A 63-year-old malewas incidentally diagnosed with liver cirrhosis and splenomegaly in another hospital 1 week before he was admitted to our center in Riyadh for further investigation and management. In addition, the patient complained of a painful red right eye and vision loss for 10 days before admission.
On clinical examination, the patient was jaundiced with stable vital signs. An ultrasound of the abdomen showed a cirrhotic liver with portal hypertension. Magnetic resonance cholangiopancreatography (MRCP)showed an uncomplicated stone with no dilatation in the common bile duct or intrahepatic duct. His FibroScan score was F4 with Child A status. Initially, there were no signs suggestive of decompensated liver failure. History of alcohol intake, ingested herbs, and hepatotoxic medications was all negative. His brother had previously received liver transplant.
He presented with a visual acuity of 20/60 in the left eye and light perception in the right eye. Intraocular pressure at presentation was 22 mmHg for the right eye and 14 mmHg for the left eye. Extraocular muscles were full. Ophthalmic examination of the right eye showed severe conjunctival congestion, shallow anterior chamber with occasional cells, central corneal haze, mature lens cataract with posterior synechiae, and mitotic nonreactive pupil. B-scan was suggestive of severe vitritis [Figure 1].
Initially, the patient was started on a topical steroid and mydriatic agents, which partially relieved his eye discomfort. Ten days after, the patient developed severe nongranulomatous bilateral panuveitis that further involved the left eye. Fundus photography and fluorescein angiography of the left eye showed peripapillary arteritis with a single Roth spot temporal to the macula. Fundus examination was not applicable for the right eye. Since infection, particularly endogenous endophthalmitis, could not be ruled out initially, intravitreal vancomycin, ceftazidime, and dexamethasone with intravenous acyclovir were administered.
Full uveitis and rheumatology workups were performed to rule out differential diagnoses, such as giant cell arteritis, polyarteritis nodosa, systemic lupus erythematosus (SLE), sarcoidosis, lymphoproliferative disease, masquerade syndrome, or infectious causes. Vitreous sampling revealed a clear yellow fluid, which came back negative for organism culture, PCR, and tumor markers. Cytology of the vitreous fluid was suggestive of a chronic inflammatory process, as pus and neutrophils were not evident. Cultures from blood, synovial fluid, and urine were all negative. There was no previous history suggestive of IBD or connective tissue disease. Chest computed tomography was negative for sarcoidosis features. In addition, the patient denied any previous history of oral or genitalia ulcers.
Blood tests were unremarkable except for elevated ESR (130 mm/h) and CRP (150 mg/dL), aspartate transaminase 83 IU/L, alanine aminotransferase 59 IU/L, gamma-glutamyl transpeptidase 384 IU/L, alkaline phosphatase 359 IU/L, total bilirubin 20 umol/L, and conjugated bilirubin 14 umol. Infectious causes such as tuberculosis, herpes simplex virus, viral hepatitis, brucellosis, and HIV were ruled out with serological tests. Rheumatoid factor, cytoplasmic antineutrophil cytoplasmic antibodies, perinuclear antineutrophil cytoplasmic antibodies, antimitochondrial antibodies, and liver-kidney microsome type 1 antibodies were all negative. Serological tests were remarkable for antinuclear antibodies of indirect immunofluorescence assay at a titer of 1:40, anti-smooth muscle antibodies at a titer of 1:320, HLA B51 B52, and high IgG 2260 mg/dL and IgA 648 mg/dL.
After ruling out infectious causes, autoimmunity etiology was suggested. Therefore, the patient was started on 40 mg oral prednisolone with intravitreal dexamethasone injection while fortified antibiotics were stopped. The patient then developed a prostatic abscess with sepsis, which was managed accordingly. As a consequence, oral steroids were temporarily stopped, and a liver biopsy was delayed until clinical stability had been achieved. As the biochemical evaluation was suggestive of AIH, a liver biopsy was performed and revealed lymphoplasmacytic infiltrate with moderate interface hepatitis.
After ophthalmology reassessment and biopsy-proven AIH, oral prednisolone dose was increased to 60 mg, and azathioprine 50 mg was added to the regimen. One week after initiating the treatment regimen, visual acuity of the left eye improved and became 20/30, while the right eye remained LP. B-scan showed a shrunken right eye with resolved inflammation, and a choroidal detachment was noticed. The patient was discharged after agreeing to taper oral prednisolone and gradually increase azathioprine to 75 mg.
After 4 months, the patient presented with a decompensated liver failure and was labeled as Child B status. A diagnosis of AIH was considered definite [Table 1]. After 6 months of managing the patient's comorbidities, a liver transplant from a living donor was successful, and the patient remained stable for 1 year following the transplant. Ophthalmic follow-up was unremarkable, but his right eye appeared to be irreversibly blind [Figure 2].