Portal de Periódicos da CAPES

Brachial artery occlusion secondary to hypereosinophilic syndrome

2005; Elsevier BV; Volume: 42; Issue: 4 Linguagem: Inglês

10.1016/j.jvs.2005.05.050

1097-6809

Todd A. Ponsky, Fredrick Brody, Joseph Giordano, Ryan Garcia, David E. Kardon, Arnold M. Schwartz,

IL-33, ST2, and ILC Pathways

Hypereosinophilic syndrome (HES) is characterized by an overproduction of eosinophils that leads to organ damage. Although most cases of HES frequently affect the lungs, heart, and gastrointestinal tract, there are a few reported cases of peripheral vascular involvement. We report a case of a patient with a history of colonic HES who presented with idiopathic occlusion of the brachial artery. A 28-year-old woman with a recent history of eosinophilic colitis presented with a several-week history of left hand pain, pallor, and paresthesias. Her hand was cool, without palpable pulses. Her eosinophilia count was 38%. An arteriogram documented a left brachial artery occlusion and diffuse left arm vasospasm. A brachial-to-brachial bypass was performed. Postoperatively, there was extensive vasospasm of her distal upper extremity arteries, which was treated with calcium-channel blockers and steroids. Her symptoms resolved and she has been asymptomatic for 9 months. The segment of occluded artery was found to contain many eosinophils on histologic examination. HES of the arterial system is an exceedingly rare cause of occlusion. Our patient presented with eosinophilia, arterial vasospasm with subsequent occlusion, and the presence of eosinophilic infiltration on the pathologic specimen. These data, combined with the patient’s previous history, demonstrate that the patient’s occlusion may have been secondary to HES. Hypereosinophilic syndrome (HES) is characterized by an overproduction of eosinophils that leads to organ damage. Although most cases of HES frequently affect the lungs, heart, and gastrointestinal tract, there are a few reported cases of peripheral vascular involvement. We report a case of a patient with a history of colonic HES who presented with idiopathic occlusion of the brachial artery. A 28-year-old woman with a recent history of eosinophilic colitis presented with a several-week history of left hand pain, pallor, and paresthesias. Her hand was cool, without palpable pulses. Her eosinophilia count was 38%. An arteriogram documented a left brachial artery occlusion and diffuse left arm vasospasm. A brachial-to-brachial bypass was performed. Postoperatively, there was extensive vasospasm of her distal upper extremity arteries, which was treated with calcium-channel blockers and steroids. Her symptoms resolved and she has been asymptomatic for 9 months. The segment of occluded artery was found to contain many eosinophils on histologic examination. HES of the arterial system is an exceedingly rare cause of occlusion. Our patient presented with eosinophilia, arterial vasospasm with subsequent occlusion, and the presence of eosinophilic infiltration on the pathologic specimen. These data, combined with the patient’s previous history, demonstrate that the patient’s occlusion may have been secondary to HES. Hypereosinophilic syndrome (HES) refers to a group of leukoproliferative disorders characterized by an overproduction of eosinophils that leads to organ damage. The diagnosis of HES requires a sustained eosinophil count of >1500 cells/mL for >6 months without any other obvious cause of eosinophilia. Patients must also have signs and symptoms of organ involvement. HES may affect any organ in the body, and the clinical manifestations can vary. HES of the cardiac system most commonly leads to congestive heart failure secondary to endomyocardial fibrosis. Neurologic manifestations include embolic or thrombotic strokes and transient ischemic attacks that are secondary to HES cardiac involvement or an idiopathic hypercoagulable state. Involvement of the hematologic system entails splenomegaly with left upper-quadrant pain. When the pulmonary system becomes involved, patients usually experience a persistent cough or dyspnea. Rheumatologic symptoms can also be present and include arthralgias and myalgias. When the gastrointestinal system is affected, the