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Fever of unknown origin in aortic dissection

Fieber unklarer Genese bei Aortendissektion

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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

  1. Asouhidou I, Asteri T (2009) Acute aortic dissection: be aware of misdiagnosis. BMC Res Notes 2:25

    Article  PubMed  PubMed Central  Google Scholar 

  2. 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

    Article  PubMed  Google Scholar 

  3. 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

    PubMed  PubMed Central  Google Scholar 

  4. 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

    Article  PubMed  Google Scholar 

  5. Petersdorf RG, Beeson PB (1961) Fever of unexplained origin: Report on 100 cases. Medicine (Baltimore) 40:1–30

    Article  CAS  Google Scholar 

  6. Durack DT, Street AC (1991) Fever of unknown origin – reexamined and redefined. Curr Clin Top Infect Dis 11:35–51

    CAS  PubMed  Google Scholar 

  7. Knockaert DC, Vanderschueren S, Blockmans D (2003) Fever of unknown origin in adults: 40 years on. J Intern Med 253(3):263–275

    Article  CAS  PubMed  Google Scholar 

  8. 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

    Article  PubMed  Google Scholar 

  9. 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

    Article  CAS  PubMed  Google Scholar 

  10. 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

    PubMed  Google Scholar 

  11. Cheng CC, Lin CY, Han CL (2007) Intramural haematoma of the aorta presenting as fever of unknown origin. Acta Cardiol 62(4):409–411

    Article  PubMed  Google Scholar 

  12. Dai MS, Cheng SM (2001) Aortic dissection presenting as fever of unknown origin. Acta Cardiol 56(1):37–38

    Article  CAS  PubMed  Google Scholar 

  13. 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

    Article  PubMed  Google Scholar 

  14. 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

    Article  PubMed  Google Scholar 

  15. Geppert AG, Mahvi A, Hainaut P, Lambert M (1998) Chronic aortic dissection masquerading as systemic disease. Acta Clin Belg 53(1):19–21

    Article  CAS  PubMed  Google Scholar 

  16. 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

    Article  CAS  PubMed  Google Scholar 

  17. 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

    Article  PubMed  Google Scholar 

  18. 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

    Article  CAS  PubMed  Google Scholar 

  19. 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

    CAS  PubMed  Google Scholar 

  20. 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

    Article  PubMed  Google Scholar 

  21. 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

    PubMed  Google Scholar 

  22. 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

    Article  PubMed  Google Scholar 

  23. 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

    Article  PubMed  Google Scholar 

  24. 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

    Article  Google Scholar 

  25. Mackowiak PA, Lipscomb KM, Mills LJ, Smith JW (1976) Dissecting aortic aneurysm manifested as fever of unknown origin. JAMA 236(15):1725–1727

    Article  CAS  PubMed  Google Scholar 

  26. McKeown PP, Campbell NP (1989) Pyrexia of unknown origin and aortic dissection. Int J Cardiol 25(1):124–126

    Article  CAS  PubMed  Google Scholar 

  27. 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

    Article  PubMed  Google Scholar 

  28. 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

    Article  CAS  PubMed  Google Scholar 

  29. 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

    Article  PubMed  Google Scholar 

  30. Murray HW, Mann JJ, Genecin A, McKusick VA (1976) Fever with dissecting aneurysm of the aorta. Am J Med 61(1):140–144

    Article  CAS  PubMed  Google Scholar 

  31. 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

    Article  CAS  PubMed  Google Scholar 

  32. 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

    Article  PubMed  Google Scholar 

  33. 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

    PubMed  PubMed Central  Google Scholar 

  34. Raza K, King P, Allison SP (1999) Back pain and fever. Postgrad Med J 75(879):51–53

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Roca B (2003) Chronic aortic dissection as a cause of fever of unknown origin. South Med J 96(5):530

    Article  PubMed  Google Scholar 

  36. Roth T, Mainguene C, Boiselle JC (2003) Acute acalculous cholecystitis associated with aortic dissection: Report of a case. Surg Today 33(8):633–635

    Article  PubMed  Google Scholar 

  37. Ruderman A, Mackowiak PA, Smith JW (1979) Fever as a manifestation of dissecting aneurysm of aorta. Am J Cardiol 44(3):581–582

    Article  CAS  PubMed  Google Scholar 

  38. 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

    PubMed  Google Scholar 

  39. Schattner A, Klepfish A, Caspi A (1996) Chronic aortic dissection presenting as a prolonged febrile disease and arterial embolization. Chest 110(4):1111–1114

    Article  CAS  PubMed  Google Scholar 

  40. Sinnamon K, Wiggam MI (2007) An unusual cause of pyrexia of unknown origin in an 81 year old lady. Ulster Med J 76:117

    PubMed  PubMed Central  Google Scholar 

  41. Smith MA, Singer C (1988) Fever of unknown origin: unusual presentation of dissecting aortic aneurysm. Am J Med 85(1):126–127

    Article  CAS  PubMed  Google Scholar 

  42. Suzuki C, Ueno T, Nishijima H, Haga R, Miki Y, Arai A et al (2011) Fever of unknown origin. Lancet 378(9804):1756

    Article  PubMed  Google Scholar 

  43. 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

    Article  CAS  PubMed  Google Scholar 

  44. Turner N, Pusey CD (1990) Aortic dissection masquerading as systemic disease – the post-dissection syndrome. Q J Med 75(277):525–531

    CAS  PubMed  Google Scholar 

  45. 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

    Article  Google Scholar 

  46. 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

    PubMed  PubMed Central  Google Scholar 

  47. 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

    Article  PubMed  PubMed Central  Google Scholar 

  48. Yuan SM, Tager S, Raanani E (2009) Fever of unknown origin as a primary presentation of chronic aortic dissection. Vascular 17(4):230–233

    Article  PubMed  Google Scholar 

  49. 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

    Article  PubMed  Google Scholar 

  50. Kouchoukos NT, Dougenis D (1997) Surgery of the thoracic aorta. N Engl J Med 336(26):1876–1888

    Article  CAS  PubMed  Google Scholar 

  51. 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

    Article  Google Scholar 

  52. 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

    Google Scholar 

  53. 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

    Article  PubMed  Google Scholar 

  54. 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

    Article  CAS  PubMed  Google Scholar 

  55. 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

    Article  PubMed  Google Scholar 

  56. 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)

    Article  PubMed  Google Scholar 

  57. Ponraj P, Pepper J (1992) Aortic dissection. Br J Clin Pract 46(2):127–131

    CAS  PubMed  Google Scholar 

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Correspondence to S.-M. Yuan.

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S.-M. Yuan declares that he has no competing interests.

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U. Lange, Bad Nauheim

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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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