Acute PR3-ANCA vasculitis in an asymptomatic COVID-19 teenager

Abstract

We present the case of an acute onset ANCA positive vasculitis in an asymptomatic COVID-19 infected teenager, resulting in significant colonic damage. The patient was initially diagnosed with Henoch-Schönlein purpura and presented with worsening symptoms with significant necrosis of her perineum and rectum requiring surgical debridement and diverting colostomy. As a part of her work-up, she tested positive for COVID-19 total IgG/IgM antibodies and ANCA antibodies. This case complements previously reported cases of COVID-19 induced autoimmune disease in children but is novel in describing extensive intestinal disease as a result of an autoimmune vasculitis in a child.

Keywords: ANA, anti-nuclear antibody; ANCA; ANCA, antineutrophil cytoplasmic antibody; CMV, Cytomegalovirus; COVID-19; COVID-19, coronavirus disease 2019; EBV, Epstein-Barr virus; HSP, Henoch Schönlein Purpura; Henoch Schonlein purpura; IgG, immunoglobulin G; MIS-C, multisystem inflammatory syndrome in children; NSAIDs, Non-steroidal anti-inflammatory drugs; Vasculitis; anti-MPO, myeloperoxidase antibody; anti-PR3, proteinase 3 antibody.

Fig. 1

Necrotic lesion surrounding anus extending up into the coccyx and down to the labia (left). Extensive purpuric lesions of lower extremities (right).

Fig. 2

MRI pelvis with contrast revealing marked bowel wall thickening of the sigmoid and rectum with surrounding edema and moderate volume ascites. Inferior anal sphincter fistula at the 6 o'clock position communicating with probable subcutaneous superior abscess at the 1 o'clock position (indicated by white arrow). Substantial subcutaneous edema with likely small abscesses from inferior and sphincter fistula to the right and left gluteal clefts.

Fig. 3

H&E staining of colonic subserosal tissue demonstrating a leukocytoclastic small vessel vasculitis (capillaritis) with sparing of larger vessels (left-100x, right-200x magnification).