Intractable epistaxis and systemic lupus: High-dose intravenous pulse steroids

Intractable epistaxis and systemic lupus: High-dose intravenous pulse steroids

AM ER IC AN JOUR NA L OF OTOLARY NG OLOG Y –H EA D A N D N E CK ME D I CI NE AN D SUR G E RY 3 5 ( 2 0 14 ) 23 6–2 3 8 Available online at www.scienc...

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AM ER IC AN JOUR NA L OF OTOLARY NG OLOG Y –H EA D A N D N E CK ME D I CI NE AN D SUR G E RY 3 5 ( 2 0 14 ) 23 6–2 3 8

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Intractable epistaxis and systemic lupus: High-dose intravenous pulse steroids☆ Emily A. Waselchuk, BS, Douglas M. Hildrew, MD⁎, Ryan D. Winters, MD, Michael S. Ellis, MD, FACS Department of Otolaryngology/Head and Neck Surgery, Tulane University School of Medicine, New Orleans, LA, USA

ARTI CLE I NFO

A BS TRACT

Article history:

Importance: Epistaxis is most commonly an easily treated ENT entity with a relatively

Received 21 November 2013

simple algorithm. Occasionally, however, it is encountered as a devastating disease process that can humble the otolaryngologist. In the setting of comorbidities that exacerbate bleeding, in this case vasculitis due to systemic lupus erythematosus (SLE), epistaxis can be life-threatening and refractory to conservative management. Observations: This case report describes the hospital course of a patient with severe SLE and intractable epistaxis. We discuss classic management options for epistaxis and offer a novel treatment option for patients with SLE-related vasculitides—goal-directed medical therapy with high-dose intravenous pulse steroid therapy. Conclusions and Relevance: To our knowledge, this report not only is the first description of targeted treatment options for intractable epistaxis in patients with SLE, but also serves to augment the traditional algorithm with the addition of a goal-directed medical therapy— control of epistaxis through high-dose intravenous pulse steroid therapy. We demonstrated that 6 mg of intravenous dexamethasone given every 6 hours can be highly effective in controlling epistaxis in patients with uncontrolled SLE. The presumed mechanism is through control of associated vasculitides. © 2014 Elsevier Inc. All rights reserved.

1.

Introduction

Epistaxis is common and generally self-limited, but can quickly become an otolaryngic emergency. The etiology of epistaxis is vast and includes causes from digital manipulation, inflammation and neoplasia to coagulopathies and systemic disease [1]. Intractable epistaxis can become life-threatening, as hypotension and hypoxia can easily occur. Although 60% of people may experience an episode of epistaxis in their lifetime, only 6% of cases will require medical intervention [2]. Conservative treatment options include nasal vasoconstrictors and/or external pressure, anterior and/or posterior nasal

packing, and electrocautery. Treatment of epistaxis refractory to conservative management includes arterial ligation and/or embolization of the maxillary, sphenoidal, and/or external carotid arteries [2]. Because severe epistaxis can cause devastating sequelae, it is important to identify the etiology and source of bleeding in order to construct a customized treatment plan for each patient. Intractable epistaxis is defined as bleeding of unidentified origin and bleeding not controlled by at least one trial of standard nasal packing [3]. These bleeds can be even harder to manage in the setting of potentiating comorbidities, such as those encountered with iatrogenic, idiopathic, acquired, and/



Original clinical research: collected from patient data at the Tulane University Hospital and Clinics. ⁎ Corresponding author. Department of Otolaryngology/Head and Neck Surgery, Tulane University School of Medicine, 1430 Tulane Avenue, SL-59, New Orleans, LA 70112-2699, USA. Tel.: + 1 504 988 5454; fax: +1 504 988 7846. E-mail address: [email protected] (D.M. Hildrew). 0196-0709/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjoto.2013.12.003

