Abstract
Aortic dissection is the most devastating sequela of thoracic aortic disorder. Patients with acute aortic dissection typically manifest as an acute onset of severe chest pain, but occasionally present with atypical symptoms including fever of unknown origin. A total of 50 patients from 41 articles based on a complete literature retrieval were included in this study. More patients had a fever prior to pain. The time to presentation was 40.7 ± 105.6 days, the time to diagnosis was 52.9 ± 110.1 days, and the time to surgery/intervention was 1.8 ± 5.6 days. The patients’ temperature on admission was 38.2 ± 0.6 °C and the maximal temperature recorded was 38.8 ± 0.4 °C. Laboratory findings showed increased white blood cell counts, cardiac enzymes, and inflammatory biomarkers. More pronounced laboratory findings of the infectious type than the inflammatory type aortic dissection could be helpful in the differential diagnosis. Half of patients warrant aortic repair with or without valve replacement, less than half of patients were conservatively managed, and a few were interventionally treated or were being followed up. The mortality rate was 9.5 %. Physicians should always bear in mind aortic dissection when patients present with fever of unknown origin particularly in those without chest pain. Laboratory findings may offer inflammatory evidence for the diagnosis. An early diagnosis as well as subsequent treatment is indispensable for patients’ outcomes.
Zusammenfassung
Eine Aortendissektion ist die schwerwiegendste Folge einer Erkrankung der thorakalen Aorta. Patienten mit akuter Aortendissektion zeigen typischerweise plötzlich einsetzende starke Thoraxschmerzen; gelegentlich weisen sie jedoch atypische Symptome auf, zu denen auch Fieber unklarer Genese gehört. Auf der Grundlage einer umfangreichen Literatursammlung wurden insgesamt 50 Patienten aus 41 Beiträgen in die vorliegende Studie aufgenommen. Bei der Mehrzahl der Patienten bestand das Fieber vor den Schmerzen. Die Dauer bis zur Vorstellung beim Arzt betrug 40,7 ± 105,6 Tage, die Dauer bis zur Diagnosestellung betrug 52,9 ± 110,1 Tage, und die Dauer bis zur Operation/Intervention lag bei 1,8 ± 5,6 Tagen. Zum Zeitpunkt der stationären Aufnahme wiesen die Patienten eine Temperatur von 38,2 ± 0,6 °C auf, und die maximal dokumentierte Temperatur betrug 38,8 ± 0,4 °C. In Bezug auf die Laborbefunde zeigte sich eine Erhöhung der Leukozytenzahl, der Herzenzyme und der Entzündungsparameter. Bei Fieber vom infektiösen Typ Aortendissektion waren entsprechende Laborwerte deutlicher erhöht als bei Fieber vom inflammatorischen Typ, was für die Differenzialdiagnose hilfreich sein könnte. Die Hälfte der Patienten benötigte eine Aortenrekonstruktion mit oder ohne Klappenersatz, weniger als die Hälfte der Patienten wurden konservativ therapiert, und einige erhielten eine interventionelle Therapie oder wurden nachbeobachtet. Die Mortalitätsrate betrug 9,5 %. Ärzte sollten stets an eine Aortendissektion denken, wenn sich Patienten mit Fieber unklarer Genese vorstellen, insbesondere bei jenen ohne Thoraxschmerzen. Bei den Laborbefunden findet sich möglicherweise im Bereich der Entzündungswerte ein Hinweis auf die Diagnose. Für die Ergebnisse bei den Patienten ist eine frühzeitige Diagnosestellung mit anschließender Behandlung unabdingbar.
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References
Asouhidou I, Asteri T (2009) Acute aortic dissection: be aware of misdiagnosis. BMC Res Notes 2:25
Issa M, Avezum Á, Dantas DC, Almeida AF, Souza LC, Sousa AG (2013) Risk factors for pre, intra, and postoperative hospital mortality in patients undergoing aortic surgery. Rev Bras Cir Cardiovasc 28(1):10–21
Dias RR, Duncan JA, Vianna DS, de Faria LB, Fernandes F, Ramirez FJ, Mady C, Jatene FB (2015) Surgical treatment of complex aneurysms and thoracic aortic dissections with the Frozen Elephant Trunk technique. Rev Bras Cir Cardiovasc 30(2):205–210
Shimada S, Nakamura H, Kurooka A, Nishioka N, Sugimura K, Ino H et al (2007) Fever associated with acute aortic dissection. Circ J 71(5):766–771
Petersdorf RG, Beeson PB (1961) Fever of unexplained origin: Report on 100 cases. Medicine (Baltimore) 40:1–30
Durack DT, Street AC (1991) Fever of unknown origin – reexamined and redefined. Curr Clin Top Infect Dis 11:35–51
Knockaert DC, Vanderschueren S, Blockmans D (2003) Fever of unknown origin in adults: 40 years on. J Intern Med 253(3):263–275
