Gastrointestinal symptoms as first remarkable signs of ANCA-associated granulomatosis with polyangiitis: a case report and reviews

Abstract

Background: Systemic vasculitis associated with antineutrophil cytoplasmic autoantibodies (ANCA) have an extremely wide variety of symptoms, therefore the fast and proper diagnosis is difficult to establish even for experienced physicians. Gastrointestinal manifestations in ANCA-associated granulomatosis with polyangiitis (GPA) may be present, however, severe, life-threatening complications (such as perforations) are rare.

Case presentation: A case of an 18-year-old male patient is presented, where gastrointestinal symptoms (abdominal pain, vomiting, diarrhoea) were the first remarkable signs of GPA. The initial diagnosis of inflammatory bowel disease delayed the administration of proper immunosuppressive therapy, which might have contributed to the rare and life-threatening complication of arterial duodenal bleeding with perforation. Our systematic review of the literature found only a few case reports where gastrointestinal symptoms were the first signs of GPA, however, this entity might be more frequent if physicians would think of this possibility more often.

Conclusions: Gastrointestinal bleeding is a rare but potential lethal complication of vasculitis. Consequently, we recommend investigating the patients diagnosed with GPA for gastrointestinal bleeding during the treatment.

Keywords: Antineutrophil cytoplasmic antibodies; Case report; Gastrointestinal haemorrhage; Granulomatosis with polyangiitis; Systemic vasculitis.

Fig. 1

Signs of small-vessel vasculitis and crescentic glomerulonephritis in the patient’s kidney biopsy sample. Inflammation of a small arteriole close to the renal capsule with fibrinoid necrosis. Magnification: × 200, haematoxylin–eosin (a). Two affected glomeruli with fibrinoid necrosis and crescent formation. Magnification: × 200, Jones’ stain (b). Crescent formation in a glomerulus. Magnification: × 600, Jones’ stain (c)

Fig. 2

Contrast radiography of the upper gastrointestinal tract 3 days after the surgery. Early contrast radiography of the stomach (a). Late contrast radiography with contrast agent diffusion next to the clipped duodenum (b)

Fig. 3

Summary of the main aspects of the reported case. The upper chart shows the serum creatinine levels (μmol/L) during the observation of the patient, while haemoglobin levels (g/L) are presented in the lower chart. The symptoms, diagnostic tests and treatment are summarized in chronologic order. ANCA antineutrophil cytoplasmic autoantibodies, BUN blood urea nitrogen, CT computer tomography, HRCT high resolution CT, PPI proton-pump inhibitor, IV intravenous