A 32-year-old woman who has been suffering from cutaneous lesions in the malar region and base of the neck for three years, oral dryness, bilateral intermittent parotid swelling and arthralgias with small asthenia feet
She was referred by a primary care physician with suspected systemic lupus erythematosus and/or Sjögren's syndrome in November 1999.
In a previous study in another hospital, an analysis was performed, highlighting antinuclear antibodies 1 and sialography reported as chronic parotitis and ductal system destructuring.
Personal history: bronchial asthma up to the age of 8 years, cryptictomized at 10 years and spontaneous abortion in 1998.
He did not smoke and alcohol intake was reduced to 10-20 g occasionally.
Occupational: administrative.
Family history was uninterested.
Physical examination: overweight with a BMI of 27 and an AT of 170/110, mild parotid hypertrophy, without skin lesions or articular inflammatory signs; the rest of the physical examination was normal.
The hemogram showed: 4, iliac, 14.6 Hb, 41% Hto, 97 MCV, 35 MCH, 408,000 platelets.
Biochemistry: triglycerides 1687 figure with subsequent decrease until November 2000 and sudden increase in May 2001; cholesterol 269 (VLDL 62% and HDL/LDL 19).PTG 74, GOT 50, GGT 400.
Proteinogram: PT 5.5, albumin 69%, gamma subunit 9%.
Immunoelectrophoresis: IgG 419.
Anti-LKM, ANA, antimitochondrial and antithyroid antibodies were negative.
Chest X-ray was normal, abdominal ultrasound showed normal size and morphology, homogeneous with no LOES and strong hyperechogenicity and bulging liver suggesting steatosis.
Biopsy of minor salivary glands of the lower lip without evaluable lesions.
Liver biopsy with marked echography: fibrosis staring at the portal spaces that sometimes joins each other without producing regeneration nodules, fibrosis dissects the sinusoids from the interstitial lobule, reaching the interstitial pattern.
Lymphocyte without clear infiltrates, intense steatosis predominantly macrovesicular affecting more than 70% of hepatocytes.
Isolated necrosis foci with polymorphous exudate and occasionally Mallory's hyalin without evidence of ferric pigment or inflammatory stasis.
1.
Equidae tendinium fixed mcg/g atomic absorption graphite: iron in hepatic tissue 1,167/g dry weight, not compatible with chromatosis.
Genetic testing for C282Y and H63D mutations affecting the HFE gene was negative.
One year after the onset of symptoms, the patient has a rapid weight loss of about 10-12 kg, malaise, and exacerbation of asthenia.
Physical examination revealed giant and painful hepatomegaly and the analyses showed a GOT/GPT ratio greater than 3 when the previous visit did not exceed 1.3, which can be interpreted as progression to liver fibrosis (11).
He had leukocytosis with left shift of white series and anemia, persisted macrocytosis and hyperlipidemia with TG above 1,000 IU/dl.
Abdominal CT: large hepatomegaly with image suggestive of fat mass.
There are no LOES or dilatation of intra or extrahepatic biliary radicals, normal gallbladder, or other abdominal structures.
Ursodeoxycholic acid and vitamin E treatment was started along with their usual therapy for hypertension and hyperlipidemia.
From then on, she was followed up in consultation with no clear clinical improvement and waiting for liver transplantation.
In November 2003, she was again admitted with worsening of her general condition, ascites and lower limb edema.
Abdominal ultrasound showed abundant ascitic fluid, large hepatomegaly without space occupying lesions, recanalization of the paraumbilical vein and collaterals in the abdominal wall.
A paracentesis was performed with evacuation of ten.
Liver function remained unchanged compared to the last controls and showed no signs of infection.
Hydrapic arthritis was ruled out and the patient was discharged with good general condition.
