Objectives To validate the previously proposed classifi cation criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA). MethodsStep 1: retrospective/prospective webdata collection for children with HSP, c-PAN, c-WG and c-TA with age at diagnosis ≤18 years.Step 2: blinded classifi cation by consensus panel of a representative sample of 280 cases.Step 3: statistical (sensitivity, specifi city, area under the curve and κ-agreement) and nominal group technique consensus evaluations. Results 827 patients with HSP, 150 with c-PAN, 60 with c-WG, 87 with c-TA and 52 with c-other were compared with each other. A patient was classifi ed as HSP in the presence of purpura or petechiae (mandatory) with lower limb predominance plus one of four criteria: (1) abdominal pain; (2) histopathology (IgA); (3) arthritis or arthralgia; (4) renal involvement. Classifi cation of c-PAN required a systemic infl ammatory disease with evidence of necrotising vasculitis OR angiographic abnormalities of medium-/small-sized arteries (mandatory criterion) plus one of fi ve criteria: (1) skin involvement; (2) myalgia/ muscle tenderness; (3) hypertension; (4) peripheral neuropathy; (5) renal involvement. Classifi cation of c-WG required three of six criteria: (1) histopathological evidence of granulomatous infl ammation; (2) upper airway involvement; (3) laryngo-tracheo-bronchial involvement; (4) pulmonary involvement (x-ray/CT); (5) antineutrophilic cytoplasmic antibody positivity; (6) renal involvement. Classifi cation of c-TA required typical angiographic abnormalities of the aorta or its main branches and pulmonary arteries (mandatory criterion) plus one of fi ve criteria: (1) pulse defi cit or claudication; (2) blood pressure discrepancy in any limb; (3) bruits; (4) hypertension; (5) Paediatric Rheumatology European Society propose validated classifi cation criteria for HSP, c-PAN, c-WG and c-TA with high sensitivity/specifi city. INTRODUCTIONIn 1990 the American College of Rheumatology (ACR) proposed classifi cation criteria for patients with vasculitides 1-5 by analysing 807 adults patients with different form of vasculitis: 85 with Henoch-Schönlein purpura (HSP), 118 with polyarteritis nodosa (PAN), 85 with Wegener granulomatosis (WG), 63 with Takayasu arteritis (TA) and 456 with other vasculitides (Churg-Strauss, hypersensitivity, giant cell arteritis and other unspecifi ed forms). 6 Patients with each specifi c vasculitis were compared with all the remaining diseases grouped into a single control category.The ACR criteria for HSP (sensitivity 87.1%, specifi city 87.7%) require the presence of at least two of the following: (1) age ≤20 years at disease onset; (2) palpable purpura; (3) acute abdominal pain; (4) biopsy showing granulocytes in the walls of small arterioles/venules. 1 The ACR criteria for PAN (sensitivity 82.2%, specifi city 86.6%) require at least three of the 10 following criteria: (1) granulocyte or mixed leucocyte infi ...

Objective: To evaluate the frequency of primary immunodeficiencies (PID) in juvenile systemic lupus erythematosus (JSLE) patients. Methods: Some 72 JSLE patients were analyzed for levels of immunoglobulin classes and IgG subclasses and early components of the classical complement pathway. Determination of C4 gene copy number (GCN) and detection of type I C2 deficiency (D) were also performed. Results: PID was identified in 16 patients (22%): C2D in three, C4D in three, C1qD in two, IgG2D (<20 mg/dl) in four, IgAD (<7 mg/dl) in three, and IgMD (<35 mg/dl) in three; one of these patients presented IgA, C2 and C4D. Two patients had low C4 GCN and two had type I C2D. Demographic data, family history of autoimmune disease and PID, JSLE clinical findings, occurrence of infections, disease activity and therapies were similar in patients with and without PID (p > 0.05). Remarkably, the median of Systemic Lupus International Collaborating Clinics/ACR-damage index (SLICC/ ACR-DI) was significantly higher in JSLE patients with PID compared with patients without these abnormalities (p ¼ 0.0033), likewise the high frequency of SLICC/ACR-DI > 1 (p ¼ 0.023). Conclusions: A high frequency of PID was observed in JSLE patients, suggesting that these defects may contribute to lupus development. Our findings indicate that these two groups of PID should be investigated in severe pediatric lupus. Lupus (2011) 20, 1275-1284.

Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (≥6 and <12 years) and group C adolescent (≥12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was significantly higher in group A compared to groups B and C (8.3 (0.1-23.4) vs 6.2 (0-17) vs 3.3 (0-14.6) years, p < 0.0001). The median Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) (0 (0-9) vs 0 (0-6) vs 0 (0-7), p = 0.065) was comparable in the three groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric (21% vs 10% vs 7%, p = 0.007), skin (10% vs 1% vs 3%, p = 0.002) and peripheral vascular involvements (5% vs 3% vs 0.3%, p = 0.008) were more often observed in group A compared to groups B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, and autoimmune hemolytic anemia were similar in all groups ( p > 0.05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p = 0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes. This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.

Background Takayasu’s arteritis (TA) is a chronic granulomatous vasculitis affecting the large and medium arteries and can result in end organ damage. The disease is rare and there are few reports about the clinical features in the pediatric population. Objectives To evaluate and to compare the clinical features of TA in Brazilian children and adolescents. Methods In this Brazilian multicenter retrospective study including 10 pediatric rheumatology centers we identified 71 children and adolescents with TA diagnosed before the age of 18 years according to the validated classification criteria for pediatric age1. Patients’ demographic, clinical, laboratory and angiographic data were collected. The angiographic type was classified according to the 1994 International Conference of Takayasu arteritis in Tokyo2. Patients were divided in 2 groups: children, younger than 10 years – group 1, and adolescents, older than or equal to 10 years – group 2 and their disease characteristics at initial presentation were compared. Results Thirty-six (50.7%) patients were in group 1 and 35 (49.3%) were in group 2. Twenty-one (58.3%) patients in group 1 and 30 (85.7%) patients in group 2 were girls (p=0.010). The mean onset age was 5.7 and 12.7 in groups 1 and 2 (p<0.001); the mean time to diagnosis was 1.8 and 0.7 in groups 1 and 2 (p= 0.001) and the mean follow-up time was 7.2 and 3.6 in groups 1 and 2 (p <0.001). The predominant clinical symptoms at onset were decreased pulses (85.9%), followed by arterial hypertension (84.5%), constitutional (77.5%) and neurological symptoms (70.4%), however without statistical differences between the two groups. The main laboratory finding was an increased erythrocyte sedimentation rate followed by leukocytosis. Anemia and thrombocytosis were significantly more frequent in group 1 (p = 0.031; p= 0.001). Angiographic data were similar in both groups. Conclusions Children under the age of 10 had more laboratory abnormalities, but similar clinical and angiographic features when compared to adolescents with TA. A delay in diagnosis is more common in younger patients and clinicians must be aware about this disease in this age group. References Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R et al. EULAR/PRINTO/PRES criteria for Henoch-Schoenlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu’s arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010;69:798-806. Hata A, Noda M, Moriwaki R, Numano F. Angiographic findings of Takayasu arteritis: New classification. Int J Cardiol.1996;54(suppl):S155-S163. Disclosure of Interest: None Declared

CONCLUSIONS:Although the second wave of COVID presented historically, with a higher number of cases in general, the healthcare workers showed some overall improvement regarding their burnout syndrome levels.