Chronic aortic dissection as a cause of fever of unknown origin.ABSTRACT
Chronic aortic dissection presenting as a prolonged febrile syndrome is an uncommon condition. We believe that only 22 cases have been previously reported. We present a case of a patient with an aortic dissection whose diagnosis was delayed because persistent fever, malaise, and night sweats dominated his clinical picture. These complaints may be accompanied by an increased erythrocyte sedimentation rate Erythrocyte Sedimentation Rate Definition
The erythrocyte sedimentation rate (ESR), or sedimentation rate (sed rate), is a measure of the settling of red blood cells in a tube of blood during one hour. , leukocytosis Leukocytosis Definition
Leukocytosis is a condition characterized by an elevated number of white cells in the blood.
Leukocytosis is a condition that affects all types of white blood cells. , thrombocytosis, and anemia of chronic disease anemia of chronic disease Hematology A form of anemia that accounts for1⁄4 of all anemias in hospitalized Pts; it is the predominant form of hypoproliferative anemia, and seen in Pts with arthritis, chronic infections, and malignancy, . Knowledge of this atypical presentation, a high degree of suspicion, and investigation using an adequate imaging method will help to avoid missing this potentially lethal entity.
THE PRESENTATION of aortic dissection may be more subtle than' the classic textbook description of an acutely ill patient with excruciating chest pain of abrupt onset. (1) In rare cases, chronic aortic dissection can have atypical prominent features, such as persistent fever, night sweats, malaise, and elevated acute phase response acute phase response
A group of physiologic changes that occur shortly after the onset of an infection or other inflammatory process and include an increase in the blood level of various proteins, especially C-reactive protein, fever, and other laboratory parameters. (2-15) This misleading clinical picture may cause the underlying diagnosis to be delayed and may prompt an extensive but unfruitful search for a systemic illness. We describe one such case in which the patient had prolonged high fever associated with chronic dissection of the thoracic aorta that was not detected when he was first admitted to hospital. We also review reported cases of patients with chronic aortic dissection and prolonged febrile syndrome.
A 61-year-old man was admitted to our hospital for evaluation of self-limited, moderately intense upper abdominal pain radiating to the back, of sudden onset, associated with a 2-week history of fever and profuse night sweats. Seventeen days before admission, the patient was seen in the emergency department after an abrupt episode of dull retrosternal and upper abdominal pain that radiated to the left arm and was accompanied by diaphoresis diaphoresis /di·a·pho·re·sis/ (-fah-re´sis) sweating, especially of a profuse type.
Perspiration, especially when copious and medically induced. . At that time, he was found to have blood pressure of 210/120 mm Hg and a tortuous aorta shown by a chest x-ray film, but normal findings on abdominal ultrasonography, unremarkable serial electrocardiograms, and normal cardiac enzyme values. Once the blood pressure was controlled, he was sent home. However, 2 days after this episode, night sweats and fever developed with daily temperature spikes up to 38.8[degrees]C (101.8[degrees]F). The patient's medical history was significant only for hypertension of 6 years' duration, treated with captopril captopril /cap·to·pril/ (kap´to-pril) an angiotensin-converting enzyme inhibitor used in the treatment of hypertension, congestive heart failure, and post–myocardial infarction left ventricular dysfunction. and atenolol atenolol /aten·o·lol/ (ah-ten´ah-lol) a cardioselective ß used in the treatment of hypertension and chronic angina pectoris and the prophylaxis and treatment of myocardial infarction and cardiac arrhythmias. .
Physical examination on admission was unremarkable, except for a temperature of 39[degrees]C, an enlarged right axillary ax·il·lar·y
Relating to the axilla.
Located in or near the armpit.
Mentioned in: Mastectomy
of or pertaining to the armpit. lymph node, and blood pressure of 160/100 mm Hg. Serial cardiac enzyme determinations and electrocardiographic electrocardiographic
emanating from or pertaining to electrocardiography.
maintenance of a more or less continuous surveillance of a patient's cardiac status by means of electrocardiography. findings were normal. A chest x-ray film disclosed only a tortuous aorta. Urinalysis, abdominal x-ray films, and ultrasonography showed no abnormalities. Initial laboratory data revealed a white blood cell count white blood cell count,
n a diagnostic clinical laboratory test to determine the number and types of leukocytes present in a measured sample of blood. Overall the normal number of leukocytes ranges from 5000 to 10,000/mm3. of 13,700/[mm.sup.3], a platelet count of 426,000/[mm.sup.3], and an elevated erythrocyte sedimentation rate (ESR ESR - Eric S. Raymond ) (84 mm/hr), with normal hemoglobin and biochemical test values. Serum albumin and globulin globulin, any of a large family of proteins of a spherical or globular shape that are widely distributed throughout the plant and animal kingdoms. Many of them have been prepared in pure crystalline form. levels were normal, as were results of protein electrophoresis. Additional studies included several negative blood and urine cultures, repeated negative serologic tests for various infectious agents (syphilis, typhoid fever, brucella Brucella /Bru·cel·la/ (broo-sel´ah) a genus of schizomycetes (family Brucellaceae). B. abor´tus causes infectious abortion in cattle and is the most common cause of brucellosis in humans. B. , rickettsiae, toxoplasma Toxoplasma /Toxo·plas·ma/ (tok?so-plaz´mah) a genus of sporozoa that are intracellular parasites of many organs and tissues of birds and mammals, including humans. T. gon´dii is the etiologic agent of toxoplasmosis. , hepatitis B and C viruses, Epstein-Barr virus, and cytomegalovirus) and autoantibodies, and normal C3 and C4 level s. Although a purified protein derivative purified protein derivative
see purified protein derivative of tuberculin. skin test was positive, an extensive search for acid-fast bacilli (including mycobacterial cultures of samples from blood, urine, sputum, and right axillary lymph node) was negative.
