Collagenous Sprue

2. V I L L O U S A T R O P H Y
D I F F E R E N T C A U S E S
 Presented by :
Dr. Waleed Mahrous

3. Patient profile
 47 year old Saudi male
 Referred at 31/12/2012 to our clinic for
further management

4. Case Presentation
 47 year old male patient NOT known to have any
medical illness
 The patient had been healthy until he developed severe
central abdominal pain colicky in nature not radiating
 His symptoms progressed to nausea and emesis after
2–3 weeks. The abdominal pain & emesis usually
occurred 30 minutes after eating, 1–5 times per
day, was bilious in nature, and was associated with
bloating.

5.  No history of diarrhea , mucus discharge
, melena , or fresh blood .
 No experienced of nocturnal symptoms.
Case Presentation

6. Case Presentation
 Patient look for medical advice in KFH in AL Medina
 After some investigations was done for him with some
image pt was Dx with partial intestinal
obstruction
 Treated with IVF, Abx, was kept NPO for sometimes
and NGT free drain
 Pt has much improvement after that .

7. Case Presentation
 Pt started to have watery diarrhea after that, on
and off but no blood with it also with mild abdominal
pain.
 Pt had significant weight loss from 105kg to
78kg since first presentation (around 5-6 months)
 No other symptoms associated with his presentation
 So, pt referred to our clinic for further management

8. Case Presentation
 No hx of fever , constipation , or PR bleeding
 No hx of hematemsis or melena
 No hx of skin discoloration or skin lesion
 No hx of eating from outside or use of antibiotics
 Wt loss with no change in his appetite since his presentation
 No eye symptoms or similar condition,
 No hx of joint pain or swelling .

9. Case Presentation
 Patient is NOT known to have any medical illness
before
with no previous hospitalization except for his early
presentation
 No past surgical history
 NOT known to have any allergy
 NOT using any medication
• Past history

10. Case Presentation
 No similar condition in the family
 No chronic illness in the family
• Family history

11. Case Presentation
Living in Medina with his family
Medium class, NOT smoker or alcoholic
Married with no extramarital activity
• Social history
• Systemic review
 Unremarkable

13. Case Presentation
1) Celiac disease
2) Infections
3) Crohn's disease
4) Lymphoma
• Differential diagnosis

14. Case Presentation

15. Case Presentation
 Patient was conscious, alert, oriented to time place
and person, NOT in distress, NOT in pain and lying
comfortable in bed, NOT cachectic, NO muscle
wasting No palpable LN
 Vital sign :
T : 36.9
BP: 116 67 HR : 87
RR : 17
SPO2 99% room air
• On Examination

16. Case Presentation
Abdominal
Soft and lax with no tenderness
No Organomegally - Spleen was NOT
palpable, Liver around 12 cm span
PR Exa. was Normal
• On Examination

17. Case Presentation
C.V.S
No scar or deformity of the chest
S1 + S2 + o , No palpable or audible murmure
• On Examination
Respiratory
Fair air entry bilaterally
No wheezs or crepeatation

18. Case Presentation
Neurological exam + MS
UNREMARKABLE and grossly intact
• On Examination
Urine depstik
Negative

19. LAB Result

20. Case Presentation
CBC & chemistry
WBC 5.5 normal diff
HB 16.2
PLT 379
Na 144
K 3.8
Urea 3.5
Cr 80
• LAB Result
ALP 56 T.Bili 8
ALB 29 ALT 11
Ca 2.38
Po4 1.05
ESR 1
CRP 65

22. Case Presentation
1) Celiac disease
2) Crohn's disease
3) Infections
4) Lymphoma
• Differential diagnosis

24. Case Presentation
Pt underwent CT scan of the abdomen
which showed :
• Work up :

26. Case Presentation

27. Case Presentation
jejunization of the ileum

28.  CT abdomen and pelvis with IV contrast
FINDINGS :
Multiple mesenteric lymphadenopathies
, largest one measuring 1.7cm.
Mild hepatomegaly.
Jejunization of the ileum

30. Case Presentation
1) Celiac disease
2) Crohn’s disease
3) Autoimmune Enteropathy
4) I.P.S.I.D ( Immunoproliferative small intestinal
disease )
• Differential diagnosis

31. Case Presentation
Pt underwent :
Upper and lower GI endoscopy +
Enteorscopy
With biopsy taken already
• Work up cont…

32. Case Presentation

33. Case Presentation
• Upper endoscopy + single balloon
From duodenum to >1 Meter inside jejunum:
Nodularity , scallooping and ulceration.
Biopsy taken for AFB C/S and histopathology.

