A 46-year-old white woman was referred to our physicians for symptom related to biliary tract injury (pruritus, abdominal pain, and fatigue), without jaundice. With a history of smoking, no other clinical information has been noted to this patient.
Baseline laboratory data obtained from blood sampling revealed alkaline phosphatase (ALP) elevation together with g-glutamyl transferase (gGT), aspartate aminotransferase (AST) and alanine aminotransferase (ALT). Bilirubin level was normal (Table 1).
The differential diagnosis will go through the evidence of specific etiological agent that cause disease. For this reason, we take into consideration three clinical hypothesis: viral, pharmacological and autoimmune aetiology. Viral serologies, summarized in table 1, was considered exhaustive to exclude viral aetiology. 
Many drugs can cause cholestasis or cholestatic hepatitis, including sulfamethoxazole/ trimethoprim, amoxicillin/clavulanate, antiepileptic medication, and antituberculosis drugs. Histologic findings are variable but usually include mild portal inflammation, ductular reaction, and cholestasis. Clinical history is important, because a patient's medication list should be reviewed for potentially offending substances, and the length of symptoms is important as well. 
Patient not referred the use of these and others pharmacological therapy, included "homemade" herbal substances. 
 Immunological evidences  
A two distinct cytoplasmic pattern were identified in a routine ANA test using HEp-2 cell slides from Euroimmun, at end dilution of 1:1280 (Figure 1, table 2).
 Mitochondrial-like cytoplasmic pattern  
IIF- pattern were characterized by the presence of larger irregular granules extending from the nucleus throughout the cytoplasm in a reticular network. Cytoplasm of dividing cells was strongly positive (Figure 2, Panel A). IIF on a commercial rat liver, kidney and stomach tissue with the use of polyclonal IgG antibodies confirmed the presence of AMAs, with a characteristic staining pattern: granular diffuse cytoplasmic staining of the Kupffer cells and hepatocytes, of the renal tubules (strongest staining is noted in distal which is mitochondria-rich) and parietal gastric cells (Figure 2, panel B). Line immunoassay Euroline profile autoimmune liver diseases (IgG; LIA, Euroimmun Lubeck) revealed antibodies against AMA-M2. M2-E3 (BPO) but not against Sp100, PML, gp210, LKM-1, LC-1 and SLA/LP (Figure 2, panel C).
 Rods and rings cytoplasmic pattern  
The structures recognized by patient serum were distinct cytoplasmic rods and rings (figure 1 and 3). We observed one to two rods and/or rings per cell including some intermediate structures such as a figure ''8'' (Figure 3, B1; B2), elongated rings (Figure 3, A2 ), twisted rings (Figure 3, C2), rods with pin loops (Figure 3). Some rods often align adjacent to the nucleus or perpendicular to the nucleus, and rings may be found in the cytoplasm (Figure 3, A3; B3; C1).
 Histological evidences  
The typical findings of PBC on hematoxylin-eosin stain were appeared, as described below: moderate lymphoplasmacytic portal inflammation with moderate interface hepatitis and bile ductular reaction along the periphery of the portal tracts, typical for early disease stages (Figure 4 panel A and B). 
 Diagnosis 
Following the American Association for the Study of Liver Diseases Guideline, PCB diagnosis was made:
1) Biochemical evidence of cholestasis based on alkaline phosphatase (ALP) elevation; 
2) The presence of anti-mitochondrial antibodies (AMAs);
3) Histological evidence of intrahepatic destructive cholangitis of interlobular bile ducts.