Abstract
Epistaxis is a common problem in children that typically is not severe and seldom requires hospitalization. The nose is a highly vascular structure with a large surface area; subsequently, it is highly predisposed to bleeding. Childhood vasculitides are very rare and are commonly diagnosed by characteristic lesions on imaging studies along with syndrome recognition by clinicians. We present a case of recurrent epistaxis that persisted over 3 months due to Wegener’s granulomatosis in an adolescent that was misdiagnosed as a benign hemorrhage from Kiesselbach’s plexus.
1
Introduction
Epistaxis is a frequent occurrence in children, typically originating in the anterior nose . The nose is a highly vascular structure with one of the most vascularized areas being the Kiesselbach’s plexus, which is an area in the anteroinferior part of the nasal septum, supplied by the sphenopalatine, greater palatine, superior labial, and anterior ethmoid arteries . The close anatomical relationship of the nasal mucosa to the nasal septum provides minimal support or protection for the underlying blood vessels, so any mucosal drying or irritation as well as congestion of the underlying vasculature increases the likelihood of bleeding. The more common causes of epistaxis in children include foreign body, mucosal dryness, trauma, and rhinitis. We present an interesting case where a 14-year-old female present with acute onset hemoptysis after being followed for chronic epistaxis over the previous three months with evaluations by two different otolaryngologists.
2
Case report
A 14-year-old female, with past medical history of obesity and chronic epistaxis for 3 months, presents to her local physician’s office because of sudden onset of small volume hemoptysis that occurred over the previous 2 days. For the past 3 months, she has had epistaxis occurring at 6–7 times daily. She was evaluated by two otolaryngologists in her local area and was diagnosed with a benign nosebleed from Kiesselbach’s plexus due to allergic rhinitis. Nasal irrigation along with nasal corticosteroid spray made no impact upon her clinical course with continuing frequent nosebleeds. Her medical history identified no symptoms of chronic nasal congestion, cough, dyspnea, rhinnorhea, headache, postnasal drip, or facial pressure. With the presentation of acute hemopytsis, pediatric pulmonology was consulted via phone, and a chest x-ray was requested and was done at the local hospital, which identified a cavitary lesion in the left lung, so she was then immediately transferred to the Children’s Hospital for evaluation. Upon arrival, the patient reported 3 months of sporadic epistaxis, followed by the sudden onset of hemoptysis over the last 2 days. She denied cough, dyspnea, fever, chills, chest pain, headache, night sweats, rhinorrhea, and weight loss. There was no change in urinary flow with no other urinary symptoms, including dysuria, frequency, hematuria, hesitancy, or urgency. She denied sexual activity as well as alcohol and other substance abuse. Her physical examination identified normal vital signs, no oral or skin lesions, and normal cardiac and pulmonary examinations. The first chest x-ray ( Fig. 1 ) at Children’s Hospital demonstrated a large left upper lobe lesion with central clearing and multiple nodules in the right lung. A computed tomographic scan of the chest ( Fig. 2 ) illustrates the extensive involvement of the left upper lobe of this cavitary lesion.
The initial laboratory work up identified a normal urinalysis and negative urine pregnancy test. Complete blood count, basic chemistry panel, and liver function tests were normal except hemoglobin level of 9.4 g/dL (reference, 11.0–16.0) and albumin 1.9 g/dL (reference, 2.9–4.2). Reticulocyte count was normal at 1.1%, and the haptoglobin level was elevated at 486 mg/dL (reference, 22–169). Serum creatinine level was normal at 0.97 mg/dL (reference, 0.70–1.20); random urine creatinine and protein and 24-hour urine creatinine were also normal. Total complement level was normal at 108 CAE units (reference, 60–144) as was C4 complement at 27 mg/dL (reference, 7–40), but the C3 complement level was elevated at 207 mg/dL (reference, 77–143). Fungal serology with complement fixation and immunodiffusion tests and urine histoplasmosis and legionella urine antigens were negative. Blood, urine, and sputum cultures were negative. Sputum was sent for bacterial, acid fast bacilli, fungal, and viral cultures, and urine was sent for bacterial and acid fast bacilli cultures. Serum cryptococcal antigen was negative. A purified protein derivative skin test for tuberculosis was negative with positive response to the histamine control.
