291. Case report of acute digital ischaemia in 12-year-old boy with Granulomatosis with Polyangiitis
Creators: name: Gallagher1, Kathy
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Background:</strong> Granulomatosis with polyangiitis (GPA) is a rare form of vasculitis involving medium and small arteries, affecting predominantly the upper and lower respiratory tracts, often associated with glomerulonephritis. GPA is typically characterised by necrotising granulomatous inflammation in the presence of antineutrophil cytoplasmic antibodies (ANCA). Objectives: To report an extremely rare presentation of GPA in a child with acute digital ischemia. <strong>Methods:</strong> This 12 year old boy, who has a background of poorly controlled type 1 diabetes and autoimmune hypothyroidism, initially presented to hospital very unwell with diabetic ketoacidosis. Treatment was initiated immediately with good response. On further questioning, he was found to have significant weight loss, chronic cough and hearing loss over the past 3 months. On examination, he was very pale and cachexic. He had a productive cough with reduced air entry and crackles on the right side. There were concerns about hypertonia and clonus in his lower limbs. Initial blood tests showed microcytic hypochromic anaemia (Hb 82g/L), normal white cell count, thrombocytosis and raised inflammatory markers (CRP 138mg/L and ESR 68 mm/hr). His chest x-ray showed enlargement of the right hilum with consolidation/ atelectasis extending into the middle and lower lobes. MRI scans of head and spine were normal apart from fluid and opacification in all the paranasal sinuses. He was extensively screened for infections including Tuberculosis and was started on intravenous antibiotics. On day 13 of admission, he developed painful bluish discolouration of left hand, particularly his thumb, index and middle fingers. His left radial and brachial pulses were not palpable. He was commenced on heparin infusion. The Doppler scan showed occlusion of both radial and ulnar arteries proximal to the wrist with no clear thrombus visualised. He subsequently had a CT thoracic aorta with contrast which showed proximal left radial artery occlusion and distal left ulnar artery occlusion with no clear evidence of proximal embolic source or evidence of vasculitis. It also showed multiple perihilar masses (lymph nodes) in the right lung and peripheral parenchymal masses in both lungs, suggestive of atypical infection or connective tissue disease. Blood tests continued to show raised inflammatory markers (CRP 107mg/L, ESR 86 mm/hr and platelets 658 10<sup>9</sup>/L). The autoantibody profile showed positive ANCA with strongly positive anti PR3 titres (&gt;100 U/mL); rest of the autoantibodies, including ANA, ds DNA and anti-phospholipid antibodies, were negative. He further developed similar ischaemic changes in his right foot, especially the right great toe, with a weakly palpable dorsalis pedis pulse. Doppler scan revealed occlusion / narrowing of the posterior tibial artery 6cm proximal to the ankle. Following advice from the vascular team, he was started on ilioprost infusion to aid re-perfusion of the extremities involved, with good results. Based on clinical and laboratory features of systemic inflammation, evidence of upper airway involvement (bilateral conductive hearing loss and sinusitis noted on MRI scan), parenchymal lesions on CT scan of the chest and strong PR3 positivity, a diagnosis of GPA was made. <strong>Results:</strong> Our patient responded well to therapy including multiple pulses of high dose methylprednisolone and cyclophosphamide, with improvement of all organs involved and no further digital ischemia. <strong>Conclusion:</strong> Although GPA is very rare in children, it is associated with high morbidity and mortality. Many studies show that the spectrum of paediatric GPA is not vastly different from adults, except for higher gender bias towards female, more constitutional and musculoskeletal symptoms, and higher risk of subglottic stenosis. Although there are a handful of case reports of digital ischaemia in adults with GPA, to our knowledge this is the first case report of acute digital ischaemia in paediatric GPA. Early diagnosis and prompt treatment with a multidisciplinary team approach is paramount for good outcome. <strong>Disclosures:</strong> None