Anti mitochondrial Ab seen typically in:

Primary Biliary cirrhosis is a disease characterized by chronic inflammation and fibrous obliteration of intrahepatic bile duclioles due to an unknown (primary) stimulus.   Etiology PBC is believed to due to an autoimmune etiology as it is often associated with other auto immune disorders such as  CRCST syndrome: Calcinosis, Raynaud's phenomena, Esophageal dysmotility, Sclero-dactily. Telangiectasia   Sicca syndrome Autoimmune thyroiditis Renal tubular acidosis Anti mitochondrial antibody (IgG is positive)   Clinical feature Most patients arc asymptomatic. Pruritis is commonest/earliest symptom Jaundice Deficiency of fat soluble vitamin due to malabsorption leads to: Vit D deficiency → Bone pain—Osteomyelitis Vit K deficiency → bleeding bruising Vit A deficiency~»Night Blindness IXanthelisma and Xanthomas arc due to elevation of serum lipids.Lab finding Increased Serum alkaline phosphates.Positive mitochondrial antibody sensitive and specific.