Schoenhoff FS, Jungi S, Czerny M, Roost E, Reineke D, Matyas G et al (2013) Acute aortic dissection determines the fate of initially untreated aortic segments in Marfan syndrome. Circulation 127(15):1569–1575
Abad C (1995) Acute infectious mitroaortic endocarditis in association with acute aortic dissection. Surgical management of an unusual combination of diseases. Cardiovasc Surg 3(6):605–606
Blas-Macedo J, Marquez-Ramírez D, Gómez-Dominguez Jde J (2007) Aortic dissection presenting as a febrile disease and atrial fibrillation. Rev Invest Clin 59(1):87–89
Cheng CC, Lin CY, Han CL (2007) Intramural haematoma of the aorta presenting as fever of unknown origin. Acta Cardiol 62(4):409–411
Dai MS, Cheng SM (2001) Aortic dissection presenting as fever of unknown origin. Acta Cardiol 56(1):37–38
Davutoglu V, Kervancioglu S, Celkan A, Soydinc S, Dinckal H (2004) Painless intimointimal intussusception and fever of unknown origin: An unusual form of aortic dissection. Cardiology 102(1):1–3
García-Romo E, López-Medrano F, Llovet A, Lizasoain M, San Juan R, Aguado JM (2010) Fever due to inflammation in acute aortic dissection: Description and proposals for diagnostic and therapeutic management. Rev Esp Cardiol 63(5):602–606
Geppert AG, Mahvi A, Hainaut P, Lambert M (1998) Chronic aortic dissection masquerading as systemic disease. Acta Clin Belg 53(1):19–21
Giladi M, Pines A, Averbuch M, Hershkoviz R, Sherez J, Levo Y (1991) Aortic dissection manifested as fever of unknown origin. Cardiology 78(1):78–80
Gorospe L, Sendino A, Pacheco R, Alonso A, Barbado FJ, Vázquez JJ (2002) Chronic aortic dissection as a cause of fever of unknown origin. South Med J 95(9):1067–1070
Hoogendoorn EH, Oyen WJ, van Dijk AP, van der Meer JW (2003) Pneumococcal aortitis, report of a case with emphasis on the contribution to diagnosis of positron emission tomography using fluorinated deoxyglucose. Clin Microbiol Infect 9(1):73–76
Iino T, Eguchi K, Sakai M, Nagataki S, Ishijima M, Toriyama K (1992) Polyarteritis nodosa with aortic dissection: Necrotizing vasculitis of the vasa vasorum. J Rheumatol 19(10):1632–1636
Iwasa S, Fukushima Y, Moriguchi-Goto S (2011) Acute aortic dissection associated with undetected congenital bicuspid aortic valve and infective endocarditis diagnosed intraoperatively. Interact Cardiovasc Thorac Surg 12(1):82–83
Jenq CC, Chen YC, Huang JY, Wu CH, Yeh CN, Yeh CH et al (2006) Type B aortic dissection with early presentation mimicking acute pyelonephritis. J Nephrol 19(3):341–345
Kida K, Osada N, Isahaya K, Mikami T, Yoneyama K, Kongoji K et al (2007) Listeria endocarditis with acute thoracoabdominal aortic dissection. Intern Med 46(15):1209–1212
Kimura N, Adachi H, Adachi K, Hashimoto M, Yamaguchi A, Ino T (2008) Chronic type A aortic dissection associated with Listeria monocytogenes infection. Gen Thorac Cardiovasc Surg 56(8):417–420
Kondo T, Uehara T, Ikegami A, Hirota Y, Ikusaka M (2015) Bilateral trapezius ridge pain with sustained low-grade fever caused by aortic dissection. Can J Cardiol 9(31):1204.e17–1204.e18
Mackowiak PA, Lipscomb KM, Mills LJ, Smith JW (1976) Dissecting aortic aneurysm manifested as fever of unknown origin. JAMA 236(15):1725–1727
McKeown PP, Campbell NP (1989) Pyrexia of unknown origin and aortic dissection. Int J Cardiol 25(1):124–126
Mészáros I, Mórocz J, Szlávi J, Schmidt J, Tornóci L, Nagy L et al (2000) Epidemiology and clinicopathology of aortic dissection. Chest 117(5):1271–1278
Miyairi T, Inaba H, Matsumoto J, Tanaka K, Kanda J, Suzuki M (1998) Dissecting aortic aneurysm presenting as pyrexia of unknown origin: Report of a case. Surg Today 28(1):102–104
Mochizuki Y, Tanaka H, Morinaga Y, Okita Y, Hirata K (2015) Infective endarteritis associated with aortic dissection underlying bacterial meningitis. Eur Heart J 36(8):471
Murray HW, Mann JJ, Genecin A, McKusick VA (1976) Fever with dissecting aneurysm of the aorta. Am J Med 61(1):140–144
Niitsuma Y, Takahara Y, Sudo Y, Nakano H (2001) Acute type A aortic dissection associated with an aortic annular abscess. Ann Thorac Surg 72(6):2136–2137
Pak M, Ito S, Takeda M, Watanabe N, Sato H, Ito S et al (2014) A case of ascending aortic dissection and rupture caused by giant cell arteritis. Int Heart J 55(6):555–559
Patris V, Whiteley J, Argiriou O, Lama N, Georgiou H, Constantinou C et al (2014) Pyrexia in patients with uncontrolled systemic hypertension: could they have an aortic dissection? J Thorac Dis 6(12):E246–E248