34. Case Presentation
• Colonoscopy (with Terminal Ileum intubation)
 RECTUM: 2 small flat polyps seen, removed with
biopsy forcips, no complications.
 SIGMOID to CECUM: No abnormalities seen.
 TERMINAL ILEUM: Diffuse nodularity with mild
erythema, no ulcers or lesions.
multiple biopsies taken.

35. Histopathology

36. Case Presentation

37. Case Presentation
Duodenal Histopathology :
The villous architecture is remarkably
distorted with shortening and focal
complete villous atrophy.
 No remarkable increase in the number of
CD3+ lymphocytes in the epithelium.

38. The lamina propria is expanded by a mixed
inflammatory infiltrate, of a
lymphoplasmacytic
There is glandular distortion, apoptosis &
regenerative changes
No definite malignant cells, granulomas or
infectious organisms detected
Suggestive of crohn's disease is high
Case Presentation

39. Case Presentation

40. Case Presentation
1) Crohn’s disease !
2) Celiac disease !!
3) I.P.S.I.D ( immunoproliferative small intestinal
disease ) !!?
• Differential diagnosis

41.  Work up ….
Villous Atrophy and Negative Celiac Serology
Villous Atrophy and Negative Enterocyte Antibody
Villous Atrophy and Negative ASCA
Case Presentation

42. Case Presentation

43. Case Presentation

44. Case Presentation

45.  Start Steroid Rx
 Prednisolone 40 mg po od for 2/52 then tapper
gradual until seen in clinic
 Seen in clinic at 6/52 where Imuran 200 mg
po od started and continue tapering steroid until
D/C
 Patient symptoms improved dramatically
Case Presentation

46. Case Presentation
Patient present to ER
4/1/2014

47. Patient present to ER with:
- Diarrhea 10 times > 3/52
- Recurrent Vomiting 15 times
2>52
- Loss Appetite
- Loss weight > 10 kg in 1/12
- Generalized weakness
Case Presentation

48. Case Presentation

49. CBC
WBC 5.6
Hg 13.5
Platelet 408
U&E
Creatinine 63
Na 124
K 3.1
Albumin 17
Case Presentation

50.  Started on Antibiotic
- Ciprofloxacin
- Metronidazole
- NPO
- Vigrous Hydration
Case Presentation

51. CT Abdomen
www.themegallery.com

52. www.themegallery.com
Case Presentation

54.  Comparison to the previous study done
on January 2013, there is still significa
nt mesenteric lymphadenopathy.
 No hepatomegaly or splenomegaly is se
en

55.  Radiology Impression :
The overall picture is compatible with severe in
flammatory process of small bowel
 The differential diagnosis may include :
- Active Crohn's disease
- Infectious Enterocolitis
Case Presentation

56. Colonoscopy up to
Terminal Ileum

57.  Colonoscopy
up to Terminal
Ileum : Only
seen nodular
ulcerated
mucosa of TI
other colon
normal
 Biopsy taken to r/o
TB CULTURE &
CMV
Case Presentation

58. Gastroscopy

59.  EGD : Thickened
edematous with
few superficial
ulceration at
gastric area
 Nodular with
large patchy
area of deep
ulcerated small
bowel
Case Presentation
2ed duodenal Part

60.  Methylprednisolone 20 mg iv bid
initiated
&
TPN - Total Parenteral Nutrition
Case Presentation

61. Terminal Ileum Histopathology

62.  Terminal Ileum Histopathology :
Fibrosis and chronic inflammation
Case Presentation

63. Duodenal Histopathology

64. Duodenal Histopathology

65.  Duodenal Histopathology :
The villous architecture is markedly
districted with shorting and focal
complete villous atrophy but without a
remarkable increase in number of CD 3
lymphocyte in epithelium.
Trichrome stain demonstrate a thick
collagenous subepithelial band
suggestive of collagenous sprue
Case Presentation

