International Journal of Infectious Diseases
Volume 13, Issue 3 , Pages e97-e99, May 2009

Are patients with POEMS syndrome at increased risk of Salmonella aortitis?

  • Jooyun Lee

      Affiliations

    • Division of Infectious Diseases, Department of Medicine, Mount Sinai School of Medicine, New York, NY 10029, USA
    • Corresponding Author InformationCorresponding author. Division of Infectious Diseases, Department of Medicine, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029, USA. Tel.: +1 212 241 6741; fax: +1 212 534 3240.
    • ,
  • Shirish Huprikar

      Affiliations

    • Division of Infectious Diseases, Department of Medicine, Mount Sinai School of Medicine, New York, NY 10029, USA
    • ,
  • Stephen G. Jenkins

      Affiliations

    • Department of Pathology, Mount Sinai School of Medicine, New York, NY 10029, USA

Received 11 February 2008; received in revised form 27 May 2008; accepted 13 July 2008. published online 24 October 2008.

Corresponding Editor: Craig Lee

Article Outline

Summary 

We report a case of Salmonella infectious aortitis in a patient with POEMS (peripheral neuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome, possibly indicating that vasculopathy associated with POEMS syndrome may increase the risk of Salmonella endovascular infection.

Keywords: POEMS syndrome, Non-typhoidal Salmonella, Infectious aortitis, Aneurysm

 

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Introduction 

POEMS syndrome is a rare disorder of plasma cells, characterized by the presence of peripheral neuropathy (P), organomegaly (O), endocrinopathy (E), M-protein (M) and skin changes (S). Associated features may include peripheral edema, pleural effusions, ascites, sclerotic bone lesions and Castleman disease.1 Although the pathophysiologic mechanism is not well understood, a cytokine, vascular endothelial growth factor (VEGF), appears to play an important role in this disorder.2, 3 VEGF targets endothelial cells, promoting angiogenesis and increasing vascular permeability. POEMS syndrome may be associated with a micro- or macro-angiopathy, resulting in pulmonary hypertension, arterial or venous thrombosis, stroke or myocardial infarction; it is hypothesized that overproduction of VEGF may contribute to the development of these complications.1, 4, 5

Arterial aneurysms are not known to be associated with POEMS syndrome. VEGF, however, may contribute to the formation of aortic aneurysms, as increased expression of VEGF was observed in the aortic wall of patients with aortic aneurysm.6, 7 Infectious aortitis complicates 2.6% of all abdominal aortic aneurysms and is most commonly caused by non-typhoidal Salmonella.8

Thus, the development of aortic aneurysms (and subsequently infectious aortitis) and the vascular complications associated with POEMS might be linked pathophysiologically by the cytokine VEGF. We report a case of Salmonella infectious aortitis in a patient with POEMS syndrome.

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Case report 

A 65-year-old Caucasian male presented with septic shock. At initial evaluation, the patient’s temperature was 39.4°C, he was lethargic and his abdomen was diffusely tender. He was admitted to the intensive care unit for mechanical ventilation and vasopressor support. Computed tomographic (CT) angiography of his chest, abdomen and pelvis showed an ascending aortic aneurysm (Figure 1A) and an infrarenal abdominal aortic aneurysm with periaortic soft-tissue inflammation, consistent with infectious aortitis (Figure 1B) extending to the common iliac arteries bilaterally. The blood cultures grew Salmonella enterica subspecies enterica, which was susceptible to ciprofloxacin, ceftriaxone, chloramphenicol and trimethoprim-sulfamethoxazole. Within several days of intravenous ciprofloxacin therapy, the patient’s condition improved and mechanical ventilation and vasopressor therapy were discontinued.

  • View full-size image.
  • Figure 1. 

    CT angiography of the chest, abdomen and pelvis. (A) Aneurysmal dilation of the aortic root. (B) Dilation of the infrarenal abdominal aorta, with adjacent soft-tissue mass and surrounding inflammatory changes.

Upon further evaluation after stabilization, the patient reported progressive weakness in the lower extremities and difficulty walking for six weeks. Physical examination revealed slight right-sided ptosis, decreased motor power and sensation in the lower extremities with a patchy distribution, gynecomastia, splenomegaly, pitting edema and multiple small macules over the anterior aspects of both lower extremities.

