Gastrointestinal Eosinophilic Sclerosing Fibroplasia

Question 1

What is FGESF?

Answer 1

• an inflammatory disorder characterised by solitary or multiple masses comprised of fibroblasts, collagen trabeculae and inflammatory cells (mainly eosinophils)
• masses located primarily in the GIT but can be in associated LNs and other abdominal organs

Question 2

Etiology of FGESF

Answer 2

• unknown
• predisposition following certain antigen exposure? - diet, intestinal microbioat dysbiosis, intracellular bacterial infection, fungal infection, hair or plant ingestion, parasites
• potential ragdoll predisposition

Question 3

Pathophysiology of FGESF

Answer 3

• characterised by solitary or multiple, intraluminal, firm, ulcerated masses most commonly located at the pylorus or ICCJ
• can cause stenosis and GI obstruction

Question 4

Endoscopic abnormalities noted in FGESF

Answer 4

• mucosal proliferation and ulceration, polypoid-like masses may be detected

Question 5

Histopathology findings of FGESF

Answer 5

• dense trabeculae of collagen
• large fibroblasts
• inflammatory cells (eosinophils)

Question 6

Treatment of FGESF

Answer 6

1. Medical; corticosteroids at immunosuppressive doses typically given after surgical resection or primary if sx. not an option (other immunosuppressive medications - cyclosporine)
2. Surgical debulking or resection - lesions can recur after complete resection of develop in different locations

Question 7

Prognosis of FGESF

Answer 7

• good outcomes with complete surgical resection although recurrence does occur