P30 Extra-ocular manifestations of children with sarcoid-like uveitis

Abstract Background Paediatric sarcoidosis represents a spectrum of disease. Early onset sarcoidosis & Blau syndrome associated with NOD2 mutations are characterized by fever, rash, arthritis & organomegaly. Later onset sarcoidosis has wider organ involvement (lungs, kidneys, lachrymal & extra-ocular glands). Both presentations may lead to long term complications due to end-stage organ damage. Ocular sarcoidosis has a well described uveitis phenotype. We aim to describe a retrospective cohort of children with sarcoid-like uveitis & their systemic manifestations at time of study; compare cohort of patients fulfilling IWOS criteria for ocular sarcoidosis versus who did not; and describe their management in retrospective cohort. Methods We performed a retrospective case review of all children currently followed at GOSH with ocular sarcoidosis phenotype with uveitis (ophthalmologist definition based on IWOS, or diagnosis of idiopathic uveitis with raised ACE level at least once. We collected demographics & all extra-ocular involvement described in sarcoidosis. Results n = 52; 27/52 males. Median age at onset of uveitis 4.20 years (1.41-15.16). 49/52 bilateral uveitis. 27/52 (50%) <8years age at onset & 2/6 NOD 2 + belonged to this group. Median ACE 68 U/L at presentation (0-90U/L). Median maximum ACE 74 (14-420). NOD2 tested in 12 patients: 6+ , 6- . 1/6 positive patient had Blau phenotype. Ethnicity: African 12/52, asian 11/52, caucasian 14/52, unknown 15/52. Uveitis: Anterior 17/52(32.6%), Anterior+Intermediate 1/52, intermediate 5/52(9.6%) , posterior 2/52, panuveitis 25/52(48%), undocumented 2/52. ANA positive (>1:80) in 15/47 (32%). Systemic involvement (n = 52): arthritis 29%, liver 29%, lymphadenopathy 19%, renal 16%, lungs 15.3%, skin 17.3%, spleen 7.7%, glands 1.9%. Patients as per adult IWOS criteria: Definite 12/52, Presumed 6/52, Probable7/52 and Not fulfilling – 27/52. Systemic involvement in patients not fulfilling IWOS criteria (27/52) – renal 14.8%, arthritis 22.2%, hilar or peripheral lymphadenopathy 0 %, skin involvement 7.4%, lung 18.5%, splenomegaly 3.7%. Comparing IWOS fulfilling (25) with the ones who did not (27) – systemic involvement consistently less common in the ones NOT fulfilling but only reaches statistical significance difference for involvement. Lymphadenopathy and skin (p < 0.001 and p < 0.050 respectively). Suggesting that paediatric age group cannot be classified as per the adult IWOS ocular sarcoidosis criteria and needs early systemic screening. Medications used to treat uveitis and/or extra-ocular manifestations: methotrexate alone 25%, methotrexate + adalimumab 21.1%, mycophenolate mofetil 9.61%, only systemic steroids 3.8%. 9 patients- no systemic medications at any time during their disease. Conclusion Most sarcoid-like uveitis patients had at least one systemic involvement. 51.9% patients did not fulfil the IWOS ocular sarcoidosis criteria, still had systemic involvement. Hilar lymphadenopathy criteria cannot be applied to the paediatric population, peripheral more common. ACE not a sensitive biomarker to predict sarcoidosis. 17.3 % had mild phenotype & required no treatment. This study demonstrates the importance of close monitoring for systemic manifestations & highlights good clinical response to steroids, MTX, MMF and anti-TNF. Conflicts of Interest The authors declare no conflicts of interest.

