An Unusual Presentation of Granulomatosis with Polyangiitis with Multiorgan Involvement

REPORTantibody (ANA) and antineutrophil cytoplasmic antibody (ANCA) by immunofluorescence were negative.Since ANA and ANCA by immunofluorescence can be negative in the initial stages, a more specific test, namely ANCA by enzyme-linked immunosorbent assay (ELISA), was performed.This was suggestive of PR3-ANCA positivity (53 units), with negative MPO-ANCA, supporting a diagnosis of GPA.She was initiated on IV steroids, bronchodilator nebulization, and supportive care.During hospitalization, she developed multiple episodes of vomiting.Abdominal ultrasound revealed mild hepatomegaly, while CECT of the abdomen showed splenic ischemia, likely secondary to vasculitis. 2 Repeat abdominal imaging demonstrated persistent splenic ischemia. 3,4 A peripheral smear showed neutrophilic leukocytosis with normocytic, normochromic red blood cells (RBCs), thus ruling out leukemia.Given the unusual splenic artery involvement, tests for antiphospholipid antibody (APLA) syndrome (anticardiolipin antibodies, beta-2 glycoprotein, and lupus anticoagulant) were performed (to rule out APLA); the reports were found to be negative.Infectious etiologies were also excluded.