452. ANCA-Associated Vasculitis Presenting with Bilateral Renal Infarction
Creators: name: Alzayer1, Husam
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 38-year-old Caucasian male with a background medical history of congenital adrenal hyperplasia and alpha-1 antitrypsin deficiency ( Pi*Z genotype) presented with recurrent hospital admissions over several months with unexplained adrenal crises. He had brisk resolution of symptoms with intravenous steroids, however, was quick to relapse when transitioned to his maintenance regimen. His inflammatory markers were persistently elevated with no localising infectious aetiology. <strong>Diagnostic Testing: </strong>The peak CRP value was 228 mg/L (0 to 5) with no associated leukocytosis and persistent sterile blood and urine cultures. His creatinine levels were normal and there were no casts on microscopy of urinary sediment. Abdominal CT imaging with contrast revealed wedge-shaped hypoattenuations in both kidneys representing acute renal infarcts (image 1). 24 hr Holter monitor, trans-thoracic and trans-oesophageal echocardiography were unremarkable. A thrombotic screen including anti-phospholipid antibodies, paroxysmal nocturnal haemoglobinuria flowcytometry, myeloma screen, and JAK2 mutation were all reported normal. A positive c-ANCA was identified with associated anti-PR3 17 IU/ml (0-2). A hepatitis viral profile and HIV status were both negative. <strong>Differential &amp; Final Diagnosis: </strong>The differential diagnosis, in this case, included a cardiogenic / atherosclerotic embolus, bilateral renal artery injury, or the presence of a hypercoagulable state. These were extensively investigated with a non-revealing workup. A positive c-ANCA with raised anti-PR3 levels was consistent with a final diagnosis of ANCA-associated vasculitis (AAV). <strong>Discussion of Management: </strong>This case highlights an uncommon presentation of ANCA-associated vasculitis. Alpha-1 antitrypsin deficiency and associated AAV is described in the literature(1). However, the presentation with bilateral renal infarcts as an isolated phenotype is rare. It was particularly challenging to diagnose this in a patient with congenital adrenal hyperplasia who has been receiving multiple courses of high dose intravenous and oral steroids that likely masked this disease for several months and blunted its clinical expression. Renal biopsy was deemed high risk and a clinical consensus on the diagnosis was reached between nephrology, rheumatology, and immunology. The patient eventually was treated with steroids and Rituximab, induction and maintenance, and has achieved clinical and immunological remission. An interval CT abdomen showed a resolution of the cortical infarcts without the need for systemic anticoagulation. <strong>Conclusions: </strong>We describe herein a rare presentation of AAV with bilateral renal infarcts in a patient with alpha-1 antitrypsin deficiency and adrenal insufficiency. This was successfully treated with B-cell depletion without the need for systemic anticoagulation. Vasculitis should be considered in the differential of patients presenting with unexplained organ infarct. <strong>Disclosures: </strong>No disclosures