DISCUSSION
AIH is characterized by an immune reaction directed towards liver tissue with autoantibodies being the culprit that not only commence, but also sustain the harm [2]. Clinically, it can have a variable presentation. There can be silent cases with no symptoms, but also there can be patients with significant symptoms related to hepatitis, which can rarely lead to acute liver failure as well.
AIH type 1, also known as Classical AIH, is known for the presence of ANA and/or smooth muscle autoantibodies (AMSA) and autoantibodies against actin and atypical perinuclear anti neutrophilic cytoplasmic antibodies (p-ANCA). Type 2 AIH shows the occurrence of specific antibodies which are targeted against liver and kidney microsomal antigens (anti-LKM Ab) and/or liver cytosol type 1 antibody (ALC-1). For making a diagnosis, we need to rule out extrinsic causes of hepatitis such as drugs, alcohol, and viruses. Also, there should be satisfying inclusive criteria such as hyperglobulinemia, the presence of autoantibodies, and characteristic histologic features including interface hepatitis, plasma cells and rosettes. In about 70-80% of AIH cases, both ANA and ASMA can be found. Only in approximately 10% of patients of AIH, there are no circulating autoantibodies. Apart from a negative panel of autoantibodies, patients with SAIH are very similar to those of classical AIH in terms of their demographic, biochemical, and histological features. That is why they can be treated very successfully like classical AIH with corticosteroids. Also, they have similar prognosis after steroid therapy. SAIH has a favorable prognosis for patients who respond to
treatment. Most of the patients who receive appropriate treatment undergo remission, and the 10-year survival rate has approached from 83.8% to 94%. MIn the majority of cases we need to institute a maintenance therapy for their entire life, since discontinuation of the treatment can cause a reappearance of the disease in about 80% patients out of 100, over a term of 3 years.
In the past, there has been a case of seronegative autoimmune hepatitis reported by J.M. Sherigar et al. where a 58 y/o female patient came to the emergency department due to pain in her epigastrium along with nausea and vomiting, all the symptoms being there only for a day. That being an acute presentation of Seronegative autoimmune hepatitis is different from our patient who had chronic manifestations of fever and yellowish discoloration for 2 months. Also, in their case, the patient underwent a core liver biopsy which revealed that the portal tracts were penetrated with lymphocytes, plasma cells, and eosinophils. It also showed a severe grade 4 circumferential interface hepatitis, and steroid therapy was started after biopsy findings were confirmed, while in our case biopsy could not be performed as the patient was too unstable, so steroid therapy was started early based on clinical suspicion, showing how sometimes we have to start the therapy even in the absence of definitive diagnosis.