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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2014  |  Volume : 25  |  Issue : 1  |  Page : 117-120
Recurrent epistaxis revealing a non-catheter-related superior vena cava syndrome in a hemodialysis patient: Unmasking undifferentiated connective tissue disease

1 Department of Nephrology and Dialysis, Louis Jaillon General Hospital, Saint Claude, France
2 Department of Secondary Care, Louis Jaillon General Hospital, Saint Claude, France
3 Department of Radiology, Louis Jaillon General Hospital, Saint Claude, France
4 Department of Emergency and Trauma, Louis Jaillon General Hospital, Saint Claude, France
5 Department of Internal Medicine, Louis Jaillon General Hospital, Saint Claude, France

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Date of Web Publication7-Jan-2014


We report a case of an 80-year-old Caucasian woman on maintenance hemodialysis for almost three years through a right-tunneled jugular catheter. She presented with recurrent epistaxis for which she was periodically blood transfused despite erythropoietin therapy. She continued manifesting epistaxis, which was progressively emerging as a sign related to superior vena cava syndrome due to mediastinal mass. Laboratory investigations revealed active immunological abnormalities thereafter. Malignant superior vena syndrome remains an uncommon com­plication in this population related to a history of or ongoing central vein catheterization. Pro­longed oozing from the vascular site was the first alerting sign of the existence of this syndrome. We conclude that sometimes the transformation of undifferentiated connective tissue disease in the presence of epidermoid carcinoma of the superior mediastinum may be revealed during the use of catheters in dialysis.

How to cite this article:
Dahmani O, Demarchi P, Sophoclis C, Abi-ayad K, Belkhalfa S, Djellid J. Recurrent epistaxis revealing a non-catheter-related superior vena cava syndrome in a hemodialysis patient: Unmasking undifferentiated connective tissue disease. Saudi J Kidney Dis Transpl 2014;25:117-20

How to cite this URL:
Dahmani O, Demarchi P, Sophoclis C, Abi-ayad K, Belkhalfa S, Djellid J. Recurrent epistaxis revealing a non-catheter-related superior vena cava syndrome in a hemodialysis patient: Unmasking undifferentiated connective tissue disease. Saudi J Kidney Dis Transpl [serial online] 2014 [cited 2020 Oct 30];25:117-20. Available from: https://www.sjkdt.org/text.asp?2014/25/1/117/124522

   Introduction Top

The diagnosis of superior vena cava syn­drome (SVC) includes a partially or totally interrupted blood return at any level of related effluent venous tree. [1] Signs and symptoms re­lated to this syndrome arise either from the intravascular obstruction due to thrombosis or long period indwelling venous catheters or from the extravascular compression due to tumors or connective tissue disease. [2],[3],[4],[5] In some circumstances, both mechanisms are involved, leading to increased morbidity and mortality. [6],[7],[8],[9] Past medical history of central vein cannulation, whether inserted for short or long period, has been reported as a predisposing risk factor. [1],[8],[9] In the hemodialysis population, patients are prone to undergo such aggressive proce­dures during their life of being dialyzed. The likelihood of undergoing a central indwelling catheterization increases with the presence or absence of permanent patent vascular access for hemodialysis, being diagnosed as having acute or chronic renal failure. In the last two decades, there has been great interest for tun­neled indwelling catheter, especially in the elderly, leading to some reports of SVC.

Herein, we present a case of SVC with an unusual clinical presentation marked by recur­rent epistaxis inaugurating the syndrome that becomes evident once venous distension with collateral circulation and face swelling com­pleted the presentation with time.

   Case Report Top

An 80-year-old Caucasian woman with end-stage renal disease (ESRD) due biopsy-proven endo- and extra-capillary glomerulonephritis performed in September 2005 was started on maintenance hemodialysis three times weekly in December 2005. She was initially put on regular hemodialysis due to sustained anuria and the presence of fluid overload in a teaching hospital, after which she was trans­ferred to our hospital. The sessions of hemodialysis were performed initially through a right femoral catheter until her left brachiocephalic arteriovenous fistula (AVF) became mature and easily cannulated. Her family and personal medical history were unremarkable apart from long-standing high blood pressure. Her surgical history included appendectomy, total hysterectomy, intestinal occlusion and fractures of the right lower limb and right humerus that required internal fixation. Her regular hemodialysis was uneventful and she received intravenous iron and erythropoietin therapy weekly guided by biologic parameter targeting a hemoglobin level of more than 12 g/dL and transferrin saturation coefficient bet­ween 30% and 40% or hypochromic red cell less than 2.5%. Her inter-dialysis weight gain had never exceeded 2.5 kg despite remaining anuric. However, the patient started to com­plain of recurrent oozing from her vascular access despite tight low molecular heparin regimen. Recorded visual arterial and venous pressure alarms were -100 mm Hg and +100 mm Hg, respectively. The blood pump rate of 240 mL/min was tolerated by the patient. According to the finding of an anastomotic stenosis by colored Doppler, angioplasty was performed. The procedure was complicated by a huge hematoma and anemia that required blood transfusion. The blood access for the subsequent sessions was ensured through a tunneled right jugular vein indwelling catheter inserted surgically. Her native fistula remained functional despite a compressing hematoma, which was exacerbating the stressed stenosis that was manipulated unsuccessfully.