66. Case Presentation

67. Case Presentation

68. Case Presentation

69.  Gluten Free Diet
&
 Anti - TNF – Adalimumab
initiated
Case Presentation

70.  Patient seen in clinic 6 weeks from discharge
Improved symptoms
- No more diarrhea
- No more vomiting
- Feeling some time abdominal discomfort and
pain
- Increase weight by 5 kg since discharge
- Normal Lab
- Normal Albumin 43
Case Presentation

72.  INTRODUCTION
 Collagenous sprue is a severe
malabsorptive disorder, histologically
characterized by small intestinal
villous and crypt atrophy, and a
subepithelial collagen deposit, thicker
than 12 µm, that entraps lamina propria
cellular elements.
REV ESP ENFERM DIG 2013; 105 (3): 171-174

73.  Collagenous sprue is a rare disease
entity, with only about small No. of
sporadic cases reported worldwide since
it was first described in 1947.
 Its exact etiology is still under
investigation, and its relationship with
classic celiac disease and other
refractory, spruelike intestinal disorders
remains controversial.
REV ESP ENFERM DIG 2013; 105 (3): 171-174

74.  CS affects the small intestine (mainly
duodenum and proximal jejunum)
in a patchy way and with variable
intensity .
 Severity of symptoms correlates with
the overall length of bowel affected
rather than with the degree of
histological alterations.
REV ESP ENFERM DIG 2013; 105 (3): 171-174

75.  Those endoscopic findings, that is,
the reduction of folds, scalloping,
mucosal nodularity, are
suggestive, but nonspecific, of
collagenous sprue because they can
also be seen in classic celiac disease.
REV ESP ENFERM DIG 2013; 105 (3): 171-174

76. Treatment
 The management of CS is very
problematic. Thus far, there are no long-
term follow-up data available to compare
the most effective treatment regimens.
 Celiac sprue must be ruled out, and
dietary investigations should be
considered to detect unusual allergies
causing refractory sprue.
REV ESP ENFERM DIG 2013; 105 (3): 171-174

77.  Dietary gluten restriction should be the
first step even though patients are often
partially or totally unresponsive to gluten-free
diet, as previously reported.
 Parenteral nutrition has been proposed as
the best therapy because corticosteroid-
related complications such as osteopenia are
magnified in a chronic malabsorptive disorder.
REV ESP ENFERM DIG 2013; 105 (3): 171-174

78.  Total parenteral nutrition allows for
time to use immunosuppressives
that have been used to treat
refractory CD, to consider dietary
investigations, and to detect
unusual allergies.
REV ESP ENFERM DIG 2013; 105 (3): 171-174

79.  Long-term high-dose corticosteroids
remain the most effective
treatment option for CS, but the
dosing, tapering period, and side-effect
management needs to be investigated.
 Other options that have been used to
treat refractory CD may be useful in
the treatment of CS.

80.  A combination of nutrition
support, steroids, and
immunosuppressors such as
azathioprine, 6-mercaptopurine,
cyclosporine, or tumor necrosis
factor antibodies may be useful,
but lack clinical trials.
REV ESP ENFERM DIG 2013; 105 (3): 171-174

81.  Infliximab treatment in refractory
collagenous sprue: report of a case and
review of the literature
 27-year-old man developed watery diarrhea with
weight loss and abdominal pain. Duodenal
biopsies showed a subtotal villous atrophy with
an extensive subepithelial layer of collagenous
fibers.
 An apparent GFD did not reduce symptoms.
Z Gastroenterol 2009; 47(6): 575-578

82.  High dose steroid treatment (75 mg
prednisone) in combination with
azathioprine (150 mg) reduced diarrhea
but did not induce complete remission.
 Based on strongly elevated mucosal TNF-
alpha transcript concentrations we
introduced infliximab (5 mg/kg body
weight) into therapy.
Z Gastroenterol 2009; 47(6): 575-578

83.  After two applications the patient's
symptoms quickly improved.
 During the following year no recurrence
of diarrhea has been observed.
 This case suggests that infliximab is an
effective treatment in complicated
cases of collagenous sprue.
Z Gastroenterol 2009; 47(6): 575-578

84. V I L L O U S A T R O P H Y
D I F F E R E N T C A U S E S

85. Dr Waleed Mahrous