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Neurological findings in early syphilis: a comparison between HIV positive and negative patients

Neurology International ◽

10.4081/ni.2013.e19 ◽

2013 ◽

Vol 5 (4) ◽

pp. 19 ◽

Cited By ~ 6

Author(s):

Alejandra González-Duarte ◽

Zaira Medina López

Keyword(s):

Venereal Disease ◽

Age At Onset ◽

Disease Onset ◽

Clinical Findings ◽

Hiv Positive ◽

Ocular Involvement ◽

Early Syphilis ◽

Ocular Manifestations ◽

Csf Pleocytosis ◽

Hiv Negative

After a decade of steady decline, syphilis has reemerged within the past few years and it is seeping back into the HIV negative population. We describe herein 16 consecutive cases of neurosyphilis and compare its clinical characteristics. Of the 16 patients, 14 (87%) were men. Mean age at onset was 43 years old (range: 23-82). Twelve patients (75%) were HIV positive; stage was B2 in 2 patients, B3 and C2 in one patient each, and C3 in 8 patients. The clinical presentation was meningitis in 6 (40%), stroke in 3 (18%), ocular manifestations in 4 (27%), and psychiatric manifestations in 2 (13%) cases. Five additional patients had ocular involvement after a formal ophthalmologic examination. High venereal disease research laboratory test (VDRL) titers in serum and cerebrospinal fluid (CSF) were found. Patients in C3 stage of HIV had less CSF pleocytosis (<5 cells/mm3) than patients in earlier stages (P=0.018). Disease onset was earlier in patients older than 50 years old with HIV (P=0.049). We found that meningitis, ocular manifestations and stroke were the most common clinical findings in early syphilis. Moreover, stroke included the carotid and cerebrobasilar vascular territories. CSF VDRL continues to be a crucial test in all idiopathic cases of meningitis, stroke and uveitis, regardless of the HIV status or CSF pleocytosis. Except for less pleocytosis, there were no important differences between HIV positive and HIV negative patients.

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Eye and Rheumatology

Skills in Rheumatology ◽

10.1007/978-981-15-8323-0_19 ◽

2021 ◽

pp. 419-428

Author(s):

Abdullah A Al-ghamdi

Keyword(s):

Systemic Lupus Erythematosus ◽

Juvenile Idiopathic Arthritis ◽

Lupus Erythematosus ◽

Visual Loss ◽

Ocular Involvement ◽

Ocular Manifestation ◽

Systemic Lupus ◽

Systemic Involvement ◽

Rheumatologic Diseases ◽

Ocular Manifestations

AbstractThe ocular involvement in rheumatology can be in a wide variety; it ranges from simple episcleritis to significant visual loss. Early detection followed by appropriate management can reserve vision. Ophthalmic involvement may occur in all of the rheumatic disorders. Ocular manifestation may be a presenting sign in some disorders, as in juvenile idiopathic arthritis (JIA), ankylosing spondylitis (AS), and Sjogren’s syndrome (SjS), or can be a presenting sign with the systemic involvement as in systemic lupus erythematosus (SLE), polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), and systemic sclerosis. Thus ocular manifestations in rheumatologic diseases (Table 19.1) can be the link in approaching the diagnosis.

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Ocular Involvement in Hereditary Amyloidosis

Genes ◽

10.3390/genes12070955 ◽

2021 ◽

Vol 12 (7) ◽

pp. 955

Author(s):

Angelo Maria Minnella ◽

Roberta Rissotto ◽

Elena Antoniazzi ◽

Marco Di Girolamo ◽

Marco Luigetti ◽

...

Keyword(s):

