12th Ljudevit Jurak
International Symposium on
June 1-2, 2001
|VENULITIS RESULTING IN ACUTE ABDOMEN AND UNTREATABLE
INTESTINAL HEMORRHAGE AS FIRST SIGN OF A SYSTEMIC LUPUS ERYTHEMATOSUS
|R. Bussani, P. Cusati, D. Camilot,
C. Trevisiol*, D. Codrich*, F. Silvestri
Department of Pathological Anatomy, University of Trieste, Trieste,
*IRCCS ”Burlo Garofalo”, Trieste, Italy
|Systemic lupus erythematosus (SLE) is a systematic disorder which may
involve the gastrointestinal tract. Symptoms may vary from non-specific
anorexia, nausea and abdominal pain to major complications such as hemorrhagic
enteropathy and intestinal infarction.
A 17-year-old boy was hospitalised for severe abdominal pain and high
fever mimicking perforated appendicitis. The operative findings revealed
a nonbacterial peritonitis with a gangrenous necrotic appendix and a large
amount of hemorrhagic ascitis. In the early postoperative period, persisting
high fever and abdominal pain, the patient developed rectal hemorrhage
with a skin rash. A vasculitic screen, showing low complement titres, thrombocytopenia
and positive specific lupus autoantibodies, allowed the suspicion of SLE.
Histology of the appendix confirmed necrotizing vasculitis. High-dose steroid
and immunoglobulin were started. Clinical and instrumental examinations
failed to uncover the site of the bleeding. In the 4th postoperatively
day, a new massive episode of rectal bleeding required emergency laparotomy,
revealing a diffuse edema and the dilatation of the last portion of the
ileum and cecum with an engorgement of the mesenteric vessels. An ileocolonic
anastomosis was performed.
Examination of the resected specimen showed several superficial longitudinal
microulcers on the antimesenteric aspect of the ileum. Some blood clots
were adhering to the ulcers in the ileum and in the colon. The more distal
colon was conspicuously edematoused. The histologic examination showed
an intensively edematous and hemorrhagic submucosa. The majority of the
submucosal arteries and arterioles were normal. In contrast, the submucosal
venules showed transmural inflammation with focal fibrinoid necrosis. Immunoperoxidase-stained
sections confirmed the presence of fibrinogen in the walls of the venules.
The patient was treated with cyclophosphamide and a decreasing oral dose
of corticosteroids. At the 7 month follow-up he had no clinical symptoms,
vasculitis screen were negative on 5 mg prednisolone daily.