By March 2007, she started presenting epi­sodic epistaxis attributed initially to seasonal environmental factors and anti-aggregant agents. We detected in her a relative thrombocytopenia at 98,000/mm 3 .

By December 2007, epistaxis re-emerged in a frequency that required blood transfusion, with progressive facial swelling not responding to gradual decrease of her dry weight. Clinical examination revealed a patient with anxiety coexisting during oozing from each nostril alternatively and, in some instances, posterior nasal bleeding packing, prophylactic antibio­tics and moisturizing the nasal mucosa were required for management. Distended neck veins as a result of increased venous pressure were noted. The left upper chest wall showed the presence of moderate collateral vein circu­lation and there was no evidence of acrosyndrome. Physiologic orifices were normal; no masses were felt either at the abdominal cavity or cervical spaces. We did not detect lymphadenopathy or skin changes apart from some traumatic ecchymosis localized on the anterior face of her lower limbs. Numerous scarring and hypopigmented areas of sclerotic fibrotic plaques were observed on the upper limbs with no signs of atrophy or telangiectasia. The fistula was well functioning and a stressed thrill on the top 3 cm post-anastomotic stenosis was auscultated. The right tun­neled jugular indwelling catheter was in place without any complications or exit-site appea­rance modifications. The patient denied having dyspnea, dysphagia, dysphonia or changes in her gastro-intestinal habits apart from poor appetite. Laboratory investigations showed pancytopenia with normocytic normochromic anemia and RDW at 22% and MCV = 90.6 fl, hemoglobin level = 7.8 g/dL, reticulocytes = 86,000/mm 3 and normal CRP despite elevated ESR and D-dimer level = 2852 μg/L (N < 0.5). Prothrombin time was slightly increased and partially activated thromboplastin time was 50/32 s, with positive circulating anticoagu­lants and negative anti-cardiolipin antibodies. Serum protein profile disclosed evidence of subacute inflammatory response with normal C3 level and ferritin and transferrin saturation coefficient. However, the IgM level was ele­vated and the haptoglobin level was slightly reduced [0.49 (0.88-2.35)]. The brain natriuretic peptide was 967 pg/mL and the pre-albumin was 0.3 g/L. Anti-nuclear antibodies (ANA) were positive, having double aspect; anticentromere (>1/1280) and homogenous (1/680) aspects corresponding to CREST or localized sclerosis and active SLE, respec­tively, with anti-DNA antibody highly posi­tive (>250 oms/L) and Farr test = 125 ku/L (N <7) and antidenatured DNA = 31 u/mL, asso­ciated with positive antihistone antibody and negative ANCAs. Indirect Coombs test was positive and anti-ENA phenotypes revealed a weakly positive anti scl-70 (1.3).

Radiological investigations included plain chest radiography, which showed pronounced dorsal discarthrosis and normal cardiac sil­houette with mildly enlarged mediastinum. She had no evidence of nasal septal defect or perforation with normal computed tomography (CT) scan of the sinus cavities and nostril. The thoracic-abdominal-pelvis CT scan detected a tumoral mass of the superior mediastinum 54 mm × 33.5 mm in dimension compressing the innominate trunk and the presence of some lymph nodes at the level of the aorto-pulmonary window at the Barety lodge [Figure 1]. The positron emission tomography scan failed to show any significant hyper fixation.
Figure 1: Un-enhanced thoracic scan showing a soft tissue mass of 54.85 mm × 33.45 mm dimension without any calcifications. This mass was localized in the anterior superior mediastinal area compressing the innominate trunk, responsible for the superior vena cava syndrome.

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The pathological exam of the tumoral tissue specimen extracted by percutaneous CT-guided biopsy revealed a predominant staining pankeratin and cytokeratin 5/6. There were no cells exhibiting cytokeratin 7 and 20 or CD20. This immunohistochemical aspect was suggestive of mildly differentiated epidermoid carcinoma of the mediastinum.

We did respect the patient's decision of con­servative measures regarding the tumor ma­nagement. During the follow-up, she developed an episode of bacteremia due to pneumococcal infection cleared without sequelae after three weeks of antibiotic therapy and removal of foreign bodies. Her native fistula is now used, cannulated with a single needle dialysis under tight heparin regimen, while continuing Sali­cylic acid prophylactic dose on other days of dialysis.

