Collagenous Sprue - A Clinico-pathological Study of 9 Cases A

Slide Seminar
BDIAP
MAY 2010
Prof Kieran Sheahan
Pathology Dept,
Centre for Colorectal Disease
St Vincent’s University Hospital
Dublin Academic Medical Centre & UCD
CLINICAL HISTORY
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57 year old male
History of coeliac disease since childhood
Mesenteric lymphadenopathy on CT
At laparotomy: stricture 30cm proximal to
ileocaecal valve
• 15cm length of small bowel
• Circumferential mucosal ulceration 10cm
long with focal stricture formation.
MICROSCOPY
Masson’s
Trichrome
CONGO RED
MARGIN
A
C
B
D
E
Figure 3. Case 12. A. CS and ulcerative jejuno‐ileitis (H&E). B. Masson’s Trichrome. C. Non‐
ulcerated mucosa showing CS and increased IELs (H&E). D. CD3. E. CD8 loss.
Diagnosis = Collagenous sprue
• Rare form of small bowel enteropathy.
• Pathologic lesion consists of subepithelial
collagen deposition associated with
variable alterations in villous architecture.
• Characterised clinically by chronic
diarrhoea and progressive malabsorption.
• It has traditionally been associated with
significant morbidity
‘Collagenous sprue-an unrecognized
type of malabsorption’
Weinstein et al., NEJM, 1970
• 51-year-old woman thought to have
refractory coeliac disease
• Transient improvement with steroids
• Post mortem - villous flattening with
thickened sub-epithelial collagen layer
• A similar lesion may have been described
by Schein (1947) and Hourihane (1963)
Patient Profile
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8 females, 4 males
Age range 41-84 years
Symptoms: weight loss, chronic diarrhoea
History of Coeliac Disease and/or positive
serology in 4 cases
• Negative serology in 4 cases without CD
JEJUNAL BIOPSYSUBEPITHELIAL
COLLAGEN
VARYING
DEGREES OF
VILLOUS
ATROPHY
EPITHELIAL
DETACHMENT
SUB-EPITHELIAL
COLLAGEN
DEPOSITION
.
ENTRAPPED LAMINA PROPRIA CELLS
AND VESSELS WITHIN COLLAGEN BAND
CD3
VARIATION OF
INTRA-EPITHELIAL
LYMPHOCYTOSIS
CD3
LAMINA PROPRIA LYMPHOCYTES,
PLASMA CELLS,
EOSINOPHILS
AND NEUTROPHILS
COLLAGENOUS
GASTRITIS
LYMPHOCYTIC
GASTRITIS
COLLAGENOUS
COLITIS
Small bowel histology
Gastric histology (4/7 bx)
Colonic histology (7/9 bx)
Collagenous sprue
Collagenous gastritis
Collagenous colitis
Lymphocytic colitis
Collagenous sprue
Chronic gastritis
No biopsy
Collagenous sprue
Lymphocytic gastritis
Normal colonic biopsy
Collagenous sprue
No biopsy
Normal colonic biopsy
Collagenous sprue
Collagenous gastritis
Collagenous colitis
Collagenous sprue
Normal antral biopsy
No biopsy
Collagenous sprue
No biopsy
Collagenous colitis
Collagenous sprue
No biopsy
Collagenous colitis
Collagenous sprue
No biopsy
Collagenous colitis
Collagenous sprue
Collagenous gastritis
Collagenous colitis
Collagenous sprue
Reactive gastropathy
Collagenous colitis
Collagenous sprue
Ulcerative jejuno-ileitis
No biopsy
No biopsy
CVID
A
B
C
D
Figure 2. Associated GIT findings. A . Collagenous colitis (H&E). B. Lymphocytic colitis (H&E). C. Collagenous gastritis (H&E). D. Lymphocytic gastritis, lymphocytes highlighted by CD45.
Treatment and Outcome
6 patients improved
clinically
- 1 improved with GFD
- 2 treated with GFD and
immunosuppressants
- 2 treated with
immunosuppressants
alone.
- Follow-up small bowel
biopsy normal in 3
cases.
3 patients died
- 2 died from complications
of malnutrition (despite
treatment with GFD and
immunosuppressive
therapy)
- 1 pt with multiple comorbidities died of
intercurrent illness
- Unknown in 3 cases
Our case
No patient has developed lymphoma
- 5 new cases of collagenous sprue and
extensive literature review
AJSP 24(5):676687, May 2000
- 13/30 patients known to have died from
complications of disease
- 7 cases of collagenous sprue.
The Lancet - Vol. 356,
Issue 9225, 15 July
2000, P 203-208
- Clonal TCR gamma configurations were found in 5/6
- 3 of these patients died from malnutrition.
Gut, Oct 2007; 56: 1373 - 1378.
23/50(46%) patients with RCD type 2 died from
Enteropathy Associated T cell Lymphoma
2010
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Mayo Clinic, n = 30
Gluten-free diet and steroid treatment are effective therapy for most
patients with collagenous sprue.
Rubio-Tapia A, Talley NJ, Gurudu SR, Wu TT, Murray JA.
Clin Gastroenterol Hepatol. 2010 Apr;8(4):344-349.e3. Epub 2010
Jan 6.
Memorial-Sloan Kettering n =30
Collagenous sprue is not always associated with dismal outcomes:
a clinicopathological study of 19 patients.
Vakiani E, Arguelles-Grande C, Mansukhani MM, Lewis SK,
Rotterdam H, Green PH, Bhagat G.
Mod Pathol. 2010 Jan;23(1):12-26. Epub 2009 Oct 23.
Take home message
• F>M, middle aged and elderly
• Some cases likely to represent a complication of
coeliac disease
• Most cases part of a collagenous enterocolitis
• ? End-point of different biological insults
• Risk of clinical lymphoma low in this series
• Role of molecular studies needs more
investigation
• Longer follow-up needed on PCR positive cases
• Prognosis is unpredictable