Relief of Suspected IgA Vasculitis-Associated Enterocolitis Manifested as Severe Diarrhea and Hypoalbuminemia Treated With Glucocorticoid Therapy and Administration of Fibrogammin® (Purified Coagulation Factor XIII Concentrate): A Case Report

Abstract

A 62-year-old female was admitted to our hospital with abdominal pain, diarrhea, and bloody stool. She suffered from severe diarrhea 30 times per day and consequently got hypoalbuminemia and hyponatremia. Esophagogastroduodenoscopy and total colonoscopy showed diffuse erosion of the duodenum, terminal ileum, and colorectum. Her endoscopic findings were similar to the former case reports of immunoglobulin A (IgA) vasculitis-associated enterocolitis. Further investigation of biopsy demonstrated that IgA-positive lymphocytes invaded the perivascular of the submucosal layer. However, we did not find IgA deposition in the capillary wall, and no granuloma, basal plasma cytosis, or crypt abscess was detected. Although a definite diagnosis could not be established, we considered the possibility of refractory IgA vasculitis-associated enterocolitis and initiated glucocorticoid therapy and administration of Fibrogammin® (purified coagulation factor XIII concentrate). The patient's diarrhea improved within one and a half months after admission.

Keywords: coagulation factor xiii activity; enterocolitis; fibrogammin; immunoglobulin a vasculitis; intractable diarrhea.

Figure 1. CT on admission

Abdominal CT showing thickened wall of the transverse part of duodenum and proximal jejunum (a, blue arrowheads), ascending colon (b, yellow arrow), transverse colon (c, yellow arrow), and sigmoid colon (d, yellow arrow).

Figure 2. Total colonoscopy on day 14 after admission

(a) The terminal ileum. (b-d) The colon. There was loss of the villi in the terminal ileum. There were small islands of the remaining villi in the terminal ileum (a, yellow arrowheads). There were a couple of round redness similar to hematoma-like protrusion in the colon (b, green arrowheads). In the colon, there were small islands, which looked alike shapes in the terminal ileum (c, d, blue arrowheads).

Figure 3. Esophagogastroduodenoscopy on day 22 after admission

(a) The stomach. (b-d) The descending part of the duodenum. There was mucosal erythema in the stomach. There were small islands of the remaining villi in the duodenum (b, yellow arrowheads). Those islands were more clearly observed with narrow band imaging (d) than with white light (c).

Figure 4. Specimens of mucosal biopsy with hematoxylin and eosin stain

(a, b) Specimens of the terminal ileum. (c, d) Specimens of the colon. (e, f) Specimens of the duodenum. Photomicrograph of biopsy from the ileal, colonic, and duodenal lesions showed inflammatory cell infiltrated submucosa, red blood cells leaked around vessels, and leukocytoclastic vasculitis with nuclear dusts (a, c, e). There were IgA-positive lymphocytes gathering around the blood vessels in the submucosa of the ileum and duodenum (b, d, f).

Figure 5. Clinical course after hospitalization

Figure 6. Endoscopic findings of total colonoscopy on day 35 after admission

(a) Terminal ileum. (b) The cecum. (c. d) The colon. Findings showed that the area of the ulcer had reduced, and some areas of the colorectum were healed moderately.

Figure 7. Endoscopic findings of esophagogastroduodenoscopy on day 49 after admission

(a) The Stomach. (b-d) The duodenum. Findings demonstrated the disappearance of erythema and regeneration of the duodenal villi.