Laboratory studies showed persistent mild pancytopenia, slightly elevated creatinine, a serum M-protein level of 1.8gm/dl with IgG kappa type, and low total and free testosterone. Serum rapid plasma reagin and cryoglobulin were negative, and thyroid-stimulating hormone, free T4 and fasting glucose were normal. Ultrasonography of the abdomen revealed moderate ascites and bilateral pleural effusion. Bone marrow biopsy was non-diagnostic, showing patchy hypercellularity with plasma cells of 0.5%; an abdominal fat pad biopsy was negative for amyloidosis. Electromyography showed diffuse demyelinating motor and sensory polyneuropathy. Echocardiography showed moderate to severe pulmonary hypertension, and magnetic resonance angiography of the brain showed a 4mm saccular aneurysm of the left vertebral artery. The diagnosis of POEMS syndrome was made based on the presence of peripheral neuropathy, splenomegaly, hypogonadism, paraproteinemia and rash, in conjunction with extravascular volume overload and pulmonary hypertension. Despite high-dose steroid therapy and therapeutic plasmapheresis, the polyneuropathy did not improve and the patient remained bedridden. Ciprofloxacin was continued for six weeks and there was no recurrence of bacteremia.

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Discussion 

Non-typhoidal Salmonella causes nearly one-third of all cases of infectious aortitis and most patients with Salmonella aortitis have pre-existing vascular disease at the site of the infection.8 The diagnosis of infectious aortitis is usually based on characteristic findings on imaging studies and isolation of causative organisms from blood cultures; histologic examination of the affected aorta is seldom performed. The majority of cases involve the infrarenal abdominal aorta (33%), as was suspected in our patient based on the CT findings. It is also possible that the thoracic aorta was infected in this patient; up to 17% of Salmonella aortitis cases involve the thoracic aorta and infectious aortitis may present as an expanding aneurysm without obvious soft-tissue inflammation upon radiography.8, 9 Although endovascular infection has not been previously described in patients with POEMS syndrome, it seems theoretically possible because any vascular lesion in which there is endothelial damage may be at increased risk of superimposed infection. The overproduction of VEGF, characteristic of this syndrome, appears to be capable of causing endothelial damage in human and animal models.5, 10, 11 An elevated level of VEGF has been suggested for inclusion in the recently proposed diagnostic criteria of POEMS syndrome.1 However, serum VEGF levels appear to be 10–50 times higher than plasma levels of VEGF12 and it is yet to be determined whether the serum or plasma level is the better indicator.

The diagnosis of POEMS syndrome has mainly been based on characteristic clinical manifestations in conjunction with several laboratory findings. Our patient met the proposed diagnostic criteria,1 but had two distinctive features. His plasma cell clones were kappa type, whereas 95% of cases of POEMS syndrome were reported to have monoclonal lambda type light chain.1 In a smaller case series, however, kappa type was more common than lambda type light chain.13 The anemia and thrombocytopenia diagnosed in our patient are also unusual in POEMS syndrome. This might have been due to the splenomegaly or the preceding sepsis.

Our case raises the question of whether the presence of POEMS syndrome might increase the risk of aneurysm formation and/or endovascular infection.

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Acknowledgements 

We thank Dr. Gary P. Wormser for his helpful advice.

Conflict of interest: No conflict of interest to declare.

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References 

  1. Dispenzieri A. POEMS syndrome. Blood Rev. 2007;21:285–299
  2. Watanabe O, Arimura K, Kitajima I, Osame M, Maruyama I. Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome. Lancet. 1996;347:702
  3. Soubrier M, Dubost JJ, Serre AF, Ristori JM, Sauvezie B, Cathebras P, et al. Growth factors in POEMS syndrome: evidence for a marked increase in circulating vascular endothelial growth factor. Arthritis Rheum. 1997;40:786–787
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  10. Fukatsu A, Tamai H, Nishikawa K, Matsukawa W, Yoshida F, Matsuo S, et al. The kidney disease of crow-fukase (POEMS) syndrome: a clinico-pathological study of four cases. Clin Nephrol. 1991;36:76–82
  11. Arimura K. Increased vascular endothelial growth factor (VEGF) is causative in crow-fukase syndrome. Rinsho Shinkeigaku. 1999;39:84–85
  12. Tokashiki T, Hashiguchi T, Arimura K, Eiraku N, Maruyama I, Osame M. Predictive value of serial platelet count and VEGF determination for the management of DIC in the crow-fukase (POEMS) syndrome. Intern Med. 2003;42:1240–1243
  13. Singh D, Wadhwa J, Kumar L, Raina V, Agarwal A, Kochupillai V. POEMS syndrome: experience with fourteen cases. Leuk Lymphoma. 2003;44:1749–1752

PII: S1201-9712(08)01454-9

doi:10.1016/j.ijid.2008.07.011

International Journal of Infectious Diseases
Volume 13, Issue 3 , Pages e97-e99, May 2009

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