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 . 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 .

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 . 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 . 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 . 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 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.