AM ER IC AN JOUR NA L OF OTOLARY NG OLOG Y –H EA D A N D N E CK ME D I CI N E AN D SUR G E RY 3 5 ( 2 0 14 ) 23 6–2 3 8

or genetic coagulopathies. Specifically, those with chronic inflammatory disorders like systemic lupus erythematosus represent an understudied population at high-risk of intractable epistaxis. Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disorder, capable of affecting almost any organ in the body. Bleeding is relatively common in patients with lupus, as autoantibodies and immune complexes formed can deplete platelets, initiate peripheral hemolysis of red blood cells and cause potentiating inflammation. Acute lupus vasculitis is classically characterized by an involvement of small vessels with foci consisting of mononuclear cells, perivascular infiltrates and fibrinoid deposits [4]. Acquired coagulopathies are also common as patients can develop antiprothrombin antibodies and lupus anticoagulant autoantibodies. Despite the significantly elevated bleeding diathesis, cases of severe and intractable mucosal bleeding in patients with SLE are rarely described. Contemporary management options exist only in the context of gastroenterology, with no mention found within the otolaryngic literature. This case will describe a female patient with a history of SLE who developed epistaxis refractory to both conservative and invasive treatment, ultimately cured using a short course of high-dose intravenous pulse steroid therapy.

2.

Case report

A 45-year-old African American female with a history of frequent hospitalizations due to the multi-system complications of poorly controlled systemic lupus erythematosus (SLE), rheumatoid arthritis and anemia, presented to the emergency department with a 24-hour history of epistaxis. A 5.5-cm anterior nasal balloon was initially placed, and the patient was discharged home in stable condition. She returned within 24 hours with persistent bleeding and emesis of approximately 2 L of blood with mixed food and gastric secretions. On physical exam she had no anterior drainage of blood from the nares, but oropharyngeal examination showed large clots of dried blood with a slow trickle of blood passing through the postcricoid pharynx. Initial vital signs revealed a normotensive, yet tachycardic status. Laboratory work was as follows: white blood cells of 5900/μL, hemoglobin of 7.8 g/dl, hematocrit of 23.5%, platelet count of 154,000/μL, prothrombin time (PT) of 12.9 seconds, activated partial thromboplastin time (PTT) of 28.4 seconds and an international normalized ratio (INR) of 1.3. Both nares were packed with 7.5-cm anterior/posterior balloons and the patient was given scheduled nasal oxymetazoline for vasoconstriction. A bilateral ooze of blood continued despite conservative measures. Bilateral anterior/posterior nasal packing via foley catheter and iodoform gauze was planned, but consent could not be obtained due to the patient's fear of further nasal discomfort. Hematology was therefore consulted for expert non-surgical opinion, but no other bleeding diathesis could be found for treatment. Laboratory values revealed no anomalies in factor VIII levels, von Wilebrand factor activity, platelet function assays, and/or disseminated intravascular coagulation (DIC) panels. Routine complete counts were only significant for low

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hemoglobin levels that remained below 8.0 g/dl; platelets never became depleted. As a non-invasive therapy known to promote clotting, the antifibrinolytic drug tranexamic acid was employed. On hospital day 3, epistaxis remained unabated and the patient elected for intra-arterial embolization. Interventional radiologists were able to embolize bilateral internal maxillary, right facial, and the left sphenopalatine arteries. Despite embolization and continuation of tranexamic acid, the patient's bilateral nares continued to ooze blood. Blood loss through hospital day 6 continued, correlating with a hemoglobin of 6.9 g/dl and 4 units of packed red blood cells was transfused. An external approach for surgical ligation of remaining ethmoidal and sphenoidal arteries was discussed, but consent could not be obtained due to cosmetic concerns. The patient ultimately elected to undergo general anesthesia for nasal endoscopy and suction electrocautery on hospital day 6. Intraoperative findings were significant for generalized ooze from multiple mucosal surfaces, addressed with suction electrocautery. Postoperative hemostasis was achieved only for a few hours, and 7.5-cm anterior/posterior balloons were once again employed. As all available treatment options had either failed or been refused, a thorough literature review was undertaken. Although no compelling contemporary otolaryngic solutions were identified, there had been recent evidence from gastroenterologists describing success with high-dose intravenous pulse steroid treatment in gaining control of lupus-related vasculitis in the bowel. On hospital day 7, the patient was started on 6 mg of intravenous dexamethasone given every 6 hours. Within 12 hours, bleeding was markedly diminished in the left and was completely absent in the right nasal cavity. All packing was removed from the right naris on hospital day 7. The 7.5-cm anterior/posterior balloon located in the left nasal cavity was deflated 48 hours later, and ultimately removed on hospital day 11. Steroids were stopped on hospital day 12, and the patient was discharged on hospital day 13 with complete resolution of bleeding and a stable hemoglobin of 9.5 g/dl.

