- Email: [email protected]
Are We Missing Early Primary Biliary Cirrhosis?
JOURNAL OF CLINICAL AND EXPERIMENTAL HEPATOLOGY
CONFLICTS OF INTEREST The authors have none to declare. http://dx.doi.org/10.1016/j.jceh.2017.05.096
9 ARE WE MISSING EARLY PRIMARY BILIARY CIRRHOSIS? Pallavi Bhuyan ∗ , Kaumudi Pattnaik, Bidyut Das, Rina Tripathy
Figure 1. Bile duct basement membrane damage was demonstrated by diastase-PAS stain.
antibody in 95% cases. Fatigue and pruritus are most common presenting features. Coexistence of PBC with other autoimmune diseases has been documented, with strongest association with rheumatoid arthritis, CREST syndrome, SLE, dermatomyositis and autoimmune thyroiditis. Rheumatologic manifestations are reported in 4–50% of patients with PBC. The coexistence of small joint polyarthritis, fever and PBC is rare. Case Summary: Clinical examination, hematological investigations, biochemical evaluation, USG of the abdomen, viral serology, immunological tests, histopathological (HP) examination with Masson’s Trichrome and PAS stain were done. Patient presented with fever and symmetrical polyarthritis, affecting small joints of the hand. Systemic examination was within normal limits. Investigations showed Hb of 9.6 g%, TLC of 13.8 × 109 /L, DC neutrophilia, TPC of 435 × 109 /L, ESR of 55 mm/1st hour, CRP was 6 mg/dl. Biochemical evaluation revealed FBS of 87 g%, total bilirubin of 0.8 mg/dl, SGOT 19 IU/L, SGPT 12 IU/L, alkaline phosphatase 119 IU/L. Widal, Mantoux, ICT for malaria and urine analysis were all within normal limits. USG of the abdomen and X-ray hands were normal. Rheumatoid factor was negative. Anti-viral antibodies like HBsAg, HCV, HIV was negative. Immunological investigations showed ANA 1.2, ds DNA 125 U/ml, C3 146 mg/dl, C4 23 mg/dl; ANA profile revealed AMA M2 +++. HP study confirmed stage I PBC. Conclusion: The detection of PBC in a young women presenting with polyarthritis and fever is unusual. Presence of normal biochemical liver profile is still rare. Presence of AMA-M2 antibody is the most significant indicator for the presence of PBC. With emerging evidence of favorable response of UDCA in early PBC, attempts to detect patients as early as possible will affect management, prognosis and outcome (Figure 1).
CONFLICTS OF INTEREST
SCB Medical College, Cuttack, India
The authors have none to declare.
E-mail address: [email protected] (P. Bhuyan). Background and Aim: Primary biliary cirrhosis (PBC) is a cholestatic autoimmune liver disease characterized by destruction of medium-sized intrahepatic bile ducts. It is associated with presence of antimitochondrial
http://dx.doi.org/10.1016/j.jceh.2017.05.097
Journal of Clinical and Experimental Hepatology July 2017 Vol. 7 No. S2
S51
CIRRHOSIS AND COMPLICATIONS
loss and jaundice. Investigations showed direct hyperbilirubinemia, high transaminases (SGOT > SGPT), high globulin (6.8 g/dl) with raised prothrombin time. Viral markers (A, B, C, E, CMV, and EBV) were negative, no significant family history or hepatotoxic drug history was noted. Autoimmune work up showed ANA positive with high IgG levels (42.5 g/l), all other antibodies were negative. Ultrasound revealed altered liver echo pattern, mild splenomegaly and no ascites. Liver biopsy showed features consistent with chronic autoimmune hepatitis (AIH). He was started on tapering doses of prednisolone and azathioprine with rapid clinical recovery, near normalisation of LFT and reduced levels of IgG. Two years after the diagnosis of autoimmune hepatitis, he presented with chronic bloody diarrhoea and weight loss. He also had iron deficiency anemia, thrombocytosis but normal LFT. Colonoscopy and mucosal biopsy revealed classical features of moderately active chronic ulcerative colitis (UC) even though patient was on immunosuppressants. He was started on topical and oral 5-ASA. He remained in remission for both AIH and left sided ulcerative colitis for two years. He again had a flare of ulcerative colitis, colonoscopy showed progression from left sided colitis to pan colitis with hypoalbuminemia but normal transaminases, treated with tapering doses of prednisolone and 5-ASA for flare of UC with good response to treatment. Conclusion: This case represents rare combination of autoimmune hepatitis without primary sclerosing cholangitis who developed ulcerative colitis two years later even on immunosuppressants. He had independent flares of UC and AIH despite being on regular doses of immunosuppressant over the years.
