423. Acute Progression of Granulomatous with Polyangiitis in a Pediatric Patient
Creators: name: Baluta1, Sarah
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Presentation of Case</strong>: A healthy 13-year-old female presented with a 3-week history of polyarthralgia and recurrent epistaxis for acute pharyngitis and fatigue, and diagnosed with streptococcus pharyngitis. A few days later she developed acute onset of hemoptysis, dyspnea, and dehydration. She was started on supplemental oxygen for hypoxemia, and transferred to a nearby pediatric hospital, where she was treated for suspected bacterial PNA. She rapidly decompensated requiring intubation for acute respiratory failure. It became difficult to maintain her oxygenation despite maximum ventilator setting, aggressive pulmonary toileting, and the use of nitrous oxide, and she was transferred to a pediatric center with ECMO compatibility. Her hospitalization involved a prolonged ICU stay complicated by pneumothorax requiring chest tubes, ARDS secondary to diffuse alveolar hemorrhage, coagulopathy with the development of an atrial thrombus and an upper extremity DVT, nephritis, anorexia, and deconditioning secondary to paralytic use. <strong>Diagnostic Testing:</strong> Initial testing was negative for streptococcus, mononucleosis, and COVID tests, and blood work was otherwise unremarkable. Approximately 4 days later, her blood work was notable for anemia with a drop in hgb from 13.2 to 9.8, elevated inflammatory markers with CRP 14.8 and ESR 82, and a respiratory viral panel that returned positive for rhinovirus/enterovirus infection and a chest x-ray showing bibasilar infiltrates. A Urinalysis showed orange-tinged urine with &gt; 180 RBCs and 11 WBC. Repeat chest imaging was significant for dense interstitial and alveolar opacities suggestive of severe edema, diffuse soft tissue emphysema along the bilateral chest consistent with pneumomediastinum and pneumopericardium, and extraparenchymal air at the lung apex representing bilateral pneumothoraces (Figure 1). A TEE showed an atrial thrombus. Rheumatological workup revealed positive ANCA with cANCA level to 1:640 and PR3 &gt;150. <strong>Differentials &amp; Final Diagnosis:</strong> Initial differentials included infectious etiologies secondary to a viral respiratory infection and superimposed pneumonia. However, the constellation of symptoms including diffuse alveolar hemorrhage, glomerulonephritis, +ANCA, and +PR3 met criteria for the diagnosis of granulomatous with polyangiitis vasculitis. Discussion of Management: Treatment included pulse dose solumedrol 1g IV for 5 days, PLEX for 3 doses, 1 dose of Cyclophosphamide, and Rituximab induction with 4 weekly doses based on the RAVE trial<sup>2</sup>. She responded well to therapy, extubated on hospital day 15, and continued on steroid dose of 2mg/kg/day IV. Despite aggressive immunosuppressive therapy, systemic inflammation continued with worsening renal disease progressing to nephrotic range proteinuria. Renal biopsy performed on day 45 of hospitalization was significant for elements of a FSGS with active inflammation prompting treatment with an additional pulse dose of Solumedrol 1g. She was discharged to home on day 49, on a 6-month oral steroid wean and maintenance rituximab. <strong>Conclusion:</strong> The triad of upper and lower respiratory tract inflammation and renal disease is characteristic of childhood GPA<sup>1</sup>. Symptoms are commonly indolent and can mimic other disease processes. The average time of diagnosis of GPA vasculitis from symptom onset is approximately 2 months<sup>1</sup>. This case demonstrates a patient with 3 weeks of symptoms prior to presentation of GPA vasculitis with rapid progression to multiorgan system failure. It is important to recognize symptoms early to improve outcomes and minimize morbidity in patients with GPA vasculitis<sup>1</sup>. <strong>Disclosures:</strong> None