symptoms are usually nonspecific and include nausea, vomiting, or abdominal pain. Constitutional symptoms of fever, night sweats, anorexia, and weight loss are also associated with the systemic nature of HES.1D’Souza M.G. Swistel D.G. Castro J.L. DeRose Jr, J.J. Hypereosinophilic thrombus causing aortic stenosis and myocardial infarction.Ann Thorac Surg. 2003; 76: 1725-1726Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar Most cases of HES frequently affect the lungs, heart, and gastrointestinal tract, although a few cases of peripheral vascular involvement have been reported.2Aoki M. Kimura Y. Kusunoki T. Tahara S. Kawanah S. Angiolymphoid hyperplasia with eosinophilia associated with anomalous dilatation of occipital artery IL-5 and VEGF expression of lesional mast cells.Arch Dermatol. 2002; 138: 982-984Crossref PubMed Scopus (27) Google Scholar, 3Arnold M. Geilen C.C. Coupland S.E. Krengel S. Dippel E. Sproder J. et al.Unilateral angiolymphoid hyperplasia with eosinophilia involving the left arm and hand.J Cutan Pathol. 1999; 26: 436-440Crossref PubMed Scopus (45) Google Scholar, 4Aurello P. Cicchini C. D’Angelo F. Di Gioia C.R. D’Amati G. Angiolymphoid hyperplasia with eosinophilia a rare artery lesion.Anticancer Res. 2003; 23: 3069-3072PubMed Google Scholar, 5Kimura Y. Tsutsumi T. Kuroishikawa Y. Kishimoto S. Angiolymphoid hyperplasia with eosinophilia arising from the facial artery.J Laryngol Otol. 2003; 117: 570-573Crossref PubMed Scopus (19) Google Scholar, 6Kobayashi H. Moriya T. Tagami H. Protruding tumorous angiolymphoid hyperplasia with eosinophilia (ALHE) of the scalp accompanied by arterial occlusion.J Dermatol. 2002; 29: 38-42Crossref PubMed Scopus (9) Google Scholar, 7Krapohl B.D. Machens H.G. Reichert B. Mailander P. A rare vasoproliferative lesion angiolymphoid hyperplasia with eosinophilia of the hand.Br J Plast Surg. 2003; 56: 168-170Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar, 8Sandstad E. Aksnes H. Sund S. Reinholt F.P. Recurrent angiolymphoid hyperplasia with eosinophilia mimicking temporal arteritis associated with nephrotic syndrome.Clin Nephrol. 2003; 59: 206-211Crossref PubMed Scopus (15) Google Scholar, 9Stephan D. Lipsker D. Sens P. Hassani S. Welsch M. Hypereosinophilia-induced digital necrosis in a smoking patient.Int Angiol. 2003; 22: 95-97PubMed Google Scholar Many of these cases are secondary to angiolymphoid hyperplasia, however. Angiolymphoid hyperplasia is an uncommon condition manifested by skin plaques and nodules in the head and neck area. On biopsy, angiolymphoid hyperplasia has a distinct histologic description, including highly proliferative blood vessels, large endothelial cells, and inflammatory infiltrates with eosinophils within the blood vessels. We report a case of a patient with a history of colonic HES who presented with idiopathic occlusion of the brachial artery and histologic evidence of leukoproliferation with eosinophilia. A 28-year-old woman with a recent history of eosinophilic colitis presented with left hand pain. The patient had presented 1 year earlier with diarrhea and abdominal pain. Colonoscopy demonstrated an inflamed segment of colon with eosinophilic infiltration and hypereosinophilia on a complete blood count. A diagnosis of HES was made. She was treated with steroids, with resolution of her symptoms for approximately 9 months. A recurrent episode of similar abdominal pain and diarrhea occurred later and resolved with steroids. One month after this second flare-up, the patient presented with a 4-month history of left hand pain with periods of numbness and pallor. The symptoms were not related to any particular season, activity, time of day, temperature, or medication. She denied any history of trauma, including any recent vascular interventions or catheter placements. She also denied tobacco use or any significant past medical history. On physical exam, the patient’s left hand was blue, cool, and edematous. Sensation to pain, temperature, and soft touch were intact. She had a faintly palpable brachial artery pulse, but the radial and ulnar arteries were not palpable. An angiogram revealed complete occlusion of the midpoint of the brachial artery with reconstitution just proximal to its