Objective To evaluate if the 2019-European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) criteria at diagnosis of childhood-onset systemic lupus erythematosus (cSLE) are associated with higher rates of early damage scored by Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI). Methods This retrospective multicenter study included 670 cSLE patients with ≤5 years of disease duration. All patients fulfilled both 2019-EULAR/ACR and 1997-ACR classification criteria. Total score of 2019-EULAR/ACR criteria and each of its specific domains were assessed at diagnosis as predictors of damage accrual at the last visit, according to the presence of any organ damage (defined by SDI ≥ 1). Results Median disease duration was 2.8 (IQR 1.8–3.8) years and 200 (29.9%) patients had at least one organ damage (SDI ≥ 1). The most frequent domains were neuropsychiatric (12%), renal (7%), and musculoskeletal (6%). There was a higher frequency of renal (58% vs 43%, p = 0.0004) and neuropsychiatric domain (21% vs 7%, p < 0.0001) of 2019-EULAR/ACR criteria in patients with damage (SDI ≥ 1) compared to those without damage (SDI = 0). Patients scoring renal or neuropsychiatric domains of the 2019-EULAR/ACR criteria at diagnosis were associated with renal damage (odds ratio 9.701, 95% confidence interval 3.773–24.941, p < 0.001) or neuropsychiatric damage (OR 9.480, 95% CI 5.481–16.399, p<0.0001) at latest visit, respectively. cSLE patients with positive anti-dsDNA at diagnosis were also associated with renal damage by the latest visit (OR 2.438, 95% CI 1.114–5.3381, p = 0.021). Constitutional, hematologic, mucocutaneous, serosal, and musculoskeletal domains and specific criteria as well as other immunologic criteria were not associated with damage accrual. Median of SLEDAI-2K was significantly higher in patients with global damage (19.5 (2–51) vs 14 (0–51), p<0.001). 2019-EULAR/ACR score >25 was associated with more overall (SDI ≥ 1) (38% vs 25%, p = 0.0002) and renal damage (11% vs 5%, p = 0.023). Conclusions The 2019-EULAR/ACR criteria at diagnosis were associated with a higher rate of early damage in cSLE patients, especially for renal and neuropsychiatric damage. Of note, damage was particularly associated with high disease activity at diagnosis and 2019-EULAR/ACR score >25.

Data represent n (%). Patients with JIA or healthy controls (control) were evaluated for parental smoking using minimally adjusted general linear models with robust errors estimators (adjusted for age, sex, disease category, parental education, family monthly income).CI indicates confidence interval.

BACKGROUNDJuvenile localized scleroderma (JLS), also known as morphea, is a spectrum of rare inflammatory pediatric disorders associated with skin and underlying tissue thickening and sclerosis. Clinical manifestations range from skin lesions to extracutaneous involvement that often vary with its subtype. Early recognition is important to reduce disease-related functional impact. METHODSReview of medical records of children and adolescents with diagnosis of JLS from 2015 to May 2022 and regularly followed up in the Pediatric Rheumatology Unit of a tertiary hospital in the state of Ceará was performed. Data included demographics, clinical manifestations, treatment and disease course. RESULTSTwenty patients were included and 13 (65%) were female. The median age at onset, age at diagnosis and time elapsed from symptom onset to diagnosis were, respectively, 6.60 (2-16.4), 9.05 (3.2-17) and 2.45 (0-10.5) years. The most prevalent subtype was mixed morphea (35%), followed by linear form (25%). Involvement was predominantly appendicular, distributing in upper and/or lower limbs in 75%. Five patients had disease restricted to the face, two with en coup de sabre variant and three with Parry-Romberg syndrome. Most of the patients (75%) already had characteristics of long-standing skin disease at diagnosis. Seventeen patients (85%) had extracutaneous manifestations, with a predominance of joint involvement (arthritis/arthralgia of small joints of the hands, wrists and knees) in 13 (65%) of them, followed by gastrointestinal tract (20%). Autoantibodies were positive in 9 (45%) patients, of which ANA (78%) and RF (22%) were the most prevalent. Eighteen patients used corticosteroids at diagnosis, with an average of 8.5 months treatment, and a partial response in 15 (83.3%) of them. Methotrexate was associated in 19 cases, allowing 12 (63.1%) patients to achieve remission. In refractory cases (35%), six received mycophenolate mofetil and two cyclophosphamide. After approximately 3 years of follow-up, 85% of patients had good disease control. CONCLUSIONThe female-to-male ratio (1.85) and the age at onset of symptoms (6.60) are in line with literature (1.7:1 to 3.7:1 and 6.1 to 8.1, respectively). Our study differs in the higher prevalence of mixed morphea subtype in relation to the linear one. Time until diagnosis was long, with most patients presenting already with chronic skin changes, warning about the lack of knowledge about the disease. Immunosuppressive standard treatment, mainly with methotrexate and corticosteroids, shows promising results in controlling the disease progression.

