collagenous sprue
DESCRIPTION
Collagenous SprueTRANSCRIPT
Does the gluten-free diet
is the wrong prescription ?
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
Patient profile
47 year old Saudi male
Referred at 31/12/2012 to our clinic for further management
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.
No history of diarrhea , mucus discharge , melena , or fresh blood .
No experienced of nocturnal symptoms.
Case Presentation
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 .
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
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 .
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
Case Presentation
No similar condition in the family
No chronic illness in the family
• Family history
Case Presentation
Living in Medina with his family
Medium class, NOT smoker or alcoholic
Married with no extramarital activity
• Social history
• Systemic review Unremarkable
Differential diagnosis
Case Presentation
1) Celiac disease2) Infections 3) Crohn's disease 4) Lymphoma
• Differential diagnosis
ON EXAMINATION
Case Presentation
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
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
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
Case Presentation
Neurological exam + MS UNREMARKABLE and grossly intact
• On Examination
Urine depstik
Negative
LAB Result
Case Presentation
CBC & chemistry
WBC 5.5 normal diffHB 16.2PLT 379
Na 144K 3.8Urea 3.5Cr 80
• LAB Result
ALP 56 T.Bili 8ALB 29 ALT 11
Ca 2.38Po4 1.05ESR 1CRP 65
Differential diagnosis
Case Presentation
1) Celiac disease2) Crohn's disease 3) Infections 4) Lymphoma
• Differential diagnosis
Work up :
Case Presentation
Pt underwent CT scan of the abdomen which showed :
• Work up :
Case Presentation
Case Presentation
jejunization of the ileum
CT abdomen and pelvis with IV contrast
FINDINGS :
Multiple mesenteric lymphadenopathies , largest one measuring 1.7cm.
Mild hepatomegaly.
Jejunization of the ileum
Differential diagnosis
Case Presentation
1) Celiac disease
2) Crohn’s disease
3) Autoimmune Enteropathy
4) I.P.S.I.D ( Immunoproliferative small intestinal disease )
• Differential diagnosis
Case Presentation
Pt underwent : Upper and lower GI endoscopy + Enteorscopy
With biopsy taken already
• Work up cont…
Case Presentation
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.
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.
Histopathology
Case Presentation
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.
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
Differential diagnosis
Case Presentation
Case Presentation
1) Crohn’s disease !
2) Celiac disease !!
3) I.P.S.I.D ( immunoproliferative small intestinal disease ) !!?
• Differential diagnosis
Work up ….
Villous Atrophy and Negative Celiac Serology
Villous Atrophy and Negative Enterocyte Antibody
Villous Atrophy and Negative ASCA
Case Presentation
Diagnosis
Case Presentation
Small Bowel Crohn's Disease
Case Presentation
Case Presentation
Management
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
Case Presentation
Patient present to ER4/1/2014
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
Work up :
Case Presentation
CBC
WBC 5.6
Hg 13.5
Platelet 408
U&E
Creatinine 63
Na 124
K 3.1
Albumin 17
Case Presentation
Started on Antibiotic - Ciprofloxacin- Metronidazole
- NPO
- Vigrous Hydration
Case Presentation
CT Abdomen
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www.themegallery.com
Case Presentation
Comparison to the previous study done on January 2013, there is still significant mesenteric lymphadenopathy.
No hepatomegaly or splenomegaly is seen
Radiology Impression :
The overall picture is compatible with
severe inflammatory process of small bowel
The differential diagnosis may include :
- Active Crohn's disease
- Infectious Enterocolitis
Case Presentation
Colonoscopy up to Terminal Ileum
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
Gastroscopy
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
Methylprednisolone 20 mg iv bid initiated
&TPN - Total Parenteral Nutrition
Case Presentation
Terminal Ileum Histopathology
Terminal Ileum Histopathology :
Fibrosis and chronic inflammation
Case Presentation
Duodenal Histopathology
Duodenal Histopathology
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
Diagnosis
Case Presentation
Case Presentation
Collagenous sprue (CS)
Case Presentation
Management
Gluten Free Diet &
Anti - TNF – Adalimumab initiated
Case Presentation
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
Collagenous sprue (CS)
Collagenous sprue (CS)
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
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.
Collagenous sprue (CS)
REV ESP ENFERM DIG 2013; 105 (3): 171-174
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.
Collagenous sprue (CS)
REV ESP ENFERM DIG 2013; 105 (3): 171-174
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
Collagenous sprue (CS)
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.
Collagenous sprue (CS)
REV ESP ENFERM DIG 2013; 105 (3): 171-174
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.
Collagenous sprue (CS)
REV ESP ENFERM DIG 2013; 105 (3): 171-174
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.
Collagenous sprue (CS)
REV ESP ENFERM DIG 2013; 105 (3): 171-174
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.
Collagenous sprue (CS)
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.
Collagenous sprue (CS)
REV ESP ENFERM DIG 2013; 105 (3): 171-174
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
Collagenous sprue (CS)
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.
Collagenous sprue (CS)
Z Gastroenterol 2009; 47(6): 575-578
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.
Collagenous sprue (CS)
Z Gastroenterol 2009; 47(6): 575-578
Does the gluten-free diet
is the wrong prescription ?
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
No
Dr Waleed Mahrous
Collagenous sprue (CS)