Local Level ◽

Retinal Pigment ◽

Diagnostic Delay ◽

Amyloid Deposition ◽

Retinal Pigment Epithelial Cells ◽

Ocular Involvement ◽

Transthyretin Amyloidosis ◽

Systemic Involvement ◽

Ocular Manifestations ◽

Eye Involvement

The term amyloidosis describes a group of rare diseases caused by protein conformation abnormalities resulting in extracellular deposition and accumulation of insoluble fibrillar aggregates. So far, 36 amyloid precursor proteins have been identified, and each one is responsible for a specific disease entity. Transthyretin amyloidosis (ATTRv) is one of the most common forms of systemic and ocular amyloidosis, due to the deposition of transthyretin (TTR), which is a transport protein mainly synthesized in the liver but also in the retinal pigment epithelial cells. ATTRv amyloidosis may be misdiagnosed with several other conditions, resulting in a significant diagnostic delay. Gelsolin and keratoepithelin are other proteins that, when mutated, are responsible for a systemic amyloid disease with significant ocular manifestations that not infrequently appear before systemic involvement. The main signs of ocular amyloid deposition are in the cornea, irido-corneal angle and vitreous, causing complications related to vasculopathy and neuropathy at the local level. This review aims at describing the main biochemical, histopathological and clinical features of systemic amyloidosis associated with eye involvement, with particular emphasis on the inherited forms. We discuss currently available treatments, focusing on ocular involvement and specific ophthalmologic management and highlighting the importance of a prompt treatment for the potential sight-threatening complications derived from amyloid deposition in ocular tissues.

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The clinical spectrum of ocular bartonellosis: a retrospective study at a tertiary centre in Malaysia

Journal of Ophthalmic Inflammation and Infection ◽

10.1186/s12348-020-00224-0 ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Michele Shi-Ying Tey ◽

Gayathri Govindasamy ◽

Francesca Martina Vendargon

Keyword(s):

Hand Movement ◽

Clinical Findings ◽

Common Finding ◽

Final Visit ◽

Ocular Involvement ◽

Exudative Retinal Detachment ◽

Tertiary Centre ◽

Ocular Manifestations ◽

Ocular Findings ◽

Clinical Records

Abstract Background Cat scratch disease (CSD) is a systemic illness caused by the gram-negative bacillus, Bartonella henselea, which can occasionally involve the ocular structures. The objective of this study is to evaluate the various clinical presentations of ocular bartonellosis at our institution. A retrospective review of the clinical records of 13 patients (23 eyes) with ocular manifestations of Bartonella infections over a 3-year period between January 2016 to December 2018 was undertaken at our institution. Results The diagnosis was made based on clinical findings and in addition, with the support of the evidence of Bartonella hensalae IgG and/or IgM. Small retinal white lesions were the most common ocular findings in this series of patients (82.6% of eyes, 76.9% of patients). Neuroretinitis was the second most common finding (47.8% of eyes, 69.2% of patients), followed by exudative retinal detachment involving the macula (34.8% of eyes, 53.8% of patients) and Parinaud’s oculoglandular syndrome (17.4% of eyes, 23.1% of patients). Other findings like isolated optic disc oedema without macular star (8.7% of eyes, 15.4% of patients) and vitritis (4.3% of eyes, 7.7% of patients) were also observed. Ten patients (76.9%) had bilateral ocular involvement. Most of the patients were young, immunocompetent and had systemic symptoms like fever prior to their ocular symptoms. The visual acuity (VA) at initial presentation ranged from 6/6 to hand movement (mean, 6/20), and at final visit 6/6 to 6/60, (mean, 6/9). 91.7% of patients were treated with antibiotics. Only 2 patients received oral corticosteroids together with antibiotics due to very poor vision on presentation. The visual prognosis of ocular bartonellosis is generally good with 16 (88.9%) of 23 eyes having VA of 6/12 or better at final follow-up visit. Conclusion Small foci of retinal white lesions were the most common manifestation of ocular bartonellosis in this series, followed by neuroretinitis, though an array of other ocular findings may also occur. Therefore, we should consider bartonella infection as a possible differential diagnosis in those patients.

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Current concepts in the diagnosis and management of antiphospholipid syndrome and ocular manifestations

Journal of Ophthalmic Inflammation and Infection ◽

10.1186/s12348-021-00240-8 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Gunay Uludag ◽

Neil Onghanseng ◽

Anh N. T. Tran ◽

Muhammad Hassan ◽

Muhammad Sohail Halim ◽

...

