Collagenous Sprue

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)

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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)