Minimally Invasive Surgical Approach in Granulomatosis with Polyangiitis Complicated with Intramural Descending Aorta Hematoma Followed by Aortic Wall Rupture
<b>Background and Clinical Significance</b>: Granulomatosis with polyangiitis (GPA) represents a rare autoimmune disease with granulomatous inflammation, tissue necrosis, and systemic vasculitis of the small and medium blood vessels. Although the clinical elements vary, aortic involvemen...
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2025-01-01
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| author | Mihai-Lucian Ciobica Alexandru-Sebastian Botezatu Zoltan Galajda Mara Carsote Claudiu Nistor Bianca-Andreea Sandulescu |
| author_facet | Mihai-Lucian Ciobica Alexandru-Sebastian Botezatu Zoltan Galajda Mara Carsote Claudiu Nistor Bianca-Andreea Sandulescu |
| author_sort | Mihai-Lucian Ciobica |
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| description | <b>Background and Clinical Significance</b>: Granulomatosis with polyangiitis (GPA) represents a rare autoimmune disease with granulomatous inflammation, tissue necrosis, and systemic vasculitis of the small and medium blood vessels. Although the clinical elements vary, aortic involvement is exceptional and it represents a challenge that requires a rapid intervention with the potential of displaying a fulminant evolution. <b>Case Presentation</b>: We report a 64-year-old male with an 18-year history of GPA who presented atypical low back pain. Following ultrasound and computed tomography exams, the initial suspicion was an intramural descending aorta hematoma, surrounded by a peri-aortic sleeve suggesting a chronic inflammation. Serial non-invasive assessments revealed a progressive lesion within the next 10 to 12 days to an aortic wall rupture, despite the absence of previous aneurysmal changes. The peri-aortic fibrous inflammatory sleeve was life-saving, and emergency minimally invasive surgery was successful, including the massive improvement in back pain. <b>Conclusions</b>: To our knowledge, this is a very rare scenario in GPA; we found only 18 other cases (the oldest report being from 1994). An interventional approach was mentioned in a few cases as seen in this instance. Glucocorticoid medication for GPA might act as a potential contributor to symptomatic osteoporotic fractures which require a prompt differential diagnosis. Unusual aortic manifestations (such as intramural aortic hematoma or aortic wall rupture) are difficult to recognize since the index of clinical suspicion is rather low. A prompt intervention may be life-saving and a multidisciplinary team is mandatory. Minimally invasive surgical correction of the aortic event represents an optimum management in the modern era. Such cases add to the limited data we have so far with respect to unusual outcomes in long-standing GPAs. |
| format | Article |
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| institution | Kabale University |
| issn | 2075-4418 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj-art-519f81689a534db085d9f41005e89c842025-01-24T13:28:53ZengMDPI AGDiagnostics2075-44182025-01-0115214410.3390/diagnostics15020144Minimally Invasive Surgical Approach in Granulomatosis with Polyangiitis Complicated with Intramural Descending Aorta Hematoma Followed by Aortic Wall RuptureMihai-Lucian Ciobica0Alexandru-Sebastian Botezatu1Zoltan Galajda2Mara Carsote3Claudiu Nistor4Bianca-Andreea Sandulescu5Department of Internal Medicine and Gastroenterology, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, RomaniaDepartment of Angiography and Cardiac Catheterization, “Dr. Carol Davila” Central Military Emergency University Hospital, 010825 Bucharest, RomaniaClinical Department of Cardiovascular Surgery, “Dr. Carol Davila” Central Military Emergency University Hospital, 010825 Bucharest, RomaniaDepartment of Endocrinology, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, RomaniaDepartment 4-Cardio-Thoracic Pathology, Thoracic Surgery II Discipline, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, RomaniaDepartment of Internal Medicine and Gastroenterology, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania<b>Background and Clinical Significance</b>: Granulomatosis with polyangiitis (GPA) represents a rare autoimmune disease with granulomatous inflammation, tissue necrosis, and systemic vasculitis of the small and medium blood vessels. Although the clinical elements vary, aortic involvement is exceptional and it represents a challenge that requires a rapid intervention with the potential of displaying a fulminant evolution. <b>Case Presentation</b>: We report a 64-year-old male with an 18-year history of GPA who presented atypical low back pain. Following ultrasound and computed tomography exams, the initial suspicion was an intramural descending aorta hematoma, surrounded by a peri-aortic sleeve suggesting a chronic inflammation. Serial non-invasive assessments revealed a progressive lesion within the next 10 to 12 days to an aortic wall rupture, despite the absence of previous aneurysmal changes. The peri-aortic fibrous inflammatory sleeve was life-saving, and emergency minimally invasive surgery was successful, including the massive improvement in back pain. <b>Conclusions</b>: To our knowledge, this is a very rare scenario in GPA; we found only 18 other cases (the oldest report being from 1994). An interventional approach was mentioned in a few cases as seen in this instance. Glucocorticoid medication for GPA might act as a potential contributor to symptomatic osteoporotic fractures which require a prompt differential diagnosis. Unusual aortic manifestations (such as intramural aortic hematoma or aortic wall rupture) are difficult to recognize since the index of clinical suspicion is rather low. A prompt intervention may be life-saving and a multidisciplinary team is mandatory. Minimally invasive surgical correction of the aortic event represents an optimum management in the modern era. Such cases add to the limited data we have so far with respect to unusual outcomes in long-standing GPAs.https://www.mdpi.com/2075-4418/15/2/144autoimmunegranulomatosis with polyangiitisWegener’s granulomatosisaortic ruptureminimally invasive surgeryaortic wall |
| spellingShingle | Mihai-Lucian Ciobica Alexandru-Sebastian Botezatu Zoltan Galajda Mara Carsote Claudiu Nistor Bianca-Andreea Sandulescu Minimally Invasive Surgical Approach in Granulomatosis with Polyangiitis Complicated with Intramural Descending Aorta Hematoma Followed by Aortic Wall Rupture Diagnostics autoimmune granulomatosis with polyangiitis Wegener’s granulomatosis aortic rupture minimally invasive surgery aortic wall |
| title | Minimally Invasive Surgical Approach in Granulomatosis with Polyangiitis Complicated with Intramural Descending Aorta Hematoma Followed by Aortic Wall Rupture |
| title_full | Minimally Invasive Surgical Approach in Granulomatosis with Polyangiitis Complicated with Intramural Descending Aorta Hematoma Followed by Aortic Wall Rupture |
| title_fullStr | Minimally Invasive Surgical Approach in Granulomatosis with Polyangiitis Complicated with Intramural Descending Aorta Hematoma Followed by Aortic Wall Rupture |
| title_full_unstemmed | Minimally Invasive Surgical Approach in Granulomatosis with Polyangiitis Complicated with Intramural Descending Aorta Hematoma Followed by Aortic Wall Rupture |
| title_short | Minimally Invasive Surgical Approach in Granulomatosis with Polyangiitis Complicated with Intramural Descending Aorta Hematoma Followed by Aortic Wall Rupture |
| title_sort | minimally invasive surgical approach in granulomatosis with polyangiitis complicated with intramural descending aorta hematoma followed by aortic wall rupture |
| topic | autoimmune granulomatosis with polyangiitis Wegener’s granulomatosis aortic rupture minimally invasive surgery aortic wall |
| url | https://www.mdpi.com/2075-4418/15/2/144 |
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