Deep cholestatic jaundice and pulmonary hypertension in a woman with Graves' hyperthyroidism
Authors: Liou M-J , Chen K-W , Huang M-J , Sun J-H , Chen N-H 
Published: May 2014

A 28-year-old woman, gravida 0, parity 0, had hyperthyroidism 6 years ago and had been treated with antithyroid drugs for 3 years. She discontinued the drugs when she returned to the euthyroid state. Her hyperthyroid symptoms recurred a few months after she withdrew the medication and she received Chinese herbal medicine as the only treatment. Progressive generalized oedema, severe dyspnoea, orthopnoea and deep-coloured urine were noted about 4 months before admission. Secondary amenorrhoea for 3 months and gynaecological ultrasound revealed ascites only. Family history revealed that her grandmother, mother and younger sister had hyperthyroidism as well and her sister has also thalassaemia. Physical examination on admission revealed a critically-ill woman with severe orthopnoea, generalized oedema, hand tremor and icterus. Her weight was 61.5 kg, height 160 cm, blood pressure was 120/90 mmHg, body temperature was 37.6°C, and pulse rate was 145 beats per minute (sinus tachycardia). She was alert. Haemotological investigations revealed microcytic anaemia and her haemoglobin (Hb) was 4.8 mmol/litre. The serum total bilirubin was 478.8 mmol/litre with direct bilirubin 224.0 mmol/litre, aspartate aminotransferase 32 u/litre, alanine aminotransferase 70 u/litre, γ-glutamyl transferase 5 u/litre and alkaline phosphatase 83 u/litre. The prothrombin time was normal and serum albumin level was 32 g/litre. Hb electrophoresis indicated α thalassaemia. Serological markers for hepatitis A, B and C were absent. High titre of antinuclear antibody (1:160, homogenous) and CA- 125 (1075.4 U/ml) were noted, but the D-coombs’ test was negative, as were tests for C3, C4, antismooth muscle antibody, antimitochondrial antibody, antidouble strand antibody and lupus anticoagulant. Cytological examination of ascites and pleural effusion indicated transudate with lymphocyte predominance. The total thyroxine level was high at 271.3 nmol/litre and the thyroid-stimulating hormone level measured by sensitive radioimmunoassay was depressed at less than 0.03 mU/litre. Although serum microsomal antibody titres were negative, thyroid binding inhibitory immunoglobulin was positive (64.8%). Chest X-ray revealed massive pleural effusion on the right side and moderate effusion on the left side (Figure 1). Abdominal ultrasound showed normal liver size, non-cirrhotic liver with moderate ascites, and bilateral pleural effusion. Gynaecological ultrasound revealed moderate ascites, but could detect no tumour. A two-dimensional echocardiogram reported severe tricuspid regurgitation, moderate pulmonary hypertension and cor pulmonale. A diffusion lung capacity test was positive. The patient was thought to have primary hyperthyroidism with pulmonary hypertension and cholestatic jaundice, and was treated with furosemide 40 mg/day, polythiouracil 300 mg/day and propanolol 40 mg/day. Three weeks later, the serum total bilirubin and CA-125 had decreased to 44.5 mmol/litre and 29.6 U/ml respectively. She loss 10 kg in weight and the generalized oedema subsided. The patient’s condition improved and laboratory tests yielded normal results. Figure 2 shows the serial serum bilirubin results.