Overlap Syndrome of Autoimmune Hepatitis and Primary Biliary Cirrhosis: A Clinical Enigma
Journal Title: Journal of Advanced Research in Medicine - Year 2017, Vol 4, Issue 3
Abstract
“Overlap syndrome” is a term used to describe variant forms of autoimmune hepatitis (AIH) which present with characteristics of AIH and primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). Patients with overlap syndrome present with both hepatitic and cholestatic serum liver test and the clinical, biochemical, and histological features of these autoimmune diseases are overlapped. Thus, it is difficult to appreciate overlap syndrome as an actual diagnostic entity. AIH-PBC is the most common form of overlap syndrome, affecting almost 10% of adults with AIH or PBC. Transitions from PBC to AIH-PBC overlap syndrome have also been reported. Ursodeoxycholic acid is usually combined with immunosuppressive therapy but end-stage disease requires liver transplantation. We present a case of Overlap Syndrome (AIHPBC) in a 16 year old girl who presented to us with features of obstructive jaundice. She had six months history of generalised itching, yellow discoloration of skin and urine, decreased appetite and intermittent abdominal pain. Liver function test revealed conjugated hyperbilirubinemia, with moderate elevation of liver enzymes. Antinuclear, anti-muscle M2 and anti-glycoprotein 210 antibodies were positive. Liver biopsy showed features of primary biliary cirrhosis. The patient responded to ursodeoxycholic acid and immunosuppression.
Authors and Affiliations
Sanjay Kumar
Keywords
Related Articles
- EP ID EP261038
- DOI 10.24321/2349.7181.201705
- Views 130
- Downloads 0
Acute Stroke as the Initial Presentation of Takayasu Arteritis: Rare but Not Infrequent
Takayasu arteritis is an idiopathic, autoimmune, large vessel vasculitis, mainly affecting the young adults. Its exact etiopathogenesis is not known and it has varied clinical presentations.Acute stroke as initial presen...
Unusual Involvement of Lateral Recess of Vertebrae in Potts Spine
Spinal tuberculosis is the commonest manifestation of extra-pulmonary tuberculosis. It usually presents with backache, paraparesis, and spinal deformity. It accounts for 2% of all tuberculosis cases, 15% of extra-pulmona...
Unilateral Ptosis - An Unusual Initial Presentation of Acute Myeloid Leukaemia in an Adolescent Girl
In case of acute myeloid leukaemia (AML), there is increased multiplication of blast cells forming the malignant clones of immature myeloid cells in the bone marrow in different body tissues and also enormously carried b...
Hirayama Disease with Rare Presentation in a Young Adult
Hirayama disease or Monomelic Amyotrophy (MMA) is a type of rare cervical myelopathy which is an asymmetrical, self-limited with progressive atrophy and weakness of the muscles of hands and forearms. It is mainly found i...
Chronic Constipation due to Hypercalcemia Associated with Parathyroid Adenoma: An Uncommon Presentation - A Brief Report
Constipation is a very common problem and many a times its cause remains obscure. We present a case of 55-year old women who presented with chronic constipation. Physical examination was essentially normal. Investigation...