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Intractable Epistaxis and Systemic Lupus Erythematosus:

High-Dose Intravenous Pulse Steroid Therapy

Douglas M. Hildrew, MD; Emily A. Waselchuk, BS; Ryan D. Winters, MD; Michael S. Ellis, MD, FACS

Tulane University School of Medicine

Introduction Case Report Abstract

Objectives:

To describe a novel therapy for the treatment

of intractable and refractory epistaxis in a

subset of patients with poorly controlled

systemic lupus erythematosus. Clinical

presentation, treatments/interventions, and a

discussion of the limited relevant literature is

presented.

Study Design:

Case report and review of the literature

Methods:

A patient's case was reviewed. A MEDLINE

search was performed using the terms:

epistaxis AND steroid AND systemic lupus

erythematosus. All existing literature on the

treatment of vasculitis in relation to systemic

lupus erythematosus was extracted.

Results:

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.

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ｭdirected

medical therapy control of vasculitis through

highｭdose intravenous pulse steroid therapy.

We demonstrated that six milligrams of

intravenous dexamethasone given every six

hours can be highly effective in controlling

epistaxis in patient with uncontrolled SLE.

The presumed mechanism is through control

of associated vasculitides.

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 twenty-four 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 twenty-four hours with persistent bleeding and emesis of

approximately two liters 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. 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. A formal anterior/posterior nasal pack was planned, but refused by the

patient due to fear of further nasal discomfort. Hematology was consulted for expert non-surgical opinion, but no other

bleeding diathesis could be found for treatment. As a non-invasive therapy known to promote clotting, the antifibrinolytic drug

tranexamic acid was employed. On hospital day three, the patient elected for intra-arterial embolization.

Bilateral internal maxillary, right facial, and the left sphenopalatine arteries were embolized, but blood loss continued

throughout hospital day six. Surgical ligation of the remaining ethmoidal and sphenoidal arteries was discussed, but refused

by the patient. Nasal endoscopy and suction electrocautery was attempted on hospital day six and was significant for

generalized ooze from multiple mucosal surfaces. 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 revealed 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 seven, the patient was started on six milligrams of intravenous dexamethasone given

every six hours. Within twelve hours, bleeding was markedly diminished in the left and was completely absent in the right

nasal cavity. All packing was removed by hospital day eleven. Steroids were stopped on hospital day twelve, and the patient

was discharged on hospital day thirteen with complete resolution.

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 are

well known and frequently reviewed, but the proactive management of patients with specific blood dyscrasias is rarely

discussed. Management therefore tends to be reactive in nature, instituted on a case-by-case basis only. By definition, this

strategy condemns the otolaryngologist to always being one step behind in the treatment algorithm.

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 [3, 5, 6]. Despite multiple surgical options for

refractory/intractable epistaxis, effective medical treatment options are few.

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 traditional treatment strategies with the addition of a goal-directed medical therapy – control

of vasculitis through high-dose intravenous pulse steroid therapy. We demonstrated that six milligrams of intravenous

dexamethasone given every six hours can be highly effective in controlling epistaxis in patient with uncontrolled SLE. The

presumed mechanism is through control of associated vasculitides.

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/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.

Douglas M. Hildrew, MD

Tulane University School of Medicine

Email: dhildrew@tulane.edu

Phone: 504-988-5454

Contact

There is a paucity of research on treating epistaxis associated with lupus-related vasculitides, and data for treating

hemorrhage in SLE is limited only to the management of lupus enteritis. Gastrointestinal literature suggests bleeding caused

by lupus enteritis is best treated with high-dose intravenous pulse steroid treatments. Tan et al reports success in obtaining

control of an extensive bowel hemorrhage using 500-mg of intravenous methylprednisolone daily for three days [7].

Additionally, Hiraishi et al describes a case of massive gastrointestinal bleeding in a 47 year-old female with lupus enteritis

who recovered rapidly after administration of 1500-2000-mg methylprednisolone daily for five days [8]. 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, high-dose intravenous pulse steroid responsiveness indicates

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 as well as 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, 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].

Literature Review

1. Pope LE, Hobbs CG. Epistaxis: an update on current management. Postgrad Med J 2005; 81:309-14.

2. Rudmik L, Smith T. Management of intractable spontaneous epistaxis. Am J Rhinol Allergy 2012; 26:55-69.

3. Liu Y, Zheng C, Wei W, et al. Management of intractable epistaxis: endoscopy or nasal packing? The J of Laryngol & Otol 2012; 126: 482-6.

4. Kumar V. Diseases of the immune system. In: Abbas K, Fausto N, Mitchell R, editors. Robbins Basic Pathology, 8th ed. Philadelphia (PA): Saunders; 2007. p. 215-7.

5. Rabelo FA, Prado VB, Valera FC, et al. Surgical treatment of nasal packing refractory epistaxis. Braz J Otorhinolaryngol 2009; 75:335-9.

6. Scaramuzzi N, Walsh RM, Brennan P, et al. Treatment of intractable epistaxis using arterial embolization. Clin Otolaryngol 2001; 26:307-9.

7. Tan T, Wansaicheonh G, Thong B. Acute onset of systemic lupus erythematosus with extensive gastrointestinal and genitourinary involvement. Lupus 2012; 21:1240-1243.

8. Hiraishi H, Konishi T, Ota S, et al. Massive gastrointestinal hemorrhage in systemic lupus erythematosus: successful treatment with corticosteroid pulse therapy. Am J Gastroenterol 1999; 94: 3349-53.

9. Janssens P, Arnaud L, Lionel G, et al. Lupus enteritis: from clinical findings to therapeutic management. Orphanet J of Rare Diseases 2013; 8:1-10 [editorial].

10. Kawashiri S, Nishino A, Sueyoshi E, et al. A patient with systemic lupus erythematosus who developed massive small intestinal hemorrhaging during treatment for chronic lupus peritonitis. Mod Rheumatol 2012; 22:312-5.

References

Discussion