Gastrointestinal Sarcoidosis

1
Gastrointestinal Sarcoidosis

• Katherine Doktor, MD
• PGY-2

2
The Patient

• 52yoF, AA p/w exacerbation of chronic N/V/abd
  pain, a/f decreased PO intake
• PMHx HTN, DM, sarcoidosis, chronic pancreatitis,
  evidence of carcinoid in duodenum, ?autoimmune
  hepatitis dxd 10yrs ago
• PSurgHx removal of sarcoid nodule on dorsum of L
  hand, s/p Whipple J-tube placement April 10
• Dx of Sarcoidosis in Sept 09 by needle Bx of
  medistinal node skin mass on hand

3
The Patients Workup

• Labs
• CA 19-9
• Alk phos
• ERCP x2 (Feb March 10) findings c/w
  pancreatic divisum, chronic pancreatitis
  demonstrated pancreatic mass in head
• Gastric emptying scan delayed gastric emptying
  in March, but not during this admission
• s/p Whipple (4/10) surgical management bc of
  pts nutritional decline, presence on pancreatic
  head mass and elevated CA 19-9
• Obstruction series no evidence of obstructing
  lesions

4
Sarcoidosis

• First described by Hutchinson in 18773
• Non-caseating granulomas
• Multisystem disease lungs, skin, cardiac,
  neurologic, LN
• Frequency of organ involvement lung (90) gt LN
  (75) gt liver (60-90)
• GI involvement gastric 2.5, intestinal 3.4
• Age group 20-40s
• Prevalence 1-40/100,000
• At least 3x higher in AA than Caucasians
• Main cause of death due to involvement of lungs
  and heart

5
Sarcoidosis Making the Diagnosis

• History
• Demonstration of granulomas in two different
  organs
• Rule-out drug-induced disease

6
Organ involvement

• Liver most common
• Hollow organs stomach, small bowel, colon
• Pancreas
• 1st described in 1937

7
Sarcoidosis of the Liver

• Involvement seen in 50-79 of pts by Bx and
  67-70 by autopsy
• Pts w/ fever /or arthralgias or extensive
  erythema nodosum tend to have abnl liver Bx
• Sx pruritis abd pain gt jaundice
• DDx of hepatic granulomas
• sarcoidosis
• Drug-induced disease
• Underlying liver disease (esp PBC)
• TB

8
Sarcoidosis of the Liver the work-up

• Liver enzymes elevated in up to 35, unrelated
  to degree or extent of disease
• Elevated Alk phos is more reliable than GGT in
  predicting liver involvement
• US parenchymal echogenicity, coarsening of the
  liver parenchyma w/or w/o discrete nodules, and
  focal calcifications as well as contour
  irregularity
• CT HSM, adenopathy, particularly of the porta
  hepatis, paraaortic region and celiac axis,
  followed in frequency by low-attenuation lesions
  of the liver and spleen
• MRI multiple, diffuse, densely packed, uniform
  nodular foci w/nml to slightly increased signal
  intensity. T2-weighted images show decreased
  signal intensity

9
Sarcoidosis of the Liver

• Granulomas
• 24-94 of liver Bx autopsies
• Granulomas classic sarcoid granulmas mainly
  found in portal triads w/a cluster of lg
  epithelioid cells, often w/ multinucleated giant
  cells.
• Schaumann bodies rarely seen in hepatic
  granulomas
• Granluomas may be of differing ages
• Progressive Liver disease

10
Sarcoidosis of the Stomach

• Most common in antrum
• Sx most common is postprandial epigastric pain
  (dull, burning or cramping) also N/V, heartburn,
  early satiety and weight loss (common, can be
  severe), occ UGIB
• Manifestations due to ulcers or lumen narrowing
  2/2 fibrosis
• Endoscopy nodular changes, gastritis, thickened
  mucosa, greater or lesser curvature deformities
  or benign or malignant-appearing ulcers
• UGI series most commonly shows segmental mucosal
  thickening nondistensibility (mimicking linitis
  plastica) /or deformities in antrum

11
Sarcoidosis of the Bowel

• Small bowel least commonly involved organ in GI
  sarcoidosis
• Presentation diarrhea, malabsorption,
  protein-losing enteropathy, periumbilical or
  epigastric pain or hemorrhage
• Colon multiple nodules, polyps, stenosis,
  obstructive lesions, aphthous erosions, or small
  punctate bleeding sites
• Over 50 pts have abd pain
• Most common cause of intestinal obstruction
  external compression by LAN

12
Sarcoidosis of the Pancreas

• Sx rare assocd w/parenchymal disease or duct
  obstruction
• abd pain, wt loss, jaundice, nausea, anorexia
• the findings may simulate carcinoma w/pain
  improving w/corticosteroids
• Findings
• a mass, usually in the head or
• a diffusely firm, nodular pancreas

13
Sarcoidosis of the Pancreasthe workup

• Labs chronically elevated lipase/amylase
• MRI masses w/low signal intensity on
  T1-weighted images, mild high signal intensity on
  T2-weighted images, and reduced enhancement
  w/gadolinium
• Endoscopic US glandular fibrosis
• Results of corticosteroid Tx decreased
  lipase/amylase, reduced size of liver spleen,
  but no improvement in fibrosis

14
Additional Involvement of Sarcoidosis with the GI
Tract

• Acute cholecystitis
• Obstructive jaundice
• Abdominal adenopathy
• Increased in number of normal-sized nodes
• Most common in the porta hepatis
• LN smaller and more discrete than in lymphoma
• IFN-a therapy for Hepatitis C

15
Treatment of GI Sarcoidosis

• Partial response to PPI and antacids
• Corticosteroids
• Start when organ function is threatened
• 66 of people show improvement in Sx
• Hepatic sarcoid
• Tx only symptomatic pts
• Only minimal effect on hepatic lesions
• Reduces liver size number of granulomas
• No effect on portal HTN
• No improvement seen in cases involving chronic
  intrahepatic cholestasis
• Some improvement in LFTs in cases of mild-mod
  elevation, but no significant improvement in
  severe elevation

16
Treatment Steroid-sparing Agents

• Methotrexate
• Shown to be effective in an RCT
• 10 pts have toxic rxns
• Others chloroquine, azathioprine and
  cyclophosphamide
• Infliximab
• Ursodeoxycholic acid

17
REFERENCES

• Ebert EC, Kierson, KD Hagspiel. Gastrointestinal
  and Hepatic Manifestations of Sarcoidosis. Am J
  Gastroenterol 2008 1033184-3192.
• Sharma OP. Gastrointestinal sarcoidosis. In
  UpToDate, Hollingsworth, H (DE), UpToDate,
  Waltham, MA 2010.
• 3 caceres m, sabbaghian s, braud r, wilks s, b
  michael. Pancreatic sarcoidosis Unusual
  Presentation Resembling a Periampullary
  Malignancy. Curr Surg, 63 (2006)179-185.

18
DDx of granulomas

• Sarcoidosis
• Drug-induced disease
• Underlying liver disease (esp PBC)
• TB
• Whipples dz
• Crohns dz
• Syphilis
• Fungal infections
• Foreign body rxn