bifurcation. Intra-arterial nitroglycerin (200 μg) was given directly into the brachial artery, but the artery did not dilate. Attempts to cross the occluded segment with guidewires were unsuccessful. There was no evidence of atherosclerotic or other peripheral vascular disease (Fig 1). The patient had a significantly elevated eosinophilia count of 38% and an absolute eosinophil count of 8.53 × 103 (white blood cell count, 22.45 × 103). The patient’s hypercoaguable workup was normal and included prothrombin time/partial thromboplastin time, antiphospholipid antibodies (lupus anticoagulants and anticardiolipin antibodies), and factor V Leiden mutation. The patient had normal levels of homocysteine, protein C, and protein S. High-dose steroid therapy was initiated based on this patient’s past response to steroids. The patient’s eosinophil count decreased to a normal level of 1500 cells/mL that persists for >6 months without any other obvious cause of eosinophilia. Patients must have signs and symptoms of organ involvement. In patients without evidence of organ involvement, the diagnosis of benign eosinophilia is made.10Hardy W.R. Anderson R.E. The hypereosinophilic syndromes.Ann Intern Med. 1968; 68 (Jun): 1220-1229Crossref PubMed Scopus (408) Google Scholar, 11Chusid M.J. Dale D.C. West B.C. Wolff S.M. The hypereosinophilic syndrome analysis of fourteen cases with review of the literature.Medicine (Baltimore). 1975; 54: 1-27Crossref PubMed Scopus (1196) Google Scholar Although the etiology of HES is unclear, there is evidence of T cell clonal proliferation with interleukin-5 (IL-5) hypersecretion. IL-5 plays a role in the differentiation of eosinophils,1D’Souza M.G. Swistel D.G. Castro J.L. DeRose Jr, J.J. Hypereosinophilic thrombus causing aortic stenosis and myocardial infarction.Ann Thorac Surg. 2003; 76: 1725-1726Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar but the pathophysiology of IL-5 and eosinophilia differentiation is not completely understood. It is known, however, that eosinophils spend approximately 8 days maturing in the bone marrow before they are released to the periphery. A variety of transcription factors within the bone marrow, including GATA-1, GATA-2, and c/EBP, regulate eosinophil production. In addition to these transcription factors, a variety of growth factors allow the lineage of eosinophils to mature. IL-5 induces the production, expansion, and release of eosinophils from the bone marrow. Multiple animal studies demonstrate that overproduction of IL-5 leads to hypereosinophilia, whereas a deletion of the IL-5 gene reduces the number of eosinophils.14Bainton D. Developmental biology of neutrophils and eosinophils.in: Gallin J. Goldstein I. Snyderman R. Inflammation basic principles and clinical correlates. 2nd ed. Raven Press, New York1992: 303Google Scholar, 15Gleich G. Adolphson C. Leiferman K. The biology of the eosinophilic leukocyte.Annu Rev Med. 1993; 44: 85Crossref PubMed Scopus (311) Google Scholar, 16Rothenberg M.E. Eosinophilic gastrointestinal disorders (EGID).J Allergy Clin Immunol. 2004; 113 (quiz, 29): 11-28Abstract Full Text Full Text PDF PubMed Scopus (708) Google Scholar Also, food allergies may be associated with HES, as 75% of patients improve with a hypoallergenic diet.16Rothenberg M.E. Eosinophilic gastrointestinal disorders (EGID).J Allergy Clin Immunol. 2004; 113 (quiz, 29): 11-28Abstract Full Text Full Text PDF PubMed Scopus (708) Google Scholar Eosinophils are recruited to sites of inflammation by IL-5 and induced and stimulated by granulocyte-macrophage colony-stimulating factor. When eosinophils arrive, they typically undergo degranulation followed by apoptosis. Compared with neutrophils, eosinophils survive longer in the tissues. As a result of their longer survival and granule components, eosinophils are associated with a greater inflammatory reaction and chronic reparative changes, particularly in conditions such as HES. Eosinophils release toxic cationic proteins, including eosinophil cationic protein (ECP), eosinophil peroxidase, eosinophil-derived neurotoxin, major basic protein (MBP), and platelet activating factor. These granules are particularly toxic against parasites. In the process of destroying