Genevieve Larkin

American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort.

We propose new classification criteria for Sjögrens syndrome (SS), which are needed considering the emergence of biologic agents as potential treatments and their associated comorbidity. These criteria target individuals with signs/symptoms suggestive of SS.

A simplified quantitative method for assessing keratoconjunctivitis sicca from the Sjögren's Syndrome International Registry.

PURPOSE To describe, apply, and test a new ocular grading system for assessing keratoconjunctivitis sicca (KCS) using lissamine green and fluorescein. DESIGN Prospective, observational, multicenter cohort study. METHODS The National Institutes of Health-funded Sjögrens Syndrome International Registry (called Sjögrens International Collaborative Clinical Alliance [SICCA]) is developing standardized classification criteria for Sjögren syndrome (SS) and is creating a biospecimen bank for future research. Eight SICCA ophthalmologists developed a new quantitative ocular grading system (SICCA ocular staining score [OSS]), and we analyzed OSS distribution among the SICCA cohort and its association with other phenotypic characteristics of SS. The SICCA cohort includes participants ranging from possibly early SS to advanced disease. Procedures include sequenced unanesthetized Schirmer test, tear break-up time, ocular surface staining, and external eye examination at the slit lamp. Using statistical analyses and proportional Venn diagrams, we examined interrelationships between abnormal OSS (>or=3) and other characteristics of SS (labial salivary gland [LSG] biopsy with focal lymphocytic sialadenitis and focus score >1 positive anti-SS A antibodies, anti-SS B antibodies, or both). RESULTS Among 1208 participants, we found strong associations between abnormal OSS, positive serologic results, and positive LSG focus scores (P < .0001). Analysis of the overlapping relationships of these 3 measures defined a large group of participants who had KCS without other components of SS, representing a clinical entity distinct from the KCS associated with SS. CONCLUSIONS This new method for assessing KCS will become the means for diagnosing the ocular component of SS in future classification criteria. We find 2 forms of KCS whose causes may differ.

Pseudo-hypopyon as the presenting feature in B-cell and T-cell intraocular lymphoma

The clinicopathologic correlation of two patients with primary intraocular lymphoma is described, both of whom had a rare and unusual presentation of hypopyon uveitis. Sampling of aqueous fluid proved the cytopathologic diagnosis of B‐cell lymphoma in the first patient. In the second patient an iris biopsy confirmed a T‐cell lymphoma. The samples were examined using haematoxylin and eosin, and immunohistochemical stains. These patients were diagnosed with primary intraocular lymphoma on initial presentation with hypopyon uveitis. Secondary glaucoma was noted in the patient with T‐cell lymphoma. Both cases appeared confined to the eye and initially responded favourably to aggressive chemotherapy and radiotherapy, but later went on to develop systemic involvement. Clinicians should be aware of this dramatic mode of presentation, which is unusual for ocular lymphoma.

The development and validation of the King's Sarcoidosis Questionnaire for the assessment of health status

Rationale Health status is impaired in patients with sarcoidosis. There is a paucity of tools that assess health status in sarcoidosis. The objective of this study was to develop and validate the Kings Sarcoidosis Questionnaire (KSQ), a new modular health status measure. Methods Patients with sarcoidosis were recruited from outpatient clinics. The development of the questionnaire consisted of three phases: item generation; item reduction, Rasch analysis to create unidimensional scales and validation; repeatability testing. Results 207 patients with sarcoidosis (organ involvement: 184 lung, 54 skin, 45 eye disease) completed a 65-item preliminary questionnaire. 36 items were removed due to redundancy or poor fit to the Rasch model. The final version of the KSQ consisted of five modules (General health status, Lung, Skin, Eye, Medications). Internal consistency assessed with Cronbachs α coefficient was 0.70–0.93 for KSQ modules. Concurrent validity of the Lung module was high compared with St Georges Respiratory Questionnaire (r=−0.83) and moderate when compared to forced vital capacity (r=0.49). Concurrent validity with skin-specific and eye-specific measures ranged from r=−0.4 to 0.8. The KSQ was repeatable over 2 weeks (n=39), intraclass correlation coefficients for modules were 0.90–0.96. Conclusions The KSQ is a brief, valid, self-completed health status measure for sarcoidosis. It can be used in the clinic to assess sarcoidosis from the patients’ perspective.