CONFLICTS OF INTEREST The authors have none to declare. http://dx.doi.org/10.1016/j.jceh.2017.05.096
9 ARE WE MISSING EARLY PRIMARY BILIARY CIRRHOSIS? Pallavi Bhuyan ∗ , Kaumudi Pattnaik, Bidyut Das, Rina Tripathy
Figure 1. Bile duct basement membrane damage was demonstrated by diastase-PAS stain.
antibody in 95% cases. Fatigue and pruritus are most common presenting features. Coexistence of PBC with other autoimmune diseases has been documented, with strongest association with rheumatoid arthritis, CREST syndrome, SLE, dermatomyositis and autoimmune thyroiditis. Rheumatologic manifestations are reported in 4–50% of patients with PBC. The coexistence of small joint polyarthritis, fever and PBC is rare. Case Summary: Clinical examination, hematological investigations, biochemical evaluation, USG of the abdomen, viral serology, immunological tests, histopathological (HP) examination with Masson’s Trichrome and PAS stain were done. Patient presented with fever and symmetrical polyarthritis, affecting small joints of the hand. Systemic examination was within normal limits. Investigations showed Hb of 9.6 g%, TLC of 13.8 × 109 /L, DC neutrophilia, TPC of 435 × 109 /L, ESR of 55 mm/1st hour, CRP was 6 mg/dl. Biochemical evaluation revealed FBS of 87 g%, total bilirubin of 0.8 mg/dl, SGOT 19 IU/L, SGPT 12 IU/L, alkaline phosphatase 119 IU/L. Widal, Mantoux, ICT for malaria and urine analysis were all within normal limits. USG of the abdomen and X-ray hands were normal. Rheumatoid factor was negative. Anti-viral antibodies like HBsAg, HCV, HIV was negative. Immunological investigations showed ANA 1.2, ds DNA 125 U/ml, C3 146 mg/dl, C4 23 mg/dl; ANA profile revealed AMA M2 +++. HP study confirmed stage I PBC. Conclusion: The detection of PBC in a young women presenting with polyarthritis and fever is unusual. Presence of normal biochemical liver profile is still rare. Presence of AMA-M2 antibody is the most significant indicator for the presence of PBC. With emerging evidence of favorable response of UDCA in early PBC, attempts to detect patients as early as possible will affect management, prognosis and outcome (Figure 1).
CONFLICTS OF INTEREST
SCB Medical College, Cuttack, India
The authors have none to declare.
E-mail address: [email protected] (P. Bhuyan). Background and Aim: Primary biliary cirrhosis (PBC) is a cholestatic autoimmune liver disease characterized by destruction of medium-sized intrahepatic bile ducts. It is associated with presence of antimitochondrial
http://dx.doi.org/10.1016/j.jceh.2017.05.097
Journal of Clinical and Experimental Hepatology July 2017 Vol. 7 No. S2
S51
CIRRHOSIS AND COMPLICATIONS
loss and jaundice. Investigations showed direct hyperbilirubinemia, high transaminases (SGOT > SGPT), high globulin (6.8 g/dl) with raised prothrombin time. Viral markers (A, B, C, E, CMV, and EBV) were negative, no significant family history or hepatotoxic drug history was noted. Autoimmune work up showed ANA positive with high IgG levels (42.5 g/l), all other antibodies were negative. Ultrasound revealed altered liver echo pattern, mild splenomegaly and no ascites. Liver biopsy showed features consistent with chronic autoimmune hepatitis (AIH). He was started on tapering doses of prednisolone and azathioprine with rapid clinical recovery, near normalisation of LFT and reduced levels of IgG. Two years after the diagnosis of autoimmune hepatitis, he presented with chronic bloody diarrhoea and weight loss. He also had iron deficiency anemia, thrombocytosis but normal LFT. Colonoscopy and mucosal biopsy revealed classical features of moderately active chronic ulcerative colitis (UC) even though patient was on immunosuppressants. He was started on topical and oral 5-ASA. He remained in remission for both AIH and left sided ulcerative colitis for two years. He again had a flare of ulcerative colitis, colonoscopy showed progression from left sided colitis to pan colitis with hypoalbuminemia but normal transaminases, treated with tapering doses of prednisolone and 5-ASA for flare of UC with good response to treatment. Conclusion: This case represents rare combination of autoimmune hepatitis without primary sclerosing cholangitis who developed ulcerative colitis two years later even on immunosuppressants. He had independent flares of UC and AIH despite being on regular doses of immunosuppressant over the years.