foreign microbes, these toxins mediate host tissue damage. The effects of hypereosinophilia on the airway are better understood than the effects on the vascular system. The eosinophilic granulocytes cause contraction of the airway smooth muscle, alter vascular permeability, and promote mucus secretion. Ultimately, these changes lead to respiratory distress. These types of changes are commonly seen in asthmatic patients. Although HES frequently causes pulmonary injury, the heart and gastrointestinal tract are also affected. In reference to the heart, there are several reports of myocardial fibrosis, aortic stenosis and congestive heart failure from HES.1D’Souza M.G. Swistel D.G. Castro J.L. DeRose Jr, J.J. Hypereosinophilic thrombus causing aortic stenosis and myocardial infarction.Ann Thorac Surg. 2003; 76: 1725-1726Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar, 11Chusid M.J. Dale D.C. West B.C. Wolff S.M. The hypereosinophilic syndrome analysis of fourteen cases with review of the literature.Medicine (Baltimore). 1975; 54: 1-27Crossref PubMed Scopus (1196) Google Scholar, 12Herrin VE, Files JC, Zhou Y, Schick P. Hypereosinophilic syndrome. Available at: http://www.emedicine.com/med/topic1076.htm Last accessed Jul 7, 2005.Google Scholar, 17Lorenzoni R. Cortigiani L. Melosi A. Cardiac involvement in idiopathic hypereosinophilic syndrome.Heart. 2002; 87: 553Crossref PubMed Scopus (6) Google Scholar, 18Terai M. Yasukawa K. Honda T. Jibiki T. Hirano K. Sato J. et al.Peripheral blood eosinophilia and eosinophil accumulation in coronary microvessels in acute Kawasaki disease.Pediatr Infect Dis J. 2002; 21: 777-781Crossref PubMed Scopus (46) Google Scholar In the gastrointestinal tract, eosinophilia can cause inflammation of the esophagus, stomach, small intestine, and colon. These patients often present with complaints of vomiting, abdominal pain, diarrhea, melena, or failure to thrive.16Rothenberg M.E. Eosinophilic gastrointestinal disorders (EGID).J Allergy Clin Immunol. 2004; 113 (quiz, 29): 11-28Abstract Full Text Full Text PDF PubMed Scopus (708) Google Scholar Reports of peripheral vascular occlusion associated with HES are anecdotal. Stephan et al 9Stephan D. Lipsker D. Sens P. Hassani S. Welsch M. Hypereosinophilia-induced digital necrosis in a smoking patient.Int Angiol. 2003; 22: 95-97PubMed Google Scholar reported the development of Raynaud’s phenomenon with associated hypereosinophilia. As seen in our patient, there was angiographically documented occlusion of the small and medium arteries of the arm with vasospasm. Others have reported eosinophilic thrombotic occlusion of the portal vein, coronary microvessels, the facial artery, and the occipital artery.1D’Souza M.G. Swistel D.G. Castro J.L. DeRose Jr, J.J. Hypereosinophilic thrombus causing aortic stenosis and myocardial infarction.Ann Thorac Surg. 2003; 76: 1725-1726Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar, 2Aoki M. Kimura Y. Kusunoki T. Tahara S. Kawanah S. Angiolymphoid hyperplasia with eosinophilia associated with anomalous dilatation of occipital artery IL-5 and VEGF expression of lesional mast cells.Arch Dermatol. 2002; 138: 982-984Crossref PubMed Scopus (27) Google Scholar, 3Arnold M. Geilen C.C. Coupland S.E. Krengel S. Dippel E. Sproder J. et al.Unilateral angiolymphoid hyperplasia with eosinophilia involving the left arm and hand.J Cutan Pathol. 1999; 26: 436-440Crossref PubMed Scopus (45) Google Scholar, 5Kimura Y. Tsutsumi T. Kuroishikawa Y. Kishimoto S. Angiolymphoid hyperplasia with eosinophilia arising from the facial artery.J Laryngol Otol. 2003; 117: 570-573Crossref PubMed Scopus (19) Google Scholar, 6Kobayashi H. Moriya T. Tagami H. Protruding tumorous angiolymphoid hyperplasia with eosinophilia (ALHE) of the scalp accompanied by arterial occlusion.J Dermatol. 2002; 29: 38-42Crossref PubMed Scopus (9) Google Scholar, 8Sandstad E. Aksnes H. Sund S. Reinholt F.P. Recurrent angiolymphoid hyperplasia with eosinophilia mimicking temporal arteritis associated with nephrotic syndrome.Clin Nephrol. 2003; 59: 206-211Crossref PubMed Scopus (15) Google Scholar, 13Kikuchi K. Minami K. Miyakawa H. Ishibashi M. Portal vein thrombosis in hypereosinophilic syndrome.Am J Gastroenterol. 