Keyword(s):

Antiphospholipid Syndrome ◽

Antiphospholipid Antibodies ◽

Clinical Manifestations ◽

Autoimmune Disorder ◽

Ocular Involvement ◽

Ocular Manifestations ◽

Different Types ◽

Thrombotic Complications ◽

Specific Factors ◽

Arteries And Veins

AbstractAntiphospholipid syndrome (APS) is an autoimmune disorder associated with obstetrical complications, thrombotic complications involving both arteries and veins, and non-thrombotic manifestations affecting multiple other systems presenting in various clinical forms. Diagnosis requires the presence of antiphospholipid antibodies. The exact pathogenesis of APS is not fully known. However, it has recently been shown that activation of different types of cells by antiphospholipid antibodies plays an important role in thrombosis formation. Ocular involvement is one of the important clinical manifestations of APS and can vary in presentations. Therefore, as an ophthalmologist, it is crucial to be familiar with the ocular findings of APS to prevent further complications that can develop. Furthermore, the ongoing identification of new and specific factors contributing to the pathogenesis of APS may provide new therapeutic options in the management of the disease in the future.

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Ocular manifestations in Herpes Zoster Ophthalmicus

Nepalese Journal of Ophthalmology ◽

10.3126/nepjoph.v3i2.5271 ◽

1970 ◽

Vol 3 (2) ◽

pp. 165-171 ◽

Cited By ~ 16

Author(s):

LR Puri ◽

GB Shrestha ◽

DN Shah ◽

M Chaudhary ◽

A Thakar

Keyword(s):

Herpes Zoster ◽

Young Patients ◽

Ocular Involvement ◽

Post Herpetic Neuralgia ◽

Visual Disability ◽

Herpes Zoster Ophthalmicus ◽

Cross Sectional ◽

Ocular Manifestations ◽

Visual Status ◽

The Mean

Background: Ocular complications of herpes zoster ophthalmicus (HZO) may lead to substantial visual disability, severe post-herpetic neuralgia and rarely fatal cerebral complications. Aim: To identify the pattern of ocular manifestation in herpes zoster ophthalmicus. Materials and methods: A cross-sectional descriptive study was under taken including the clinically diagnosed cases of HZO. All of them underwent a complete ophthalmological evaluation. Results: Sixty-eight cases of HZO were examined, of which 37 (54.4 %) were male and 31 (45.6%) female. The mean age was 48.7 ± 18.5 years. Most of the patients (64.7 %) were above the age of 40 years. 77.94 % of the patients had some form of ocular involvement. Pain (77.9 %) was the commonest ocular complaint. In young patients less than 35 years, HIV was the most common risk factor (19.3 %).Visual status was good in the majority (73.5 %) of patients at presentation. Lid and adnexal findings (45.8 %) were most common ocular involvement followed by conjunctivitis (41.1 %). Corneal complication was seen in 38.2 % of cases, uveitis in 19.1 % and post-herpetic neuralgia (PHN) and secondary glaucoma each in 5.8 %. Conclusion: Eyelid and ocular adnexal involvement is most commonly found in patients with herpes zoster ophthalmicus followed by corneal complication and uveitis. There needs to be awareness of ocular involvement, which can be sight threatening, among the HZO patients and other medical departments and an increased emphasis on regular ophthalmic examination. Key words: herpes virus, herpes zoster, conjunctivitis, keratitis DOI: http://dx.doi.org/10.3126/nepjoph.v3i2.5271 Nepal J Ophthalmol 2011; 3(2): 165-171

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A CASE OF DISSEMINATED TUBERCULOSIS WITH OCULAR INVOLVEMENT

Journal of Health and Allied Sciences NU ◽

10.1055/s-0040-1703875 ◽

2015 ◽

Vol 05 (01) ◽

pp. 094-096

Author(s):