Gallium and technetium scans, upper gastrointestinal series Upper GI series, also upper gastrointestinal (GI) tract radiography, is a radiologic examination of the upper gastrointestinal tract. It consists of a series of X-ray images of the esophagus, stomach and duodenum. , abdominal computed tomography (CT), and ocular funduscopy were all unremarkable. Transthoracic echocardiography showed mild left ventricular hypertrophy left ventricular hypertrophy Cardiology Enlargement of the left ventricle often linked to the prolonged hemodynamic stress of CHF, characterized by myocardial cell hypertrophy, ↑ left ventricular wall thickness, ↓ ventricular compliance, ↑ , but no pericardial effusion or vegetations. However, thoracic CT revealed widening of the descending aorta and an intramural intramural /in·tra·mu·ral/ (-mu´r'l) within the wall of an organ.
Occurring or situated within the walls of a cavity or organ. thrombus thrombus /throm·bus/ (throm´bus) pl. throm´bi a stationary blood clot along the wall of a blood vessel, frequently causing vascular obstruction. within a false lumen in the left anterolateral anterolateral /an·tero·lat·er·al/ (an?ter-o-lat´er-al) situated anteriorly and to one side.
In front and away from the middle line. wall of the descending thoracic aorta, which extended from the level of the origin of the left subclavian artery to just above the diaphragm (Fig 1). This finding was confirmed by transesophageal echocardiography (Fig 2) and thoracic magnetic resonance imaging magnetic resonance imaging (MRI), noninvasive diagnostic technique that uses nuclear magnetic resonance to produce cross-sectional images of organs and other internal body structures. (MRI 1. (application) MRI - Magnetic Resonance Imaging.
2. MRI - Measurement Requirements and Interface. ) (Fig 3).
Chronic aortic dissection type III was diagnosed, and conservative medical management was chosen after surgical consultations. Until the eighth hospital day, the patient had daily spiking temperatures to 39[degrees]C. During this time, he had only one brief episode of mild, dull, retrosternal pain, but electrocardiogram electrocardiogram /elec·tro·car·dio·gram/ (-kahr´de-o-gram?) a graphic tracing of the variations in electrical potential caused by the excitation of the heart muscle and detected at the body surface. and cardiac enzyme levels remained normal. Over the subsequent days, fever spontaneously remitted, and he was discharged on the 23rd hospital day, receiving a regimen of hypotensive hypotensive /hy·po·ten·sive/ (-ten´siv) marked by low blood pressure or serving to reduce blood pressure.
1. Of or characterized by low blood pressure.
2. medications (captopril and atenolol at higher doses), which controlled his blood pressure at approximately 120/80 mm Hg. The patient has remained afebrile afebrile /afe·brile/ (a-feb´ril) without fever.
afebrile adjective Feverless , and the laboratory parameters of acute inflammatory response have returned to normal levels.
Aortic dissection is usually an acute event characterized by excruciating chest pain and other classic symptoms of sudden onset, although it might also present in a more subtle manner, making diagnosis difficult. (1,16) Fever during the acute phase of aortic dissection is frequent; however, chronic aortic dissection manifested as a prolonged febrile syndrome associated with constitutional symptoms and an elevated ESR is extremely uncommon. (17) Previous case reports of chronic aortic dissection presenting as a prolonged febrile syndrome are listed in the Table. (2-15) Among the 23 cases to date (including our case), 10 of the aortic dissections occurred in hypertensive patients, but an absence of any known predisposing condition was not so uncommon. Although most of the patients first presented with chest, back, or abdominal pain, this was self-limited or of low intensity in many and was overshadowed by their systemic symptoms and laboratory findings, which dominated the clinical pictures. Aortography aortography /aor·tog·ra·phy/ (a?or-tog´rah-fe) radiography of the aorta after introduction into it of a contrast material.
n. was the study that established the diagnosis in most cases reported before the development and widespread use of newer imaging methods.
Thus, aortic dissection may closely mimic other entities such as an infectious, neoplastic neoplastic /neo·plas·tic/ (ne?o-plas´tik)
1. pertaining to a neoplasm.