Raza K, King P, Allison SP (1999) Back pain and fever. Postgrad Med J 75(879):51–53
Roca B (2003) Chronic aortic dissection as a cause of fever of unknown origin. South Med J 96(5):530
Roth T, Mainguene C, Boiselle JC (2003) Acute acalculous cholecystitis associated with aortic dissection: Report of a case. Surg Today 33(8):633–635
Ruderman A, Mackowiak PA, Smith JW (1979) Fever as a manifestation of dissecting aneurysm of aorta. Am J Cardiol 44(3):581–582
Russo A, Angeletti S, Lorino G, Venditti C, Falcone M, Dicuonzo G et al (2010) A case of Lactobacillus casei bacteraemia associated with aortic dissection: Is there a link? New Microbiol 33(2):175–178
Schattner A, Klepfish A, Caspi A (1996) Chronic aortic dissection presenting as a prolonged febrile disease and arterial embolization. Chest 110(4):1111–1114
Sinnamon K, Wiggam MI (2007) An unusual cause of pyrexia of unknown origin in an 81 year old lady. Ulster Med J 76:117
Smith MA, Singer C (1988) Fever of unknown origin: unusual presentation of dissecting aortic aneurysm. Am J Med 85(1):126–127
Suzuki C, Ueno T, Nishijima H, Haga R, Miki Y, Arai A et al (2011) Fever of unknown origin. Lancet 378(9804):1756
Svensson LG, Labib SB, Eisenhauer AC, Butterly JR (1999) Intimal tear without hematoma: An important variant of aortic dissection that can elude current imaging techniques. Circulation 99(10):1331–1336
Turner N, Pusey CD (1990) Aortic dissection masquerading as systemic disease – the post-dissection syndrome. Q J Med 75(277):525–531
Yamabe K, Mimura R, Yasaka Y (2004) Early diagnosis of type A aortic dissection using transthoracic echocardiography in a patient with fever of unknown origin. J Echocardiogr 2(3):78–79
Yamada S, Tokumoto M, Ohkuma T, Kansui Y, Wakisaka Y, Uchizono Y et al (2013) Slowly progressive and painless thoracic aortic dissection presenting with a persistent fever in an elderly patient: the usefulness of combined measurement of biochemical parameters. Case Rep Med 2013:498129. doi:10.1155/2013/498129
Yih Lim PC, Hua Lee JM, Chua YL, Chia S (2013) Staphylococcal thoracic aortitis complicated by aortic dissection. World J Emerg Med 4:154–156
Yuan SM, Tager S, Raanani E (2009) Fever of unknown origin as a primary presentation of chronic aortic dissection. Vascular 17(4):230–233
Svensjö S, Bengtsson H, Bergqvist D (1996) Thoracic and thoracoabdominal aortic aneurysm and dissection: An investigation based on autopsy. Br J Surg 83(1):68–71
Kouchoukos NT, Dougenis D (1997) Surgery of the thoracic aorta. N Engl J Med 336(26):1876–1888
Hirst AE Jr, Johns VJ Jr, Kime SW Jr (1958) Dissecting aneurysm of the aorta: A review of 505 cases. Medicine (Baltimore) 37(3):217–279
Komatsu Y, Kohno I, Watanabe S, Inoue Y, Sekiguchi M, Hashimoto A et al (1976) The clinical features of acute dissecting aneurism of aorta. Shinzo 8(2):143–152
Suzuki T, Distante A, Zizza A, Trimarchi S, Villani M, Salerno Uriarte JA et al (2009) Diagnosis of acute aortic dissection by D‑dimer. The International Registry of Acute Aortic Dissection Substudy on Biomarkers (IRAD-Bio) experience. Circulation 119(20):2702–2707 (for the IRAD-Bio Investigators) doi:10.1161/CIRCULATIONAHA.108.833004
Eggebrecht H, Naber CK, Bruch C, Kröger K, von Birgelen C, Schmermund A et al (2004) Value of plasma fibrin D‑dimers for detection of acute aortic dissection. J Am Coll Cardiol 44(4):804–809
Akutsu K, Sato N, Yamamoto T, Morita N, Takagi H, Fujita N et al (2005) A rapid bedside D‑dimer assay (cardiac D‑dimer) for screening of clinically suspected acute aortic dissection. Circ J 69(4):397–403
Harris KM, Strauss CE, Eagle KA, Hirsch AT, Isselbacher EM, Tsai TT et al (2011) Correlates of delayed recognition and treatment of acute type A aortic dissection: The International Registry of Acute Aortic Dissection (IRAD). Circulation 124(18):1911–1918 (for the International Registry of Acute Aortic Dissection (IRAD) Investigators)
Ponraj P, Pepper J (1992) Aortic dissection. Br J Clin Pract 46(2):127–131
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S.-M. Yuan declares that he has no competing interests.
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Yuan, SM. Fever of unknown origin in aortic dissection. Z Rheumatol 76, 364–371 (2017). https://doi.org/10.1007/s00393-016-0203-2
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DOI: https://doi.org/10.1007/s00393-016-0203-2