Md Fekarul Islam ◽

Devdeep Mukherjee ◽

Ritabrata Kundu ◽

Prabal Chandra Niyogi ◽

Joydeep Das

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Corneal Opacity ◽

Ocular Involvement ◽

Disseminated Tuberculosis ◽

Ocular Manifestations ◽

The Central Nervous System ◽

Immunocompromised State ◽

Ocular Tb ◽

Posterior Synechiae

AbstractDisseminated Tuberculosis (DTB) refers to tubercular involvement of two or more non-contiguous sites and is commonly associated with immunocompromised state. It is an unusual presentation of Tuberculosis (TB), especially in the absence of immunodeficiency. 1.4% of patients with Pulmonary Tuberculosis (PTB) develop ocular manifestations but many patients with ocular TB have no evidence of PTB. Tuberculosis can cause a wide variety of ophthalmic findings, ranging from the ocular surface through the optic nerve and to the central nervous system. In this article, we report a case of Disseminated Tuberculosis with ocular involvement in a 5years old female. Our case is unique for the presence of bilateral squint, unilateral nebular type of corneal opacity, bilateral iritis with posterior synechiae and cataract at the same time. It lays emphasis on the fact that a patient with tuberculosis should be screened for multiple foci.

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Necrotizing Choroiditis-Retinitis as Presenting Symptom of Disseminated Aspergillosis after Lung Transplantation

European Journal of Ophthalmology ◽

10.1177/112067219700700316 ◽

1997 ◽

Vol 7 (3) ◽

pp. 294-296 ◽

Cited By ~ 12

Author(s):

I. Anteby ◽

M. Kramer ◽

G. Rahav ◽

D. Benezra

Keyword(s):

Lung Transplantation ◽

Fungal Infection ◽

Aspergillus Fumigatus ◽

Endogenous Endophthalmitis ◽

Ocular Involvement ◽

Blurred Vision ◽

Systemic Manifestations ◽

Lung Transplants ◽

Disseminated Aspergillosis ◽

Aspergillus Endophthalmitis

Background. Endogenous endophthalmitis due to Aspergillus is rare affecting the severely immunosuppressed population, in particular recipients of heart and lung transplants. Ocular involvement of aspergillosis has always been observed late in the course of the disease. Subject. A young woman noted blurred vision in one eye three weeks after lung transplantation. At this stage, no systemic manifestations of fungal infection were detected and the ocular findings were attributed to viral infection. Results. Twenty-four hours after the original ocular complaint, an aggressive endophthalmitis developed in the left eye. The possibility of fungal endophthalmitis was raised. Within 48 hours of her first ocular complaint the patient died. Cultures from a vitreous tap and from autopsy ocular specimens were positive for Aspergillus fumigatus. Conclusions. Aspergillus endophthalmitis may occur in patients undergoing lung transplantation despite antifungal therapy. Increased awareness of this unusual entity may be life and vision saving in these patients.

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P38 Never say never: new onset uveitis in adolescence

Rheumatology ◽

10.1093/rheumatology/kez416.005 ◽

2019 ◽

Vol 58 (Supplement_4) ◽

Cited By ~ 1

Author(s):

Imogen Kelly ◽

Joyce Davidson ◽

Mary Brennan ◽

Mary MacRae ◽

Julie Duncan

Keyword(s):