2. pertaining to neoplasia.
pertaining to neoplasia or a neoplasm. , or autoimmune disease, with persistent fever, night sweats, malaise, and increased laboratory parameters of acute phase reaction. As in our case, the systemic symptoms may dominate the clinical picture and mask the underlying diagnosis. On review of our patient's symptoms, it is evident that although he had some of the features of aortic dissection (chest and/or abdominal pain, hypertension, diaphoresis), these were misinterpreted because fever and night sweats became the prominent complaints, which along with an elevated ESR, raised the question of an underlying systemic disorder. (16) Fever prompted an extensive search that initially failed to reveal any infectious, neoplastic, or autoimmune disease to explain the pyrexia pyrexia /py·rex·ia/ (pi-rek´se-ah) pl. pyrex´iae fever.pyrex´ial
The mechanism by which aortic dissections may produce fever and a systemic inflammatory response remains a matter of conjecture. Some authors have postulated that active dissecting hematoma hematoma /he·ma·to·ma/ (he?mah-to´mah) a localized collection of extravasated blood, usually clotted, in an organ, space, or tissue. with marked tissue destruction can cause release of cytokines, which would be responsible for the inflammatory reaction. (2,3) Similarly, the cessation of the active dissection and the resolution of tissue ischemia (with resorption resorption /re·sorp·tion/ (re-sorp´shun)
1. the lysis and assimilation of a substance, as of bone.
n. of the sequestered blood) may lead to the disappearance of both the fever and the inflammatory response.
Continued fever led to a thorough study for fever of unknown origin Fever of Unknown Origin Definition
Fever of unknown origin (FUO) refers to the presence of a documented fever for a specified time, for which a cause has not been found after a basic medical evaluation. , which included thoracic CT. The incidental finding of a dissection involving the descending thoracic aorta was unexpected. The development of new reliable, less invasive diagnostic procedures such as MRI, helical CT, or transesophageal echocardiography has modified the approach to and evaluation of suspected aortic dissection, to the extent of even precluding the need for aortography. (18) Although MRI is currently considered to be the method of choice, this technique cannot be used on hemodynamically unstable patients. (19,20) On the other hand, helical CT and transesophageal echocardiography are not only more readily available in the emergency setting, but also can be done on patients who need to be closely monitored. (21,22) When the diagnosis of chronic aortic dissection is suspected, MRI is probably the preferred method to confirm the dissection and the most appropriate technique for preoperative evaluation, provided surgical interventi on is indicated. (18) Our patient's aortic dissection (type III DeBakey or type B Stanford) was managed medically after surgical consultations.
Important clinical, diagnostic, and therapeutic implications are associated with this challenging and potentially lethal condition. Chronic aortic dissection should be considered as a possible cause of fever of unknown origin, especially if there is evidence of previous hypertension and a relatively recent episode of chest, back, or abdominal pain. Knowledge of this atypical presentation and investigation using an appropriate imaging method will result in earlier diagnosis and better patient prognosis.
TABLE. Reported Cases of Chronic Aortic Dissection Manifested as Prolonged Febrile Syndrome (N = 23) (2-15) No. (%) Sex Male 15 (65) Female 8 (35) Medical history Hypertension 10 (44) Unremarkable 10 (44) Other 3 (13) Clinical course Prolonged fever 23 (100) Malaise 10 (44) Night sweats 6 (26) Weight loss 7 (30) Congestive heart failure 3 (13) Pleural effusion 4 (17) Pericardial effusion 2 (9) Laboratory abnormalities * Elevated elevated sedimentation rate 20 (91) Decreased hemoglobin/hematocrit values 11 (50) Increased white blood cell count 6 (27) Increased platelet count 6 (27) Diagnosis first established by Aortography 5 (22) Abdominal ultrasonography 2 (9) Computed tomography 11 (48) Transthoracic echocardiography 2 (9) Transesophageal echocardiography 2 (9) Emergency thoracotomy 1 (4) Type of dissection (DeBakey classification) Type I 11 (48) Type II 3 (13) Type III 9 (39) Type of treatment * Conservative 11 (50) Surgery 9 (41) Initially conservative, surgery later 2 (9) * Not reported in one case.
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RELATED ARTICLE: KEY POINTS
* Chronic aortic dissection can misleadingly present with persistent fever, malaise, weight less, and elevated acute phase response laboratory parameters.
* This atypical presentation may prompt an extensive but unfruitful search for a systemic disorder.
* Chronic aortic dissection should be considered as a possible cause of fever of unknown origin, especially in hypertensive patients with a recent episode of chest, back, or abdominal pain.
* Investigation using an appropriate imaging technique will result in earlier diagnosis and better patient prognosis.
From the Departments of Radiology and Internal Medicine, La Paz University Hospital, Madrid, Spain.
Reprint requests to Luis Gorospe, MD, La Paz University Hospital, Department of Radiology, [P.sup.0] de la Castellana 261, 28046 Madrid, Spain.