Purpose: To report a case of non-paraneoplastic Autoimmune Retinopathy (npAIR) treated with intravenous immunoglobulin (IVIG). Case report: A 12-year-old boy presented with progressive visual field loss, nyctalopia, and flashing for three months. He had suffered from common cold two weeks before the onset of these symptoms. On the basis of clinical history and paraclinical findings, he was diagnosed with npAIR, and IVIG without immunosuppressive therapy was started. During the one-year follow-up period after the first course of IVIG, flashing disappeared completely. Visual acuity remained 10/10, but nyctalopia did not improve. Multimodal imaging showed no disease progression. Conclusion: Although established retinal degenerative changes seem irreversible in npAIR, IVIG may be a suitable choice to control the disease progression.

Autoimmune hepatitis (AIH) is one of causes of chronic liver diseases. It is an unresolving inflammation of liver tissue and characterized by elevated transaminases, hypergammaglobulinemia, and circulating autoantibodies. The disorder occurs mostly in females (F:M ratio is 3.6 to 1) and is a relatively uncommon disorder with point prevalence of 8-16.8 per 100 000 population in western countries. Hiostologic hallmarks are interface hepatitis (also called piecemeal necrosis), and portallymphoplasmacytic infiltration.Dignostic criteria are based on excluding other etiologies of chronic liver disease,such as viral hepatic (A, B, C), metabolic disorders eg Wilson disese,drug induced hepatitis and alcoholic liver disease. Conventional autoantibodies are Antinuclear antibody (ANA), Smooth muscle antibody (SMA) and Anti liver kidney microsomal 1 (Anti LKM1).Some cases have combined clinical, laboratory or histologic features of Primary Biliary Cirrhosis (PBC) or Primary Sclerosing Cholangitis (PSC) with AIH and are known as overlap syndrome. Standard treatments of AIH as the most successful treated form of chronic hepatitis are based on immunosuppression with corticosteroids alone or in combination with azathioprine.

Purpose: To study whether rituximab and bortezomib combination therapy is more effective than rituximab monotherapy in the treatment of nonparaneoplastic Autoimmune Retinopathy (npAIR). Methods: Retrospective case series involving six patients with npAIR, taking either rituximab and bortezomib combination therapy (three cases) or rituximab monotherapy (one case and two historical patients). Results: Patients on both treatment regimens showed stability in most of the visual function parameters during the one year of follow-up. Combination therapy resulted in improvement of scotopic combined rod and cone a-wave and b-wave amplitudes in all eyes where they were available (four eyes),however, rituximab monotherapy resulted in only two eyes with stable scotopic combined rod and cone a-wave and b-wave amplitudes, while four eyes showed a decrease in both a-and b-wave amplitudes. The average improvement in b-wave amplitude (50. 7% ± 29. 4% [range 25–90%]) was higher than the average improvement in awave amplitude (35. 7% ± 9. 74 [range 25–63%]). No severe adverse effects were reported. Conclusion: Rituximab and bortezomib combination therapy may not be more effective than rituximab monotherapy in npAIR patients for most of the visual function parameters,however, this combination therapy may be more effective in improving scotopic combined rod and cone a-and b-wave amplitudes. This may indicate the higher efficacy of combination therapy when there is involvement of the inner retina.

Background: Advanced hepatic fibrosis and cirrhosis has generally been considered to be irreversible. The aim of this study was to determine whether cirrhosis is reversible.Methods: Seven patients with Autoimmune hepatitis with histologic evidence of cirrhosis were enrolled. After treatment, they had follow-up liver biopsy while in clinical and biochemical remission. Biopsy specimens were randomly coded in unpaired manner according to patient and were read independently by two pathologists using the modified hepatitis activity index (with a maximum stage of 6).Results: Three patients still had extensive fibrosis in the second liver biopsy. But four patients had almost total regression of their liver fibrosis. In the latter patients, the mean alanine aminotransferase level decreased from 776.3 U/L to 23 U/L. The mean bilirubin level decreased from 5.85 mg/dL, to 0.98 mg/dL. Extensive fibrosis or cirrhosis were present in all patients at diagnosis but were not present on follow-up liver biopsy. The mean fibrosis score decreased from 5.88 to 0.5 (P=0.0002), and the mean grading score from 11.38 to 2.5 (P=0.0008.).Conclusion: Frank cirrhosis due to Autoimmune hepatitis may be reversible in some patients who respond to treatment.

Background & Purpose: Acute compressive injuries to the head and chest, visual loss with a special form of Retinopathy called Purtscher Retinopathy may be followed by this Retinopathy is characterized by cotton-wool spots, hemorrhages and retinal edema around the optic disc in one or both eyes. A case of unilateral Purtscher-Like Retinopathy is introduced after blunt trauma to one eye without compression. Patients & Findings: A 16-year-old boy came with history of blunt trauma to his right eye and decreased vision since 2 days prior to presentation. Visual acuity was counting fingers at 1 m in the right eye and 10/10 in the left. Except for chemosis and subjconjunctival hemorrhage no other finding was seen on slit lamp biomicroscopy. Conclusion: According to clinical manifestations, changes like Purtscher Retinopathy seems to occur not only after compressive head and chest injuries, but also following direct injuries to the eye.  

Purpose: To report a case of purtscher Retinopathy associated with valsalva Retinopathy after car accident.Methods: A 54-year-old man presented with a sudden visual loss in his left eye after car accident. Fundus examination of the left eye showed cotton-wool spots and retinal hemorrhages in the posterior pole associated with subhyaloid hemorrhage in macula.Results: Fundus examination showed typical findings for both purtscher Retinopathy and valsalva Retinopathy.Conclusion: These findings suggest the purtscher Retinopathy features has occurred as a result of valsalva maneuver in our case.