BACKGROUNDLeprosy, a chronic infectious disease caused by Mycobacterium leprae, affects the skin, peripheral nerves, upper respiratory tract, musculoskeletal system and eyes. As it presents a wide spectrum of clinical manifestations, it can be a diagnostic challenge, especially in early stages of disease. Some of these manifestations resemble pictures of rheumatic diseases that affect adults and children. Among the musculoskeletal manifestations of childhood leprosy are inflammatory chronic arthritis, mimicking juvenile idiopathic arthritis or spondyloarthritis, inflammatory swelling of the hands and feet, neuropathic arthritis, septic arthritis, arthralgias/ myalgias, soft tissue rheumatism and multisystem involvement similar to collagenases, including vasculitis and myositis. CASE REPORTWe report the case of a previously healthy 8-year-old boy admitted to the pediatric rheumatology unity due to the presence of puffy hands and fingers and hard and shiny edema of the lower limbs, suggesting systemic scleroderma diagnosis. He had an 11-month history of recurrent episodes of intermittent fever, plaque-like, erythematous, nonpruritic facial skin lesions and joint pain in the knees and ankles. On physical examination, he presented, in addition to the aforementioned findings, purpuric lesions on the toes, hepatosplenomegaly, arthralgias (wrist, small joints of the hands, knees and ankles) and erythematous plaques infiltrated in the bilateral malar region, nasal region and auricular pavilion. Complementary tests showed normocytic and normochromic anemia, leukocytosis, neutrophilia, thrombocytosis, elevation of inflammatory tests and negativity of ANA, RF, ANCA, anti-RNP and anti-Scl70. Due to the infiltrated skin lesions, lymph smear testing was performed, which was strongly positive, and a diagnosis of Virchowian leprosy associated with mixed leprosy reaction was made. There was clinical and laboratory improvement after initiation of multidrug therapy and systemic corticosteroid therapy. CONCLUSIONLeprosy is known as a great mimic of rheumatic diseases, often fulfilling diagnostic criteria for many of them. The multibacillary forms present greater musculoskeletal involvement. It should be considered in differential diagnosis of children with musculoskeletal symptoms, autoantibody positivity and cutaneous and/or neurological involvement.

Medical semiology is a crucial assessment method for the construction of diagnostic hypotheses, through the collection of anamnesis and physical examination in search of important findings for the creation of an effective clinical reasoning. In addition, it directs the rational request for exams and helps to establish a good doctor-patient relationship. Thus, it is essential that this assessment is improved during medical training. In this context, a cross-sectional, observational, analytical, quantitative and qualitative study was carried out, with the application of a questionnaire using the GoogleForms tool, to evaluate students of the medical course at the University of Fortaleza, in order to analyze the importance of different methodologies of teaching semiology during medical training. The results obtained showed that the practical performance in a hospital environment is an effective method of teaching semiology, favoring the solidification of learning through contact with patients, observation of clinical findings, collection of anamnesis and constant physical examination.