National and Kapodistrian University of Athens, Attikon Hospital, 12462 Athens, GreeceDepartment of Nephrology, National and Kapodistrian University of Athens, Attikon Hospital, 12462 Athens, GreeceDepartment of Pathology, National and Kapodistrian University of Athens, Laiko Hospital, 11527 Athens, GreeceDepartment of Nephrology, National and Kapodistrian University of Athens, Attikon Hospital, 12462 Athens, GreeceEarly 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.https://www.mdpi.com/2073-4468/11/2/33vasculitisANCAkidneyear nose thorat involvement |
| spellingShingle | Dimitra Petrou Minas Karagiannis Petros Nikolopoulos George Liapis Sophia Lionaki MPO–ANCA-Positive Granulomatosis with Polyangiitis with Rapidly Progressive Glomerulonephritis and Saddle-Nose Deformity: A Case Report Antibodies vasculitis ANCA kidney ear nose thorat involvement |
| title | MPO–ANCA-Positive Granulomatosis with Polyangiitis with Rapidly Progressive Glomerulonephritis and Saddle-Nose Deformity: A Case Report |
| title_full | MPO–ANCA-Positive Granulomatosis with Polyangiitis with Rapidly Progressive Glomerulonephritis and Saddle-Nose Deformity: A Case Report |
| title_fullStr | MPO–ANCA-Positive Granulomatosis with Polyangiitis with Rapidly Progressive Glomerulonephritis and Saddle-Nose Deformity: A Case Report |
| title_full_unstemmed | MPO–ANCA-Positive Granulomatosis with Polyangiitis with Rapidly Progressive Glomerulonephritis and Saddle-Nose Deformity: A Case Report |
| title_short | MPO–ANCA-Positive Granulomatosis with Polyangiitis with Rapidly Progressive Glomerulonephritis and Saddle-Nose Deformity: A Case Report |
| title_sort | mpo anca positive granulomatosis with polyangiitis with rapidly progressive glomerulonephritis and saddle nose deformity a case report |
| topic | vasculitis ANCA kidney ear nose thorat involvement |
| url | https://www.mdpi.com/2073-4468/11/2/33 |
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