Summary: A 41-year-old white woman presented with a 1-day history of fever to
102.6˚F and tenderness around her Hickman catheter site. The catheter
had been placed approximately 3 years earlier for administration of
continuous Flolan therapy to control pulmonary hypertension. No erythema
or discharge was noted at the catheter site, although the patient did
report intermittent diarrhea for
1 month occurring approximately 3 or 4 times per week, associated with
nausea, vomiting, and abdominal soreness. Past medical history was
significant for autoimmune hepatitis resulting in orthotopic liver
transplant and postoperative pulmonary embolus. A physical exam revealed
protrusion of the Hickman catheter 3 inches from the skin without
redness or drainage. Her lungs were clear, although an S4 was
auscultated on the heart exam. Bowel sounds were normal. Laboratory data
revealed a white blood cell (WBC) count of 22,600. Stool studies were
negative of ova and parasites. Computed tomography (CT) of the abdomen
was performed to exclude possible abscess (Figures 1 and 2).
The abdominal CT demonstrates absence of the spleen and multiple rounded
hypodensities in the retrocrural and aortocaval regions representing
cavitary lymph nodes. The differential diagnosis includes lymphoma,
infection (Mycobacterium avium-inreacellulare), Whipple’s disease, and metastatic carcinoma post-therapy.
Celiac sprue, or gluten-induced enteropathy as it is more
appropriately known, is characterized by intolerance to wheat glutens.
More common in women, the disease has widely variable symptomology.
Clinically, the symptoms range from severe malabsorption to mild
abdominal pain, though many patients are asymptomatic. The classic
presentation of malabsorption and weight loss occurs in only a minority
of patients. Diarrhea is the most common feature of the disease. The
cause of diarrhea in celiac sprue is multifactorial, resulting from
impaired absorption by the duodenum and jejunum, and net secretion by
jejunal mucosa. Additionally, osmotic diuresis in the colon from
unabsorbed fats contributes to water in the stool.
Celiac sprue typically presents in the second decade of life. While no clear
inheritance pattern has been established, the disease does occur more
often in patients demonstrating the serum histocompatibility antigens
HLA-DR3 and HLA-DQw2. This association suggests a possible immune
reaction mediated within the intestinal mucosa. Additional hypotheses
include adenovirus infection, secondary to homology of the amino acid
sequence involving gluten and a viral-encoded protein.
As no pathognomic clinical features exist, diagnosis is made by biopsy of
intestinal mucosa. The pathologic changes are villous atrophy and crypt
hyperplasia secondary to toxic prolamines. Inflammatory cells are
identified within the lamina propria. Changes are usually most severe in
the proximal small bowel. Confirmation of diagnosis is made by
improvement with a gluten-free diet.
Radiologic findings are nonspecific. The jejunum and ileum demonstrate dilation, with the degree
of distension correlating with severity of disease. Transient
intussusceptions typically occur. The pathologic findings result in
diminished mucosal folds within the jejunum, the (ilealization of
jejunum) appearance. The ileum, however, demonstrates prominent folds
(jejunization of ileum). The “Moulage sign,” a featureless column of
barium in the small bowel, is sometimes seen. Radiographic signs
secondary to metabolic effects of malabsorption include osteopenia,
compression fractures, and Looser’s zones (insufficiency) fractures.
Less common radiographic findings include lymph node cavitation and
splenic atrophy, which may be associated with more widespread lymphoid
tissue atrophy.1 Splenic atrophy with lymph node cavitation
is an ominous sign, associated with lack of response to gluten-free diet
and poor prognosis.2 Howat et al reported 5 cases of lymph
node cavitation and splenic atrophy with poor response to withdrawal of
gluten from the diet. Of the 5 cases reported, 3 patients died.2 Additional case reports in the literature demonstrate similar findings.3
The major cause of death in celiac disease associated with cavitary
lymph nodes and splenic atrophy is overwhelming infection complicated by
hyposplenism and cachexia.1,2
Celiac disease may be asymptomatic. The abdomen CT excludes possible
abscess. In other patients, studies have found cavitation of mesenteric
lymph nodes is an original feature, which may be associated with splenic
atrophy and a flat small intestinal mucosa; and some of these patients
may have celiac disease.1
- Matuchansky C, Colin R, Hemet J, et al. Cavitation of mesenteric
lymph nodes, splenic atrophy, and a flat small intestinal mucosa. Gastoenterology. 1984;87:606–614.
- Howat AJ, McPhie JL, Smith DA, et al. Cavitation of mesenteric lymph
nodes: A rare complication of celiac disease, associated with a poor
outcome. Histopathology. 1995;27:349–354.
- Holmes G. Mesenteric lymph node cavitation in coeliac disease. Gut. 1986;27:728–733