Frosted Branch Angiitis (FBA): A case report

The purpose of the study is to describe a rare case report for raising the awareness of existence of this rare ocular disease and also review the characteristics of this disease. A 35-year-old man presented with acute vision loss in both eye after high grade fever. On fundoscopic examination flower petal pattern blood vessel sheathing in temporal quadrant of both eyes was seen. A provisional diagnosis of frosted branch angiitis was considered. The patient responds promptly with Intravenous and oral corticosteroid. A total of 58 cases have been reported in worldwide literature. The disease is likely to represent a common immune pathway in response to multiple infective agents. CBMJ 2020 July: Vol. 09 No. 02 P: 42-44

Frosted branch angiitis due to cytomegalovirus-associated unmasking immune reconstitution inflammatory syndrome: a case report and literature review

Background Cytomegalovirus (CMV) retinitis is a common opportunistic infection in patients with acquired immunodeficiency syndrome. The common funduscopic manifestations are haemorrhagic necrotising variety and granular variety. Frosted branch angiitis (FBA), as a special form, when it occurred after antiretroviral therapy(ART), could possibly be associated with immune reconstitution. We report a case of FBA secondary to CMV infection-associated unmasking immune reconstitution inflammatory syndrome (IRIS). Case presentation A 27-year-old man with human immunodeficiency virus infection developed FBA after 35 days of ART. The left Aqueous humour (AqH) tested positive for CMV DNA, and the patient was diagnosed with CMV retinitis. The degree of intraocular inflammation was reflected by increased levels of interleukin (IL)-6 and IL-8 in AqH. After anti-CMV treatment and continuous ART for several months, his FBA and vision significantly improved. CMV DNA became undetectable in the left AqH, and the IL-6 and IL-8 levels in AqH decreased. Conclusion FBA could be a sign of CMV-associated unmasking IRIS. Anti-CMV treatment alone or combination with steroid treatment may be administered, depending on the changes in CMV DNA load and immunologic profile of AqH.

Retinal Vasculitis Associated With Dermatomyositis Treated With Intravenous Steroids

Purpose: This work aims to present treatment and long-term follow-up of a 31-year-old woman with dermatomyositis who presented with hemorrhagic retinal vasculitis and macular edema. Methods: A retrospective case report is presented. Results: A 31-year-old woman with dermatomyositis treated with systemic immunosuppression was evaluated for acute, reduced vision. Best-corrected visual acuity was hand motion in the right eye and 20/200 in the left eye. Fundus examination revealed diffuse intraretinal hemorrhages, cotton-wool spots, and vascular sheathing with a frosted branch angiitis–like appearance. Optical coherence tomography revealed significant macular edema and subretinal fluid that quickly resolved after admission and treatment with intravenous steroids. Multimodal imaging at 7-year follow-up disclosed long-term sequelae including peripheral nonperfusion and retinal neovascularization. Conclusions: When vasculitis associated with dermatomyositis is treated aggressively with intravenous steroids at initial presentation, good visual acuity outcomes can be achieved, but long-term consequences of retinal nonperfusion and neovascularization persist.

A rare case of Polyarteritis Nodosa associated with autoimmune hepatitis: a case report

Background Polyarteritis nodosa is a type of vasculitis affecting medium- and small-sized arteries that has been associated with hepatitis B but does not have an established relationship with autoimmune hepatitis. Here we report the case of an adult patient with autoimmune hepatitis who, shortly after diagnosis, developed life-threatening polyarteritis nodosa. Case presentation A 45-year-old woman was diagnosed with autoimmune hepatitis after initially presenting with a two-month history of fatigue, nausea, and anorexia and a three-week history of scleral icterus. Her liver biopsy showed mild portal fibrosis and her liver chemistries improved with prednisone and azathioprine. Three months later, she presented to the emergency department with fever, bilateral ankle pain, rash, oral ulcers, and poor vision. Physical examination was notable for erythema nodosum, anterior uveitis, retinal vasculitis, and frosted branch angiitis (frosted branch angiitis (a widespread florid translucent perivascular exudate). She subsequently developed repeated episodes of ischemic acute bowel necrosis that required multiple surgeries and extensive small bowel resections. Surgical pathology of the small bowel resection revealed ischemic necrosis, medium and small vessel vasculitis with microvascular thrombi consistent with polyarteritis nodosa. Azathioprine was discontinued and she was treated with pulse steroids followed by a prednisone taper, cyclophosphamide, and intravenous immune globulin with overall improvement in her symptomatology. Since her hospitalization, she has been maintained on low-dose prednisone and mycophenolate mofetil. Conclusions In patients with recent diagnosis of autoimmune hepatitis, there should be a modest suspicion for concomitant polyarteritis nodosa if symptoms and signs of multisystem vasculitis develop.

Unilateral frosted branch angiitis in an human immunodeficiency virus-infected patient with concurrent COVID-19 infection: a case report

Background Frosted branch angiitis (FBA) is an uncommon ocular sign with multiple causes. With the recent outbreak of coronavirus disease 2019 (COVID-19), many cases of ocular manifestation in association with this disease have been reported. However, as yet we have no complete understanding of this condition. We report here the first case of FBA in a human immunodeficiency virus-infected patient with coexisting cytomegalovirus (CMV) and COVID-19 infection. Case presentation A 33-year-old Malay man with underlying acquired immunodeficiency syndrome receiving highly active antiretroviral therapy was referred to the Opthalmology Department with complaints of blurry vision for the past 2 months. He had tested positive for and been diagnosed with COVID-19 1 month previously. Clinical examination of the fundus revealed extensive perivascular sheathing of both the artery and vein suggestive of FBA in the right eye. Laboratory testing of nasal swabs for COVID-19 polymerase chain reaction (PCR) and serum CMV antibody were positive. The patient was then admitted to the COVID-19 ward and treated with intravenous ganciclovir. Conclusion Clinicians should be aware of and take the necessary standard precautions for possible coexistence of COVID-19 in an immunocompromised patient presenting with blurred vision, eye redness, dry eye and foreign body sensation despite the absence of clinical features suggestive of COVID-19. Whether FBA is one of the ocular signs of co-infection of COVID-19 and CMV remains unknown. Further studies are needed to provide more information on ocular signs presented in patients with concurrent COVID-19 and CMV infections.