BackgroundJuvenile Idiopathic Arthritis (JIA) is the commonest inflammatory arthritis of childhood worldwide.ObjectivesTo describe the incidence, clinical characteristics and experience with JIA in Singapore over a 10-year period.MethodsA prospective study was conducted in KK Women’s & Children’s Hospital and National University Hospital, Singapore from Jan 2009-Dec 2018. JIA was defined and classified according to ILAR criteria1. Demographics and clinical information were collected for every patient diagnosed with JIA.Results379 children were diagnosed with JIA (60.2% male, 71.2% Chinese). Average estimated incidence was 5.8 per 100000/yr. Clinical characteristics and treatment used are summarized in Table 1.OverallSystemicOligo-articularPolyarticular (RF -)Polyarticular (RF +)PsoriaticERAUndifferen-tiatedN (%)379 (100)32 (8.4)133 (35.1)47 (12.4)13 (3.4)10 (2.6)137 (36.1)7 (1.8)Onset age, yr*10.4(6.6-13.3)6.6(4.5-10.1)8.5(5.1-12.1)7.9(3.5-12.8)9.9(7.1-13.5)13.3(6.2-14.3)12.2(10.0-14.3)9.7(6.2-13.5)Diagnosis age, yr*10.9(7.1-14.0)6.4(4.1-10.2)9.0(6.0-13.4)8.6(3.7-13.3)10.2(7.4-13.7)13.8(10.7-15.3)12.6(10.7-14.7)11.2(9.2-15.1)Lag time, mth*2.9(1.0 – 7.4)0.7(0.3-1.2)3.1(1.1-7.6)3.0(1.6-9.8)2.6(2.1-4.6)5.0(1.2-12.4)3.6(1.2-8.1)8.3(1.6-36.5) ANA (n = 253)Positive124 (32.9)11 (34.4)50 (40.3)23 (48.9)5 (38.5)8 (80.0)27 (20.0)0 (0.0) RF (n = 374)Positive23 (6.1)5 (17.2)2 (1.5)0 (0.0)13 (100.0)0 (0.0)2 (1.5)1 (14.3) HLA-B27 (n = 368)Positive112 (30.4)0 (0.0)4 (3.1)2 (4.3)0 (0.0)0 (0.0)104 (75.9)2 (28.6)Uveitis diagnosed11 (2.9)0 (0.0)6 (4.5)1 (2.1)0 (0.0)0 (0.0)4 (2.9)0 (0.0)DMARD use257 (67.8)21 (65.6)47 (35.3)40 (85.1)13 (100.0)10 (100.0)121 (88.3)5 (71.4)Biologic use145 (38.3)17 (53.1)17 (12.8)17 (36.2)9 (69.2)3 (30.0)79 (57.7)3 (42.9)Table 1. *Median (IQR). Rest expressed in n (%).Disease modifying anti-rheumatic drugs (DMARD) were used in 67.8%, and younger age of diagnosis (p=0.002), longer lag time (p=0.004), males (p=0.018) and ERA (p=0.043) predicted their use. 38.3% required biologics, and males (p=0.047), longer lag time (p=0.037) and HLA-B27 positivity (p=0.030) were associated with their use. Uveitis incidence was 2.9%; 54.5% had oligoarthritis, 36.4% ERA, 9.1% polyarthritis (RF-). Younger age at JIA diagnosis was associated with uveitis (p=0.030).ConclusionERA is the commonest subtype of JIA in our cohort, explaining the male predominance and higher HLA-B27 positivity as compared to the West2. Longer lag time was associated with DMARD and biologic use, likely due to more severe disease at presentation. HLA-B27 association with biologic use may predict ineffectiveness of DMARD. Uveitis incidence was low as compared to Western literature that cites a prevalence of 11-30%3,4. Young age at diagnosis is a risk factor for uveitis. Interestingly, ANA-positivity and female gender were not associated with uveitis cases.References[1] Petty RE, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol. 2004Feb;31(2):390-...

This a preprint and has not been peer reviewed. Data may be preliminary.

BackgroundThe diagnosis of Kikuchi’s Disease (KD), also known as histiocytic necrotizing lymphadenitis, is often delayed as patients often undergo multiple investigations to exclude other causes of cervical lymphadenopathy, such as infection and malignancy1.ObjectivesTo describe the clinical features and treatment response of pediatric patients diagnosed with KD in our institution to aid early recognition.MethodsA retrospective study was conducted on patients < 18 years old diagnosed with KD in National University Hospital, Singapore, from Jan 2006 – Dec 2018. Demographics, clinical characteristics and treatment information were collected.Results14 patients were diagnosed with KD. All were Asian and majority were Chinese (64.2%). 57.1% were male and the age of presentation ranged between 2.8 to 17.9 years old. Fever duration at diagnosis ranged between 8 - 50 days. Median height of temperature was 39.8 (IQR: 39.4 – 40.0) deg C. This was frequently associated with neck pain and swelling (57.1%) and constitutional symptoms (71.4%) such as anorexia and weight loss. Other symptoms include abdominal symptoms of pain, vomiting or diarrhea (57.1%), rash (35.7%) and oral ulcers (28.6%).All patients had cervical lymphadenopathy either in the anterior cervical triangle or both anterior and posterior triangles, but not in the posterior triangle alone. This was usually multiple (92.9%), measuring ≥2cm in diameter (100%), tender (85.7%), and tended to be unilateral (64.3%); none had overlying warmth or skin changes. 14.3% had isolated hepatomegaly while 14.3% had hepatosplenomegaly.Common lab abnormalities included cytopaenias (50% bicytopaenia, 28.6% leucopaenia, 7.1% anaemia), elevated inflammatory markers C-reactive protein (CRP) (range: 12 – 117 mg/L), erythrocyte sedimentation rate (ESR) (range 15 – 116 mm/h) and lactate dehydrogenase (range: 480-1514 U/L). Only 3/14 had mild transaminitis. Interestingly, almost half had low alkaline phosphatase (ALP). All patients had normal to high complements and were negative for anti-dsDNA antibodies. Ultrasound of the neck typically revealed non-specific findings of reactive lymphadenopathy while 1 showed suspicious features of thickened cortex and loss of fatty hilum. 10/14 patients underwent excision biopsy which confirmed the diagnosis of Kikuchi Disease on histology. 3/14 did not have excision biopsy as they responded promptly to non-steroidal anti-inflammatory drugs (NSAIDs), one declined excision biopsy.All patients had received empiric antibiotics with no response. 64.3% required treatment with NSAIDs for an average of 2-4 weeks. Of the 4 patients who required steroid therapy, one was started at the diagnosis of KD, while the other 3 received steroids upon failure of NSAIDs. Response to either NSAIDs or prednisolone, if effective, was usually prompt, within 24 hours.ConclusionProlonged high grade fever with multiple tender cervical lymphadenopathy ≥ 2cm without warmth or redness and non-response to antibiotics, should raise concerns for KD. The presence of cytopaenia with mod...