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
Abstract
Introduction
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.
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].
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.
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.
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 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.
Literature Review
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].
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 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.
References
Contact
Douglas M. Hildrew, MD
Tulane University School of Medicine
Email: [email protected]
Phone: 504-988-5454
Poster Design & Printing by Genigraphics® - 800.790.4001
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