PATHOLOGY

PRESENTATION
ANOKHI PANCHAL
RAMKRUPAL GAUD
 CASE
A 19-year-old man presented at the emergency care unit complaining of lower abdominal pain, cramps, and bloody mucus
diarrhea for several weeks. He had a previous history of chronic diarrhea and pancolitis, which was treated initially but the
diarrhea relapsed. The physical examination on admission disclosed a severely ill patient who was pale and dehydrated. He had a
tender, distended abdomen with a positive rebound test, and cutaneous lesions of pyoderma gangrenosum, which was later
confirmed by a skin biopsy. Abdominal plain radiography and computed tomography revealed ascites and colonic wall edema, not
fulfilling the criteria for toxic megacolon. The results of the laboratory tests are given.

The risk of intestinal perforation was considered and the patient was referred for
possible colectomy. However, due to the severe clinical status impairment,
colectomy could not be undertaken and the patient died of septic shock. Autopsy
findings included involvement of the entire colon by extensive shallow ulcers
and pseudo polyps along with intestinal perforations in the right colon and acute
suppurative peritonitis. Histologically, there was effacement of the mucosal
architecture. Mucosal inflammation and crypt atrophy were also found in the
rectum along with backwash ileitis. The histopathological examination revealed
chronic inflammation surrounding the bile ducts together with concentric
periductal fibrosis and bridging portal fibrosis. Bile ducts were lacking in some
portal tracts. The spleen was enlarged and soft, weighing 220 g
DIAGNOSIS
 CLASSIC SYMPTOMS OF ULCERATIVE COLITIS
 CHRONIC INFLAMMATORY BACKGROUND
 ELEVATED ALP AND GGT
 STRICTURING OF BILE DUCT
 ONION SKIN FIBROSIS
 HEPATOSPLENOMEGALY

PATIENT HAD PRIMARY SCLEROSING COLANGITIS IN THE

SETTING OF SEVERE FORM OF ULCERATIVE COLITIS
 PRIMARY SCLEROSING COLANGITIS

 IMMUNOLOGICALLY MEDIATED DISEASE

 INFLAMMATION AND OBLITERATIVE
FIBROSIS OF INTRAHEPATIC AND
EXTRAHEPATIC BILE DUCTS
 THIRD THROUGH FIFTH DECADES OF LIFE
 MALE PREDOMINANCE
 CHARACTERISTIC “BEADING”
 UNPREDICTABLE BUT PROGRESSIVE
COURSE
 PATHOGENESIS

 GENETIC PREDISPOSITION - ASSOCIATION WITH HLA-DR52A (100%) AND HLA-CW7 (86%)

 LINKAGE TO ULCERATIVE COLITIS
 PRESENCE OF AUTOANTIBODIES = pANCA
 GUT LYMPHOCYTE HOMING
 MORPHOLOGY AND LABORATORY FINDINGS

 LARGE DUCT = RESEMBLES INFLAMMATION IN ULCERATIVE  INCREASED SERUM ALP AND GGT
COLITIS, STRICTURES  INCREASE BILIRUBIN
 SMALLER DUCTS = ONION SKIN FIBROSIS, TOMBSTONE SCAR
CLINICAL FEATURES
 DIAGNOSIS AND TREATMENT

 GOLD STANDARD FOR DIAGNOSIS = ENDOSCOPIC  TREATMENT –

RETROGRADE CHOLANGIOPANCREATOGRAPHY
 CAN ALSO DO MAGNETIC RESONANCE
CHOLANGIOPANCREATOGRAPHY
 IMMUNOSUPPRESSANTS
 ENDOSCOPIC DILATION WITH SPHINCTEROTOMY OR
STENTING
 LIVER TRANSPLANTATION
 REFERENCES
ROBBINS BASIC PATHOLOGY TENTH EDITION
GOLJAN RAPID REVIEW PATHOLOGY FOURTH EDITION
FIRST AID FOR THE USMLE STEP 1
Primary sclerosing cholangitis associated with severe ulcerative colitis in a yo
ung man - PMC (nih.gov)
https://www.xiahepublishing.com/2310-8819/JCTH-2021-00344
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3236286/
Primary Sclerosing Cholangitis - PMC (nih.gov)
The Role of Microbiota in Primary Sclerosing Cholangitis and Related Biliary
Malignancies - PMC (nih.gov)
THANK
YOU !!