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Objective: To achieve a consensus-based definition of Low Disease Activity (LDA) for use in cSLE trials. Methods: The International cSLE T2T Task Force, comprising of paediatric rheumatologists/nephrologists, and adult rheumatologists undertook a series of Delphi surveys/consensus meetings to discuss, refine, and vote upon cSLE LDA criteria. Results: The Task Force agreed that LDA should be based upon the adult-SLE Lupus Low Disease Activity State definition (LLDAS), with modifications to make it applicable to cSLE (cLLDAS). They agreed upon five cLLDAS criteria: (1) SLE Disease Activity Index (SLEDAI)-2 K ≤4, with no activity in major organ systems; (2) no new features of lupus disease activity compared with the last assessment; (3) Physician Global Assessment score of ≤1 (0–3 scale); (4) prednisolone dose of ≤0.15 mg/kg/day, 7.5 mg/day/maximum; while on (5) stable antimalarials, immunosuppressives, and biologics. Conclusions: A cSLE-appropriate definition of cLLDAS has been generated, maintaining alignment with the adult-SLE definition to promote life-course research.

Background Systemic lupus erythematous (SLE) is a systemic autoimmune/inflammatory condition. Approximately 15–20% of patients develop symptoms before their 18th birthday and are diagnosed with juvenile-onset SLE (JSLE). Gender distribution, clinical presentation, disease courses and outcomes vary significantly between JSLE patients and individuals with adult-onset SLE. This study aimed to identify age-specific clinical and/or serological patterns in JSLE patients enrolled to the UK JSLE Cohort Study. Methods Patient records were accessed and grouped based on age at disease-onset: pre-pubertal (≤7 years), peri-pubertal (8–13 years) and adolescent (14–18 years). The presence of American College of Rheumatology (ACR) classification criteria, laboratory results, disease activity [British Isles Lupus Assessment Group (BILAG) and Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K) scores] and damage [Systemic Lupus International Collaborating Clinics (SLICC) damage index] were evaluated at diagnosis and last follow up. Results A total of 418 JSLE patients were included in this study: 43 (10.3%) with pre-pubertal disease onset; 240 (57.4%) with peri-pubertal onset and 135 (32.3%) were diagnosed during adolescence. At diagnosis, adolescent JSLE patients presented with a higher number of ACR criteria when compared with pre-pubertal and peri-pubertal patients [pBILAG2004 scores: 9(4–20] vs. 7(3–13] vs. 7(3–14], respectively, p = 0.015] with increased activity in the following BILAG domains: mucocutaneous ( p = 0.025), musculoskeletal ( p = 0.029), renal ( p = 0.027) and cardiorespiratory ( p = 0.001). Furthermore, adolescent JSLE patients were more frequently ANA-positive ( p = 0.034) and exhibited higher anti-dsDNA titres ( p = 0.001). Pre-pubertal individuals less frequently presented with leukopenia ( p = 0.002), thrombocytopenia ( p = 0.004) or low complement ( p = 0.002) when compared with other age groups. No differences were identified in disease activity (pBILAG2004 score), damage (SLICC damage index) and the number of ACR criteria fulfilled at last follow up. Conclusions Disease presentations and laboratory findings vary significantly between age groups within a national cohort of JSLE patients. Patients diagnosed during adolescence exhibit greater disease activity and “classic” autoantibody, immune cell and complement patterns when compared with younger patients. This supports the hypothesis that pathomechanisms may vary between patient age groups.

Purpose To have an insight of the outcomes of half-dose photodynamic therapy (PDT) for patients with chronic central serous chorioretinopathy (CSC) in a real world population. Material and methods This is a retrospective consecutive case series, one-centre study, with a 12-month follow-up period. Results The study took into account 15 eyes of 13 patients. All patients were male in gender and only 1 eye belonged to a non-caucasian. The sample mean age was 51,27 ± 11,66 (between 36 and 74 years); mean baseline best-corrected visual acuity (BCVA) was 0,40 ± 0,25 logMAR and central macular thickness (CMT) was 411,40 ± 88,44 μm. All patients had subretinal fluid (SRF) and retinal pigment epithelium (RPE) detachment involving the fovea. In a month time, BCVA and CMT had remarkable improvements with only one eye not gaining letters. At 6-month visit, 46,7% of the eyes were provided with additional treatment (intra-vitreal anti-VEGF or PDT). In the final visit no eye had worsened more than 5 letters and 53,3% had a BCVA improvement of at least 5 letters, with only 2 eyes remaining with RPE detachment and SRF. There was a steady regression in CMT throughout the follow-up; 93,3% had CMT under 300 μm and CMT mean was 249,93 ± 72,78 μm. There were no reported complications. Conclusion These results support PDT as a valuable approach for therapeutic decisions on chronic CSC as well as the need of other add up treatments for a better final outcome. Further evaluations with longer follow-up’s should be taken into consideration for safety issues.

Objective: One of the primary factors in managing patients with retinoblastoma is early diagnosis. The main idea of this study was to recognize the consequences of delay in diagnosis on therapy of the disease. Methods: A retrospective review of all children with proven retinoblastoma, who had presented to MAHAK hospital in Tehran, from April 2007 to Dec 2011, was performed. Grouping of intraocular tumors was applied as A to E according to International Classification of Retinoblastoma. Findings : There were 157 (91 boys) children eligible for study. The mean age was 1.21±0.11 years with average delay in diagnosis of 3.4±0.53 months. Classification of D group in both unilateral (93 patients) and bilateral tumors was the largest category. A significant relation (P=0.05) between delayed diagnosis time and tumor grouping was evident. The most frequent symptoms were leukocoria and strabismus. Age was significantly lower in the subgroup of bilateral tumors than in unilateral retinoblastomas (0.6±0.12 year vs 1.6±0.15 years). The diagnosis was delayed in subgroup of extra ocular retinoblastoma more than in intraocular tumors (8.7±2.9 months vs 2.9±0.52 months). Conclusion: The authors recommend early referring of suspected cases to ophthalmologists and pediatric oncologists and to organize educational programs to publisize signs and symptoms of the disease such as leukocoria, strabismus and ocular inflammatory disorders through national media. In conclusion, early diagnosis of retinoblastoma can be the primary factor in managing the patients as the delay in diagnosis accounts for highly advanced disease and poor prognosis.