Endogenous Klebsiella endophthalmitis as the presentation of both Klebsiella liver abscess and underlying anti-IFN-3 autoimmunity.

This case study is one of the first ever reported examples of infection in a patient with anti-IFN-3 autoimmunity and demonstrates how overwhelming infection can sometimes present with visual symptoms. We report the case of a previously fit middle-aged patient presenting with painless loss of vision and loss of appetite. Examination showed choroidal abscess and a pan-uveitis, leading to admission for vitreous biopsy. Klebsiella pneumoniae was isolated both in the vitreous and in blood cultures. Subsequent investigation discovered a liver abscess which was treated with percutaneous drainage. Despite the administration of intravitreal antibiotics from the time of presentation, intravenous antibiotics and vitrectomy on the same day, the patient proceeded to need enucleation 19 days later, and now has only light perception in the remaining eye. The strong association between K. pneumoniae endophthalmitis and underlying liver abscess leads to a significant mortality rate. Early diagnosis is essential, with prompt aggressive treatment with antibiotics, but sadly the visual prognosis remains poor. In cases of suspected choroidal abscess, initiation of sepsis screen and immediate empirical treatment is vital to improve this prognosis. This patient had no significant past medical history, no known immunocompromise, was not diabetic and had no recent significant foreign travel. However, further immunological analysis demonstrated the presence of anti-IFN-3 antibodies, a hitherto under-reported potential cause of increased susceptibility to infection, and so cases of sepsis in previously healthy individuals should be considered for further immunology assessment.

Small cell carcinoma presenting as ocular paraneoplastic syndrome due to CRMP-5.

We report the case of a lady who presented with 3 weeks of visual floaters and optic disc swelling. Subsequent investigations revealed deep white matter changes on brain imaging, and enlarged mediastinal nodes. The presence of anti-CRMP-5 antibodies finally led to the diagnosis of a paraneoplastic syndrome, and mediastinal lymph node biopsy confirmed the diagnosis of small-cell lung cancer. The learning points from this case include that optic neuritis can be the only presenting feature of a paraneoplastic neurological syndrome, and the usefulness of anti-neuronal antibody measurement as a diagnostic marker of an underlying paraneoplastic disease process. The great challenge is to recognise these tumour-associated autoimmune system presentations early, as they often appear long before the primary cancer is evident. Prompt treatment leads to an earlier reduction in circulating auto-antibody possibly due to reduction in tumour size, and thus less likelihood of permanent neuronal damage.

Non-Infectious Uveitis: Optimising the Therapeutic Response.

Non-infectious uveitis mainly affects the working-age population and can contribute to significant social and economic burden. It comprises a heterogeneous group of conditions with varied aetiology. Precise and early diagnosis, excluding masquerade syndromes, is the key to early therapeutic intervention. Treatment should be appropriately selected according to the anatomical sites of inflammation, the diagnosis and known prognosis, and whether there is a systemic inflammatory drive. Corticosteroids in the form of local or systemic therapy form the mainstay of treatment; however, due to unacceptable side effects, the need for long-term use or suboptimal response, corticosteroid-sparing medications may need to be considered early on in the management of non-infectious uveitis. With newer insights into the immunopathology of uveitis and the availability of biologic agents, treatment can be tailored according to individual needs. Many patients have systemic involvement, and hence a multidisciplinary approach is often required to achieve the best outcome in an individual. Patient involvement in the management of non-infectious uveitis, ensuring compliance, and continual monitoring of both the treatment and therapeutic response are the key to achieving optimal outcomes.

Overview and recent developments in the medical management of paediatric uveitis.

INTRODUCTION: Although rarer than its adult counterpart, non-infectious uveitis remains a significant cause of ocular morbidity in children. Owing to the chronicity of the disorder and when refractory to first-line treatment, namely corticosteroids, systemic immunosuppressive treatment may be required to control the disease. AREAS COVERED: Following a literature search using the keywords 'paediatric uveitis', 'juvenile idiopathic arthritis-associated uveitis', 'immunosuppression' and 'treatment', we reviewed the range and effectiveness of treatments employed in the management of non-infectious, paediatric uveitis. EXPERT OPINION: Corticosteroids (topical, periocular, intraocular or systemic) remain the initial drug of choice in ameliorating the signs and symptoms of non-infectious paediatric uveitis. Failure to control the disease and/or failure to reduce the oral dose of prednisolone at least 0.15 mg/kg within 4 weeks often requires additional immunosuppressant therapy. Methotrexate and azathioprine have shown to be effective in the management of juvenile idiopathic arthritis (JIA)-associated uveitis with the former considered the first-line corticosteroid-sparing agent. Biologic therapies are increasingly used earlier in the disease with investigators in the UK currently recruiting patients for the SYCAMORE trial evaluating the efficacy of methotrexate and adalimumab vs methotrexate alone for the treatment for JIA-associated uveitis. Until further randomised controlled trials are conducted, the use of other biologic agents should only be used with an appreciation that there are potentially unknown side-effects and that there is not a full knowledge of their efficacy.