Tubulointerstitial nephritis associated with primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is an immunemediated chronic cholestatic liver disease. The etiology is unknown, although it is presumed to be autoimmune in natural (1). PBC is most frequently a disease of women and occurs between the fourth and sixth decades of life. The symptoms may strongly affect patients’ quality of life and may induce incapacitation (2). The major pathology of this disease is a destruction of the small to median bile ducts, with leads to progressive cholestasis and often end-stage liver disease (3). Anti-mitochondrial antibody (AMA) can be found in 90–95% of patients with PBC, and they have a specificity of 98% for this disease. Biochemical pattern reveals cholestasis (elevation of alkaline phosphatase and γ-glutamyl-transferase; γ-GT), and the liver biopsy findings are compatible with bile duct injury, cholestasis and granulomas (4). Extra-hepatic associated disorders arise in 70% of patients with PBC (5). Tubulointerstitial nephritis is a primary injury to interstitial area and renal tubules, resulting in decreased renal function (6). The acute from is most often due to allergic drug reaction or to infection. The chronic from occurs with a diverse array of causes, including genetic or metabolic disorders, obstructive uropathy and chronic exposure to environmental toxins (7). Diagnosis is suggested by history and urinalysis and often confirmed by biopsy. In this paper, we report a patient with this rare association of tubulointerstitial nephritis (TIN) and PBC. Furthermore we evaluated clinicopathological features and outcomes in this patient. A 28–year–old woman was admitted to our hospital with abdominal pain and jaundice. Pain has identified in right upper quadrant of abdomen and was progressive. Abdominal pain has continued since one year ago until now. There was excessive sweating, itching and anorexia. Patient had no drug history consumption, blood injection, using infected water or food in two years ago. On clinical examination blood pressure was normal. A one plus edema of the lower limbs was found and tenderness of right upper quadrant was detected. Cardiovascular examination and echocardiography were normal. Doppler sonography of the abdominal and lower limb vessels was normal, a non-obstructing renal stone in kidney was detected. Sonography showed upper normal limit of liver and some degree of liver coarseness. Gallbladder volume, echo and wall were normal and there was not any obstruction. Pancreas, spleen and biliary tract were normal. Kidneys showed mild decrease in size with increasing echotexture. Computed tomography of the abdomen showed length of right kidney and left was 9 cm and 8 cm respectively. Endoscopy of stomach Mohammad-Hosein Rasolzadegan1, Hanie Bakhshayesh2*, Nakisa Amid2