A 28-year-old woman was admitted to our hospital due to recurrent abdominal pain, low-grade fever, and icterus. The abdominal pain was from 1 year ago, and it was intermittent without clear reason after outpatient work up. She had weight loss and pruritus as well. She did not mention cough, chest pain, or shortness of breath. She did not have other associated symptoms.
In habitual history, she denied using alcohol or tobacco. Family history was negative, and there were no such symptoms in other members of the family. The patient appeared ill on physical examination with a temperature of 38.2°C, blood pressure of 112/75 mm Hg, pulse rate of 92 beats per minute, and respiratory rate of 17 per minute.
Icteric conjunctiva was prominent. Pupils were equally reactive to light and accommodation. Auscultation of the chest and cardiac was normal. Abdominal exam showed tenderness in the right upper quadrant and right lower quadrant. Other examinations were normal. The results of the patient’s laboratory tests are shown in
Table 1. Chest radiograph was normal, and tuberculin (purified protein derivative) skin test was negative at 2 mm induration.
Table 1. Results of Laboratory Tests in Our Patient
Parameters Values White blood cell count 16000 Differential (%) Granulocytes 45 Lymphocytes 19 Monocytes 5 Eosinophils 31 Hemoglobin (g/dL) 10.9 Hematocrit (%) 84 Platelet count (×10 3 /μL) 317000 Erythrocyte sedimentation rate (mm/h) 100 C protein reactant 75 Amylase 20 Liver Function Tests Total bilirubin (mg/dL) 1.2 Aspartate aminotransferase (U/L) 39 Alanine aminotransferase (U/L) 14 Alkaline phosphatase (U/L) 1056 Gamma-glutamyl transferase 43 Viral markers negative S/E NL Anti-smooth muscle antibody NL Antimitochondrial antibodies NL Antinuclear antibody NL
On ultrasound evaluation the size of the liver was 158 mm with mild heterogeneous echo pattern and diffuse internal hepatic duct dilatation with common bile duct (CBD) measuring 10 mm. One hypoechoic focal lesion measuring 12 × 7 mm in the IV
th segment of the liver was also reported.
Magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography (MRCP) elicited increased wall thickness in one-third of the proximal CBD and common hepatic duct, as well as cystic duct extending to intrahepatic main ducts and gall bladder neck, which caused bifurcation in the hepatic duct, right and left ducts, in particular the right proximal duct. Increased wall thickness of the gall bladder neck was observed. Brief dilatation in intrahepatic bile ducts was also noted.
Endoscopy and colonoscopy were performed, which showed normal findings. Biopsy of the abdominal lymph node revealed chronic granulomatous inflammation with high eosinophilia, which was negative for acid-fast bacilli. Bone marrow flow cytometer showed 2% immature myeloid cells without evidence of leukemia or lymphoma.
According to MRCP and IgG4 and high eosinophil with impression of autoimmune cholangiopathy, corticosteroid was administered. A few weeks later, the patient’s symptoms deteriorated, with fever and leukocytosis and the creation of multiple lymph nodes in the neck and abdomen, thus, she were hospitalized again.
Ultrasound evaluation of the abdomen revealed common bile duct with a diameter of 11 mm, which had intraluminal echoes. Intrahepatic bile duct was dilated locally. Gall bladder wall was thickened (4 mm). A necrotizing lymph node measuring about 21 × 22 mm near gall bladder fundus was observed, which had mass effect. Multiple lymph nodes in porta hepatis, para aortic, peripancreatic, and around common iliac vessels were noted, most of which were necrotizing. Multiple hypoechoic lesions were seen in the liver.
Abdominal computed tomography (CT) confirmed these data (
Figure 1). Also, endoscopic retrograde cholangiopancreatography (ERCP) was performed for the patient, but it was normal and could not help with neither definite diagnosis nor treatment. Biopsy of the cervical lymph node was carried out, the results of which revealed accumulation of histiocytes, eosinophils, and polymorphonuclear leukocytes (PMN), therefore, it was not diagnostic.
Figure 1. Multiple hypoechoic lesions in the liver
Smear, culture, and polymerase chain reaction (PCR) for tuberculosis were negative and branching filament suggested
Nocardia infection. According to negative acid-fast bacillus and ruling out malignancies (lymphoma) and because of eosinophilia and hypointense masses with the possibility of parasitic causes ( Fasciola hepatica) triclabendazole was started for the patient. The symptoms briefly improved and she had little weight gain, but four months later she was hospitalized with pulmonary symptoms such as cough and shortness of breath. Chest CT was performed and miliary pattern was observed ( Figure 2). Culture and sputum smear were positive for acid-fast bacilli. Anti-tuberculosis treatment was started with the standard four-drug regimen (i.e., isoniazid, rifampin, pyrazinamide, and ethambutol). Generally, the patient recovered completely after two months, and four months after starting treatment, the patient got pregnant.
Figure 2. Miliary pattern of tuberculosis in chest computed tomography