Are We Missing Early Primary Biliary Cirrhosis

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 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). The authors have none to declare.