3.

Discussion

The most important aspect of managing epistaxis is identification of the bleeding source and control of exacerbating factors, in this case vasculitis secondary to systemic lupus erythematosus (SLE). Classic methods of initially controlling epistaxis include external pressure, vasoconstrictors, anterior and/or posterior nasal packing, and/or topical cautery/electrocautery. Hemostasis can be achieved in refractory epistaxis using intra-arterial embolization, direct ligation via endoscopic or external approach, or direct cautery of the offending arteries. Studies have shown nasal endoscopy with direct mucosal electrocautery as an effective way to achieve hemostasis in intractable epistaxis, and have advocated its use as a first-line therapy after failure of nasal packing [3]. Historically, the next surgical step in the treatment algorithm has been direct surgical ligation of the offending vessels via Lynch incision. An endoscopic approach for surgical ligation, although not available at all institutions, allows for better visualization and

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AM ER IC AN JOUR NA L OF OTOLARY NG OLOG Y –H EA D A N D N E CK ME D I CI NE AN D SUR G E RY 3 5 ( 2 0 14 ) 23 6–2 3 8

identification of the bleeding source [5]. It has the benefit of being a definitive surgical treatment, but spares the patient the cosmetic worries from the Lynch incision [5]. Compared to direct arterial ligation, intra-arterial embolization is a less invasive procedure, yields similar success rates, and also obviates the need for facial incisions [6]. Early surgical intervention may be especially important in high-risk patients, such as those with coagulopathies or vasculitis, in an attempt to avoid exsanguination and resultant blood transfusions [5]. Despite multiple treatment methods for uncomplicated intractable epistaxis, there is a paucity of research on treating epistaxis associated with lupus-related vasculitides. Data for treating hemorrhage in SLE are limited only to the management of lupus enteritis. Gastrointestinal literature suggests that bleeding caused by lupus enteritis is best treated with high-dose intravenous pulse steroid treatments. Tan et al. [7] report success in obtaining control of an extensive bowel hemorrhage using 500 mg of intravenous methylprednisolone daily for 3 days. Additionally, Hiraishi et al. [8] describe a case of massive gastrointestinal bleeding in a 47-year-old female with lupus enteritis who recovered rapidly after administration of 1500– 2000 mg methylpredisolone daily for 5 days. Both investigators emphasize the efficacy of steroid pulse therapy in rapid resolution of clinical signs and diagnostic studies. Although surgical intervention is a definitive option, highdose intravenous pulse steroid responsiveness indicates that corticosteroids could become a potential first-line treatment for lupus enteritis [9]. Although no current studies exist that prove a similar result for epistaxis associated with lupus vasculitis, empiric evidence and the anatomic and physiologic similarities between the two systems offer a potential corollary; both disease processes involve systems with a central lumen/cavity lined with mucosal cells and robust vascularity, and contain a strong presence of lymphoid tissue. As a result, gastroenterologic research provides a potential foundation on which to base treatment for acute lupus vasculitis causing epistaxis [10]. Despite aggressive measures including medicinal therapy, anterior/posterior packing, electrocautery, and embolization generalized posterior oozing persisted in the above-described patient. Within only 12 hours of starting a 5-day course of 6 mg intravenous dexamethasone, given every 6 hours, hemostasis was achieved.

4.

Conclusion

To our knowledge, this report is not only the first description of targeted treatment options for intractable epistaxis in patients with SLE, but serves to augment the traditional algorithm with

the addition of a goal-directed medical therapy—control of vasculitis through high-dose intravenous pulse steroid therapy. We demonstrated that 6 mg of intravenous dexamethasone given every 6 hours can be highly effective in controlling epistaxis in patient with uncontrolled SLE. The presumed mechanism is through control of associated vasculitides.

Financial disclosure There was no financial or material support for the research of this work. None of the contributing authors or involved parties have any financial interests in any company associated with the products in the body of this work, nor do they have any financial interests in any competitor companies. The authors have no financial affiliations to disclose.

Conflict of Interest None. REFERENCES

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