The serological evaluation looking for an autoimmune process initially identified an elevated Westergren sedimentation rate (WSR) at 157 mm/h (reference range, 0–20), C-reactive protein (CRP) 13.6 mg/dL (reference, 0–0.9), and rheumatoid factor 87 IU/mL (reference, <20). The antinuclear antibody was negative. The perinuclear antineutrophil cytoplasmic antibody (ANCA) titer was negative at <1:20 dilution, but the classic antineutrophil cytoplasmic antibody (C-ANCA) titer was positive at ≥1:80 dilution. Further serological analysis demonstrated no myeloperoxidase (MPO) antibodies; however, there was significant elevation of serine protease 3 (PR3) antibodies at 1319 AU/mL (negative <19, equivocal 20–25). An acute hepatitis panel was also sent that included hepatitis A IgM antibody, Hepatitis B surface antigen and core IgM antibody, and hepatitis C antibody with all being negative. Iron studies obtained were consistent with anemia due to chronic blood loss.
An offered thorascopic biopsy of the mass versus open lung biopsy on the affected side was refused by the parents. With the patient’s history, laboratory evaluation, and clinical findings, the diagnosis of Wegener’s granulomatosis (WG) was made, so the patient was started on combination therapy with cyclosphosamide 50 mg PO daily and prednisone 40 mg PO twice daily. The epistaxis and hemoptysis resolved almost immediately, and the patient was discharged after a 5-day hospital stay. The patient remains asymptomatic at subsequent follow-ups in clinic. Two months after discharge, her chest x-ray demonstrated significant improvement as illustrated in Fig. 3 , which identified a course density with stranding that extended from the left hilum to the pleural surface consistent with residual postinflammatory scarring in the left mid lung. Laboratory studies at this same time demonstrated a reduction in the WSR to 30 mm/h and CRP to <0.5. Nearly 6 months after discharge, the PR3 antibody level decreased to 218 AU/mL, and the MPO level remains negative.
2
Case report
A 14-year-old female, with past medical history of obesity and chronic epistaxis for 3 months, presents to her local physician’s office because of sudden onset of small volume hemoptysis that occurred over the previous 2 days. For the past 3 months, she has had epistaxis occurring at 6–7 times daily. She was evaluated by two otolaryngologists in her local area and was diagnosed with a benign nosebleed from Kiesselbach’s plexus due to allergic rhinitis. Nasal irrigation along with nasal corticosteroid spray made no impact upon her clinical course with continuing frequent nosebleeds. Her medical history identified no symptoms of chronic nasal congestion, cough, dyspnea, rhinnorhea, headache, postnasal drip, or facial pressure. With the presentation of acute hemopytsis, pediatric pulmonology was consulted via phone, and a chest x-ray was requested and was done at the local hospital, which identified a cavitary lesion in the left lung, so she was then immediately transferred to the Children’s Hospital for evaluation. Upon arrival, the patient reported 3 months of sporadic epistaxis, followed by the sudden onset of hemoptysis over the last 2 days. She denied cough, dyspnea, fever, chills, chest pain, headache, night sweats, rhinorrhea, and weight loss. There was no change in urinary flow with no other urinary symptoms, including dysuria, frequency, hematuria, hesitancy, or urgency. She denied sexual activity as well as alcohol and other substance abuse. Her physical examination identified normal vital signs, no oral or skin lesions, and normal cardiac and pulmonary examinations. The first chest x-ray ( Fig. 1 ) at Children’s Hospital demonstrated a large left upper lobe lesion with central clearing and multiple nodules in the right lung. A computed tomographic scan of the chest ( Fig. 2 ) illustrates the extensive involvement of the left upper lobe of this cavitary lesion.