Disease Control ◽

Conflicts Of Interest ◽

Age At Onset ◽

Low Grade ◽

Eye Drops ◽

Biologic Treatment ◽

Steroid Injections ◽

Eye Screening ◽

History Of ◽

New Onset

Abstract Background To highlight the importance of considering new onset uveitis in adolescent patients with longstanding juvenile idiopathic arthritis (JIA). Methods We performed a retrospective review of electronic medical records. Results A 16 year-old girl was diagnosed in 2004, aged 15 months with extended oligoarticular JIA (ANA positive, >1/640 homogeneous). Her initial treatment included intra-articular steroid injections and approximately eighteen months later methotrexate, which she remained on for four years. During this time she received occasional intra-articular steroid injections. Etanercept was added in 2009 due to persistent active disease and she continued on this treatment regime for the next five years. Methotrexate was discontinued in 2013 due to intolerance. Etanercept was discontinued in 2014 due to disease remission but was restarted after three months when her arthritis flared. In 2018 biologic treatment was changed due to poor disease control. Etanercept was switched to adalimumab, initially alternate weeks but increasing to weekly together with multiple joint injections. Six months later, her disease control remained poor and drug levels and antibodies were measured. She had a strongly positive anti-drug antibody level>200 AU/ml and her adalimumab level was <0.4mgs/L (normal range 5-10). At this time our patient now aged 16 presented with a four-week history of intermittent reduced vision in her right eye. Ophthalmology review found bilateral anterior uveitis. This patient had regular eye screening from her JIA diagnosis until her 11th birthday, showing no previous evidence of uveitis. Following diagnosis of uveitis she was commenced on steroid eye drops and IV methylprednisolone to treat both her uveitis and arthritis. Infliximab, (6mg/kg) was commenced then increased (10mg/kg) for ongoing uveitis and methotrexate was restarted. Despite this treatment she has persistent low grade right uveitis. Her left eye has settled and she has no lens opacities or raised intraocular pressures. She continues with joint discomfort secondary to her hypermobility but her arthritis is currently quiescent. Conclusion Type of arthritis and age at onset historically dictated the risk of developing uveitis. However uveitis can present in any age group and in patients with any type of JIA. In our case uveitis developed for the first time almost 15years after diagnosis. With disease modifying treatments now widely used early in the management of JIA, uveitis may be masked and therefore present late or atypically. It is rare for uveitis to present so long after diagnosis, however this case highlights that rheumatology teams should be aware that it can still develop. Therefore we should never say never! Conflicts of Interest The authors declare no conflicts of interest.

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Incidence of Heparin Induced Thrombocytopenia in the Transplant Population: A Large Retrospective Cohort, Single Transplant Centre Experience

Blood ◽

10.1182/blood.v120.21.3320.3320 ◽

2012 ◽

Vol 120 (21) ◽

pp. 3320-3320

Author(s):

Syed Hassan ◽

Dania Khoulani ◽

Ami Badami ◽

Zaid Alirhayim ◽

Mohamad A. Younes ◽

...

Keyword(s):

Retrospective Cohort ◽

Conflicts Of Interest ◽

Organ Transplant ◽

Serotonin Release ◽

Confirmatory Test ◽

Thrombin Inhibitor ◽

Thromboembolic Events ◽

Transplant Recipients ◽

Heparin Induced Thrombocytopenia ◽

Transplant Patients

Abstract Abstract 3320 Objective: Heparin induced thrombocytopenia (HIT), a prothrombotic complication of heparin therapy, can lead to serious thromboembolic events and cause significant morbidity and mortality. Its occurrence has never been studies in transplant patients, where use of heparin products is very common. We aim to study its prevalence in the transplant population at our institute. Methods: This is a retrospective cohort, single center study which looked into the clinical and laboratory database of all the patients that has undergone any kind of transplant at our institution over a period of 25 years (January 1985 - December 2010). In patients with clinical suspicious of HIT, a pre-test probability was calculated using the 4T scoring system. Results of the laboratory test like the ELISA HIT antibody (HIT ab) test and the functional serotonin release assay test (SRA) along with clinical manifestation of skin necrosis or thromboembolic events were reviewed. Results: Medical records of 2800 patients that has undergone transplant from January 1985- December 2010 were reviewed. HIT antibody assay was performed in 262 patients in which HIT was suspected. Of these, only 48 (18%) patients (mean age 57 ± 11 years, 71% women) had HIT ab positive, 9 were pre transplant recipient and remaining 39 were post transplant recipients. Baseline characteristics of the transplant population are illustrated in Table.1. Confirmatory test, SRA was performed in 8 HIT antibody positive patients, of whom only 4 were positive. The mean 4T score in HIT suspected patients was 3.7 ±1.3, while the score in HIT ab positive patients was 4.2 ± 1.2. Thrombotic complications were seen in 11(0.4%) patients, with the highest incidence rate of 1% in heart transplant recipients. No transplant patient had skin manifestations. Direct Thrombin inhibitor (DTI) was used only in 5 patients who had thrombotic events. No other complication or mortality was reported in any of the HIT ab positive transplant patients. Conclusion: To our knowledge, this is the first study of its kind that has shown very low incidence of HIT in transplant population. In conclusion, transplant patients can safely undergo any type of organ transplant, without having any peri or post operative complications or immediate mortality related to HIT. Disclosures: No relevant conflicts of interest to declare.

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