Asymptomatic choroidal granulomas in common variable immunodeficiency

Common variable immunodeficiency disease represents the most common cause of significant antibody deficiency in adults. It is a heterogeneous group of disorders that can be sporadic or familial with various modes of inheritance with defects in B‐cell function, regulatory T‐cells and macrophage function all being reported. Herein a case of asymptomatic bilateral choroidal granulomatous disease in a 27‐year‐old man with common variable immunodeficiency disease is described.

Evaluation of a minor eye conditions scheme delivered by community optometrists

Background The establishment of minor eye conditions schemes (MECS) within community optometric practices provides a mechanism for the timely assessment of patients presenting with a range of acute eye conditions. This has the potential to reduce waiting times and avoid unnecessary referrals to hospital eye services (HES). Objective To evaluate the clinical effectiveness, impact on hospital attendances and patient satisfaction with a minor eye service provided by community optometrists. Methods Activity and outcome data were collected for 12 months in the Lambeth and Lewisham MECS. A patient satisfaction questionnaire was given to patients at the end of their MECS appointment. A retrospective difference-in-differences analysis of hospital activity compared changes in the volume of referrals by general practitioners (GPs) from a period before (April 2011–March 2013) to after (April 2013–March 2015) the introduction of the scheme in Lambeth and Lewisham relative to a neighbouring area (Southwark) where the scheme had not been commissioned. Appropriateness of case management was assessed by consensus using clinical members of the research team. Results A total of 2123 patients accessed the scheme. Approximately two-thirds of patients (67.5%) were referred by their GP. The commonest reasons for patients attending for a MECS assessment were ‘red eye’ (36.7% of patients), ‘painful white eye’ (11.1%) and ‘flashes and floaters’ (10.2%). A total of 64.1% of patients were managed in optometric practice and 18.9% were referred to the HES; of these, 89.2% had been appropriately referred. First attendances to HES referred by GPs reduced by 26.8% (95% CI −40.5% to −13.1%) in Lambeth and Lewisham compared to Southwark. Conclusions The Lambeth and Lewisham MECS demonstrates clinical effectiveness, reduction in hospital attendances and high patient satisfaction and represents a successful collaboration between commissioners, local HES units and primary healthcare providers.

Correlation between ultrasound imaging of major salivary glands and histopathological findings of labial gland biopsy samples in Sjogren's syndrome

Abstract Background Ultrasound imaging has been recognised as a non-invasive, reproducible method for assessing the major salivary glands. The aim of this study was to investigate the relation between ultrasound imaging of major glands and focal lymphocytic score (FS) of minor salivary glands in Sjogrens syndrome. Methods Biopsies of labial glands and ultrasound imaging of major glands were done on 196 patients (mean age 55 years, 19 male, 177 female) attending the Sjogrens Clinic at Guys Hospital. FS of labial glands was judged by two histopathologists whose assessment was standardised. Ultrasonography was done with a single Philips-iU22 Ultrasound machine, and a disease severity score (US) was determined. Patients were categorised into three groups: those diagnosed with Sjogrens syndrome according to the American-European classification criteria (Sjogrens syndrome group), those with non-specific sialadenitis (sialadenitis group), and those with normal biopsy results (non-salivary gland disease group). Findings There was a highly significant correlation between FS and US scores (mean score 1·8 [SD 2·4, range 0–11·3] vs 2·7 [2·9, 0–9], r =0·665, n=196; p vs 5·0 [2·4, 0–9], r =0·395, n=87; p vs 1·1 [1·7, 0–7], r =0·321, n=66; p vs 0·5 [SD 1·2, 0–5], n=43). Interpretation This is the first large-scale study to show the strong relation between US scores of major salivary glands, and FS scores of minor salivary glands, suggesting a uniform disease process. Ultrasound analysis proves to be an important methodology in management and studies of Sjogrens syndrome. Funding National Institute for Health Research.