2002; 97: 1274-1275Crossref PubMed Scopus (11) Google Scholar, 18Terai M. Yasukawa K. Honda T. Jibiki T. Hirano K. Sato J. et al.Peripheral blood eosinophilia and eosinophil accumulation in coronary microvessels in acute Kawasaki disease.Pediatr Infect Dis J. 2002; 21: 777-781Crossref PubMed Scopus (46) Google Scholar There have been several reports documenting coronary artery vasospasm with hypereosinophilia.19Gersh B.J. Bassendine M.F. Forman R. Walls R.S. Beck W. Coronary artery spasm and myocardial infarction in the absence of angiographically demonstrable obstructive coronary disease.Mayo Clin Proc. 1981; 56: 700-708PubMed Google Scholar, 20Takagi S. Goto Y. Hirose E. Terashima M. Sakuragi S. Suzuki S. et al.Successful treatment of refractory vasospastic angina with corticosteroids coronary arterial hyperactivity caused by local inflammation?.Circ J. 2004; 68: 17-22Crossref PubMed Scopus (65) Google Scholar, 21Horigome H. Sekijima T. Ohtsuka S. Shibasaki M. Life threatening coronary artery spasm in childhood Kimura’s disease.Heart. 2000; 84: E5Crossref PubMed Scopus (13) Google Scholar, 22Okada H. Koganei H. Yoshioka S. Enta K. Suzuki K. Kato J. et al.[Multi-vasospastic angina refractory to medical therapy caused by hyperthyroid stage of chronic thyroiditis and hypereosinophilia a case report].J Cardiol. 2000; 35: 189-196PubMed Google Scholar, 23Takahashi N. Kondo K. Aoyagi J. Acute myocardial infarction associated with hypereosinophilic syndrome in a young man.Jpn Circ. 1997; 61: 803-806Crossref PubMed Scopus (16) Google Scholar, 24Hertzman P.A. Maddoux G.L. Sternberg E.M. Heyes M.P. Mefford I.N. Kephart G.M. et al.Repeated coronary artery spasm in a young woman with the eosinophilia-myalgia syndrome.JAMA. 1992; 267: 2932-2934Crossref PubMed Scopus (22) Google Scholar, 25Kounis N.G. Zavras G.M. Histamine-induced coronary artery spasm the concept of allergic angina.Br J Clin Pract. 1991; 45: 121-128PubMed Google Scholar, 26Hirakawa Y. Koyanagi S. Matsumoto T. Takeshita A. Nakamura M. A case of variant angina associated with eosinophilia.Am J Med. 1989; 87: 472-474Abstract Full Text PDF PubMed Scopus (19) Google Scholar Hertzman et al24Hertzman P.A. Maddoux G.L. Sternberg E.M. Heyes M.P. Mefford I.N. Kephart G.M. et al.Repeated coronary artery spasm in a young woman with the eosinophilia-myalgia syndrome.JAMA. 1992; 267: 2932-2934Crossref PubMed Scopus (22) Google Scholar described a woman with eosinophilia-myalgia syndrome who presented with repeated episodes of coronary artery vasospasm. They proposed that eosinophilic secretory products caused arterial vasospasm.24Hertzman P.A. Maddoux G.L. Sternberg E.M. Heyes M.P. Mefford I.N. Kephart G.M. et al.Repeated coronary artery spasm in a young woman with the eosinophilia-myalgia syndrome.JAMA. 1992; 267: 2932-2934Crossref PubMed Scopus (22) Google Scholar Other illnesses such as Buerger’s disease are associated with highly cellular, thrombotic processes and can present similarly to our patient. One of the most common aspects of Buerger’s disease is a substantial smoking history, and most individuals are from Japan, Israel, and India. Our patient, however, did not smoke and was from the United States. Morphologically, the thrombus associated with Buerger’s disease contains microabscesses with a central focus of neutrophils. Ultimately, this process entails contiguous involvement of veins and nerves encased in fibrous tissue. One case report cannot prove a relationship, causal or otherwise, between HES and arterial occlusive disease; however, a confluence of findings support such a relationship. This patient is a young woman with no risk factors or other evidence of atherosclerotic occlusive disease. She had an elevated white blood cell count with 38% eosinophils, eosinophils within the brachial artery specimen, and intense vasospasm noted on the operative angiogram. Since eosinophils can induce arterial vasospasm, smooth muscle contraction, and Raynaud’s phenomenon, we are suggesting that this type of vasospasm was mediated by hypereosinophilia that caused chronic arterial changes and eventually thrombosis. Although HES may be a rare and unusual disease, the presence of this disease may be a risk factor for arterial disease.