   Discussion Top

Factors contributing to the development of epistaxis are classified as traumatic, local or systemic, environmental or genetic and drug-induced hypocoagulation. [10] Many of the parti­cipating factors associated with the develop­ment of epistaxis are frequently present in patients with ESRD as such in our patient who developed progressive SVC. Characteristic signs and symptoms that alert clinicians to this syndrome include distended neck veins, swol­len face, upper arms and eyelids. The degree of back pressure-related findings are nega­tively correlated to the development of colla­teral veins. In our case, unexplained prolonged oozing from fistula was probably the initial silent sign, followed by epistaxis as a result of back pressure. The picture became complete once collateral veins occurred on the upper left hemithorax, which was followed by a decrease in the frequency of epistaxis and oozing from her native fistula.

SVC is classified as malignant (malignancy related) and benign (non-malignancy related). The former is the most common form, accoun­ting for 80-90% of cases. [10],[11] The latter is observed as a result of fibrosing mediastinitis, granulomatosis, post-radiotherapy and post-indwelling wires or dialysis catheter insertion. Intraluminal clots and trapped dialysis catheter represent other pathophysiological modes ini­tiating the syndrome.

Catheter-related SVC accounts for 70% of benign SVC. [8] Aging patients on long-term hemodialysis are prone to undergo such aggressive procedures even repeatedly due to unavailability of other patent vascular acces­ses. This made them, unfortunately, predis­posed to more cases of SVC. [1] Even though the diagnosis depends on a high degree of alert­ness in a patient with a history of previous central vein cannulation, it can be quite chal­lenging to rule out malignancy-induced SVC. In our case, we had initially suspected the syn­drome to be due to the coexistence of indweling tunneled catheter for almost two years without any dysfunction. Our patient was having concomitant hematological abnormali­ties with normal bone marrow aspiration re­sults, completed by immunological investiga­tions that confirmed an immune dysregulation due to active lupus erythematosus and loca­lized sclerosis. Subsequently, the diagnosis was confirmed by a thoracic CT scan, which showed a compressing mass in the upper mediastinum. On retrospective analysis, the patient had in the past a significant positive antinuclear antibody with normal anti-DNA antibody. This feature was consistent with undifferentiated connective tissue disease. Withdrawal of indwelling materials realized during a pneumococcal pneumonia bacteremia was not followed by any improvement of her signs and symptoms.

The link between cancer and autoimmunity is bidirectional. [2] The high prevalence of auto-antibodies found in aged cancer subjects could be attributed to several cellular and humoral immunological aberrations, confirming the ob­servation that tissue necrosis could be an im­portant factor for the production of antibodies. [3],[5] Thus, para-neoplastic autoimmunity carries a high risk of morbidity and mortality in aging patients, which was not the case in our patient who is still stable without any treatment.

Recurrent epistaxis associated with malignant SVC was the first alarming sign mandating further investigations in this hemodialysis pa­tient. Furthermore, she was found to have mul­tiple precipitating factors, including immune dysregulation.

   References Top

1.Akoglu H, Yilmaz R, Peynircioglu B, et al. A rare complication of hemodialysis catheter: Superior vena cava syndrome. Hemodial Int 2007;11:385-91.  Back to cited text no. 1
2.Elgert KD, Alleva DG, Mullins DW. Tumor- induced immune dysfunction: The macrophage connection. J Leukocyte Biol 1998;64:275- 90.  Back to cited text no. 2
3.Imran A, Neelam F, Tariq M. Incidence of circulating antinuclear antibodies in cancer patients. Indian J Med Sci 2003;57:113-6.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.Krane NK, Burjak K, Archie M, O'donovan R. Persistent lupus activity in end stage renal disease. Am J Kidney Dis 1999;33:872-9.  Back to cited text no. 4
5.Lernmark A. Series introduction: Autoimmune disease: Are markers ready for prediction? J Clin Invest 2001;108:1091-6.  Back to cited text no. 5
6.Pons-Estel GJ, Alarcon GS, McGwin G Jr, et al; Lumina Study Group. Protective effect of Hydroxychloroquine on renal damage in patient with lupus nephritis: LXV Data from A Multiethnic US cohort. Arthritis Rheum 2009;61:830-9.  Back to cited text no. 6
7.Rietveld A, Berden JH. Renal replacement therapy in lupus nephritis. Nephrol Dial Transplant 2008;23:3056-60.  Back to cited text no. 7
8.Rice TW, Rodriguez RM, Light RW. The superior vena cava syndrome: Clinical characteristics and evolving etiology. Medicine (Baltimore) 2006;85: 37-42.  Back to cited text no. 8
9.Schifferdecker B, Shaw JA, Piemonte TC, Einsenhauer AC. Non malignant Superior vena cava Syndrome: Pathophysiology and management. Catheter Cardiovasc Interv 2005;65:414-6.  Back to cited text no. 9
10.Schlosser RJ. Clinical practice. Epistaxis. N Engl J Med 2009;360:784-9.  Back to cited text no. 10
11.Markman M. Diagnosis and management of superior vena cava syndrome Cleve Clin J Med 1999;66:59-61.  Back to cited text no. 11

Correspondence Address:
O Dahmani
Department of Nephrology, Louis Jaillon General Hospital, Saint Claude
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DOI: 10.4103/1319-2442.124522

PMID: 24434394

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