P86 The assessment of health related quality of life in sarcoidosis with the King's Sarcoidosis Questionnaire (KSQ)

Introduction The Kings Sarcoidosis Questionnaire (KSQ) is a recently developed and validated sarcoidosis specific health related quality of life (HRQOL) tool comprising of 5 modules: general HRQOL (10 items), lung (6 items), medication/side-effects (3 items), skin (4 items), and eye (7 items). We set out to evaluate HRQOL in a large group of patients with wide ranging sarcoidosis and determine the factors that influence it. Methods 207 patients with sarcoidosis (89% lung, 26% skin, 22% eye, 29% other organ involvement) attending outpatient clinics at Kings College and Royal Brompton Hospitals completed the KSQ. KSQ domain scores range from 0 to 100, a higher score representing a better HRQOL. Demographic data, immunosuppressant medication, organ involvement, lung function, Scadding CXR stage, physicians global assessment (PGA) of severity of skin disease and visual acuity (VA) were recorded. Results Patients had a mean (SEM) age 48 (11) years, 54% were female and 30% were Afro-Caribbean. Patients had a mean (SEM) FEV1 80 (23)% predicted, FVC 94 (19)% predicted and TLCO % predicted 66 (17). HRQOL was impaired in all domains, mean (SEM) scores: general HRQOL 51 (2), lung 61 (2), medication/side-effects 49 (3), skin 54 (4), and eye 50 (4). Patients with 2 or more organ involvement compared to single organ involvement had worse general HRQOL (44 (3) vs 58 (3); p<0.01) and worse medication/side-effects scores (44 (3) vs 58 (3); p=0.04). Female patients compared to males had worse general HRQOL (45 (3) vs 57 (3); p<0.01) and medication/side-effects scores (41 (3) vs 58 (4); p<0.01). There were no associations between HRQOL and age (r=−0.02 to 0.13) or ethnicity (p=0.42). There was a weak but significant relationship between lung HRQOL and FEV1 (r=0.38, p<0.01), FVC (r=0.38, p<0.01) and TLCO % predicted (r=0.22, p<0.01). Patients with Scadding CXR stage 3–4 disease compared to stage 0–2 disease had significantly worse lung HRQOL (51 (4) vs 63 (3); p=0.02). Skin health was associated with physicians global assessment (PGA) of severity of skin disease (r=0.51, p<0.01). Eye health was associated with VA (r=−0.56, p<0.01). Patients taking immunosuppressant medication for sarcoidosis compared to those not taking immunosuppresants had significantly worse general HRQOL (45 (2) vs 66 (4); p<0.01) and lung HRQOL scores (58 (3) vs 70 (4); p=0.01). Conclusions HRQOL is impaired in sarcoidosis. Gender, immunosuppressant medication, multi-system organ involvement and severity of lung function impact on HRQOL. This study provides further clinical validation of the KSQ.

Clinical safety of a minor eye conditions scheme in England delivered by community optometrists

Objective The aim of this study was to monitor the activity and evaluate the clinical safety of a minor eye conditions scheme (MECS) conducted by accredited community optometrists in Lambeth and Lewisham, London. Methods and analysis Optometrists underwent an accredited training programme, including attendance at hospital eye services (HES) clinics. Patients who satisfied certain inclusion criteria were referred to accredited MECS optometrists by their general practitioners (GPs) or could self-refer. Data were extracted from clinical records. A sample of MECS clinical records was graded to assess the quality of the MECS optometrists’ clinical management decisions. Referrals to the HES were assessed by the collaborating ophthalmologists and feedback was provided. Results A total of 2123 patients (mean age 47 years) were seen over 12 months. Two-thirds of the patients (67.3%) were referred by their GP. The most common reasons for patients needing a MECS assessment were ‘red eye’ (36.7% of patients), ‘painful white eye’ (11.1%), ‘flashes and floaters’ (10.2%); 8.7% of patients had a follow-up appointment. Of the patients seen, 75.1% were retained in the community, 5.7% were referred to their GP and 18.9% were referred to the HES. Of the HES referrals, 49.1% were routine, 22.6% urgent and 28.3% emergency. Of the records reviewed, 94.5% were rated as appropriately managed; 89.2% of the HES referrals were considered appropriate. Conclusion The findings of this study indicate that optometrists are in a good position to work very safely within the remits of the scheme and to assess risk.