A 15-year-old woman with a history of allergic rhinitis and mild intermittent asthma was referred to the University of Oklahoma Children's Hospital Emergency Department after having a CT chest demonstrating diffuse bilateral cavitary lesions Figure 1A. Upon further history, 6 months ago, she developed congestion and epistaxis presumed to be sinusitis after a 2-week camping trip in rural southwestern United States. Family history was significant for a maternal grandmother with systemic lupus erythematosus. She was managed conservatively initially with antihistamines and supportive care. But, after 8 weeks, she continued to progress and developed worsening cough and dyspnea despite the typical regimen for her asthma. Additionally, she further developed intermittent fevers, migratory arthralgias, and right acute otitis media (AOM). She completed a course of antibiotics, but continued to have symptoms and further developed night sweats, weight loss, and small volume hemoptysis. She was treated with further courses of antibiotics without improvement. Her fatigue and malaise progressed and she was unable to go to school or even participate in most activities of daily living. It was at this point that she went to a local emergency room and underwent the aforementioned CT scan.
Upon arrival to OU Children's, her vital signs were significant for ongoing pyrexia with a temperature of 38.6  C, mild tachycardia (122 beats per minute), and tachypnea (respiratory rate of 26 per minute) necessitating 2 L of supplemental O2 for increased work of breathing having SaO2 readings of within physiologic range. Physical exam was significant for boggy turbinate's, dried blood in nasal passages, and cobble stoning of the posterior oropharynx. Pulmonary exam revealed coarse breath sounds diffusely without egophony, persistent coughing with respiratory distress. The abdominal exam was benign. Musculoskeletal exam was significant for tenderness to palpation of the hips and knees without evidence of synovitis. She had been exposed to COVID, but never had a positive test herself, and denied any tick bites during her recent camping trip. There were no known exposures to mycobacterium tuberculosis (TB), but it was noted she had been exposed to a household mold.
Initial laboratory studies revealed a leukocytosis of 17.3 k/mm3 with a granulocytic predominance, thrombocytosis of 698 k/mm3, and anemia with a hemoglobin of 8.3 g/dL and hematocrit of 26.7%. Furthermore, she had a sedimentation rate elevated to 119 mm/hr, C-reactive protein of 197 mg/L, ferritin of 687 ng/mL, and D-dimer of 2,184 ng/mL. Electrolyte panel was unremarkable and venous blood gas testing was unrevealing. Urinalysis had a spec gravity of 1.044 in addition to 2+ protein, 1+ ketones, 2+ blood, 5-10 white blood cell (WBC), 50-100 red blood cell (RBC), and an elevated urine microalbumin/creatinine ratio of 40. A respiratory PCR20 (polymerase chain reaction) including severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) via nasopharyngeal swab was negative. Further CT imaging was significant for marked sinusitis with partial absence of the medial walls of both maxillary sinuses (Figure 1B) and plain chest radiography (CXR) (Figure 1C) was consistent with the initial CT for cavitary lesions. Given her presentation, our specialists in rheumatology and infectious disease were involved. They recommended further testing including cardiac enzymes, ANCAs, and a sputum sample with initial high suspicion for vasculitis vs. a fungal infection. Troponin T and brain natriuretic peptide (BNP) were both elevated prompting an echocardiogram which showed right coronary dilation Figure 2A,2B with noted right coronary dilation measuring 3.9 mm described as ectatic and diffusely dilated.
ANCA panel was obtained and positive at 1:80; however interestingly, both p-ANCA and c-ANCA negative. Sputum samples were negative; however, given a recent camping trip and mold exposure there was concern for possible fungal infection. As such, further fungal testing was done which was negative. All mycobacterium TB testing was negative. Bronchioalveolar lavage revealed no significant findings and cultures remained negative for bacteria including additional PCR testing for mycobacterium, but a small colony of Acremonium species growth was noted. Blood cultures remained negative, all drawn during acute febrile events. SARS-CoV-2 antibody titers were obtained and negative. Otorhinolaryngology was consulted and completed a bedside scope of the sinus cavity consistent with our CT findings demonstrating the partial absence of the medial walls of both maxillary sinuses and right middle turbinate with biopsies obtained negative for vasculitis and fungal etiologies, but positive for pseudomonas.
She was treated with a variety of antimicrobials during her stay including antibiotics and antifungals without complete resolution of symptoms. There was gradual improvement of lung cavitation's and edema with therapy on chest CT; however, with increasing C-reactive protein (CRP) levels. Additionally, she was given intravenous immune globulin (IVIG) given the significant concern for vasculitis. Due to a lack of improvement further investigation with endobronchial ultrasound (EBUS) was done and lymph node biopsy was negative. Due to the technical difficulty of EBUS, the pulmonologist was unable to obtain a lung biopsy, a recognized diagnostic gold standard, thus further complicating diagnosis. With ongoing rises in Troponin-T and BNP cardiology suspected coronary dilation attributable to vasculitic changes, though uncommon, due to rising suspicion on our differential for GPA compared to a sole infectious etiology and no other current explainable cause for ongoing dilation with repeat echocardiography revealing the right coronary artery size increasing to 4.4 mm from 3.9 mm in diameter and recommended daily aspirin therapy with plans for outpatient follow-up.
Her symptoms of hemoptysis progressed and she was transferred to the ICU. As her infectious labs were all negative and she continued to have progressive symptoms, she was started on intravenous glucocorticoids. After initiation, she began to show improvement and was weaned off supplemental oxygen in addition to remaining afebrile. Given her improvement on steroids, all antibiotics were stopped and she continued to have improvement. Finally, our specialty lab testing for PR3-ANCA returned elevated confirming the GPA diagnosis. She started on induction cyclophosphamide and was safe for discharge with Pediatric Rheumatology follow-up.
All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient's parent for publication of this case report and accompanying images which are also declassified from patient identifiers. A copy of the written consent is available for review by the editorial office of this journal.